312 results on '"Christian Agard"'
Search Results
2. History of pre-eclampsia does not appear to be a risk factor for vascular phenotype in women with systemic sclerosis
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Arsène Mekinian, Eric Hachulla, Alain Lescoat, Elisabeth Pasquier, Christian Agard, Sabine Berthier, Perrine Smets, Grégory Pugnet, Emmanuel Chatelus, Marie-Elise Truchetet, Viviane Queyrel, Nathalie Tieulie, Bénédicte Rouvière, Claire de Moreuil, Emilie Brenaut, Valérie Devauchelle-Pensec, Olivier Lidove, Jérémy Keraen, Benjamin Subran, Nicolas Belhomme, Elizabeth Diot, Mathieu Delplanque, Sandy Lucier, and Emmanuelle Courtois-Communier
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Medicine - Abstract
Background Vascular phenotype is associated with a poor prognosis in systemic sclerosis (SSc). The identification of its risk factors could facilitate its early detection.Objectives To explore risk factors for a vascular phenotype of SSc, among them a history of pre-eclampsia.Methods This observational multicentre case–control study enrolled adult women fulfilling European Alliance of Associations for Rheumatology 2013 diagnosis criteria for SSc and having a pregnancy history≥6 months before SSc diagnosis in 14 French hospital-based recruiting centres from July 2020 to July 2022. Cases had specific vascular complications of SSc defined as history of digital ischaemic ulcers, pulmonary arterial hypertension, specific cardiac involvement or renal crisis. Women with SSc were included during their annual follow-up visit and filled in a self-administered questionnaire about pregnancy. A case report form was completed by their physician, reporting data on medical history, physical examination, clinical investigations and current medication. The main outcome was the presence/absence of a personal history of pre-eclampsia before SSc diagnosis, according to the validated pre-eclampsia questionnaire.Results 378 women were included: 129 cases with a vascular phenotype and 249 matched controls. A history of pre-eclampsia was reported in 5 (3.9%) cases and 12 (4.8%) controls and was not associated with a vascular phenotype (OR=0.96, 95% CI 0.28 to 3.34, p=0.9). Besides, Rodnan skin score and disease duration≥5 years were risk factors for vascular phenotype.Conclusions In women with SSc and a pregnancy history≥6 months before SSc, a history of pre-eclampsia is not associated with a vascular phenotype.
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- 2024
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3. Factors associated with satisfaction with social roles and activities among people with systemic sclerosis: a Scleroderma Patient-centered Intervention Network (SPIN) cohort cross-sectional study
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Arsène Mekinian, Thierry Martin, Eric Hachulla, Carter Thorne, Danielle Rice, Andrea Benedetti, Brooke Levis, Virginia Steen, Paul R Fortin, Vincent Poindron, Aurélien Guffroy, David Launay, Luc Mouthon, Mandana Nikpour, John Varga, Benjamin Chaigne, Sindhu R Johnson, Sébastien Rivière, Michael Hughes, Daphna Harel, Marie-Eve Carrier, Karen Nielsen, Susan J Bartlett, Karen Gottesman, Ghassan El-Baalbaki, Kim Fligelstone, Catherine Fortune, Tracy Frech, Marie Hudson, Maggie Larche, Catarina Leite, Janet Pope, Anne A Schouffoer, Maria E Suarez-Almazor, Christian Agard, Marc André, Sabine Berthier, Lyne Bissonnette, Alessandra Bruns, Patricia Carreira, Marion Casadevall, Lorinda Chung, Christopher Denton, Robyn Domsic, James V Dunne, Bertrand Dunogue, Regina Fare, Dominique Farge-bancel, Jessica Gordon, Brigitte Granel-Rey, Genevieve Gyger, Monique Hinchcliff, Alena Ikic, Niall Jones, Suzanne Kafaja, Nader Khalidi, Marc Lambert, Hélène Maillard, Joanne Manning, Ariel Masetto, François Maurier, Susanna Proudman, Alexis Régent, David Robinson, Sophie Roux, Perrine Smets, Vincent Sobanski, Robert Spiera, Evelyn Sutton, Pearce Wilcox, Laurent Alric, Grégory Pugnet, François Rannou, Amy Gietzen, Christelle Nguyen, Michelle Richard, Nancy Maltez, Isabelle Marie, Mara Cañedo Ayala, Geneviève Guillot, Elana J Bernstein, Brett Thombs, Paul Legendre, Thylbert Deltombe, Sabrina Hoa, Laura K Hummers, Sophie Blaise, Yvonne C Lee, Louis Olagne, Marie-Claude Geoffroy, Richard S Henry, Robyn Wojeck, Maureen Mayes, Tiffany Dal Santo, Kimberly Lakin, Gabrielle Virgili-Gervais, Vanessa Malcarne, Claire E Adams, Rodriguez-Reyna Tatiana Sofia, Floryan Beaslay, Eva Bories, Carlotta Cacciatore, Benjamin Crichi, Tannvir Desroche, Loraine Gauzère, Anne Gerber, Maria Martin Lopez, Sheila Melchor Díaz, Morgane Mourguet, Loïc Raffray, Frederic Renou, Esther Rodríguez Almazar, Damien Vagner, Vanessa Cook, Sophie Hu, Elsa-Lynn Nassar, Marieke Alexandra Neyer, and Sabrina Provencher
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Medicine - Abstract
Objective The objectives were to (1) compare satisfaction with social roles and activities in a large multinational systemic sclerosis (SSc) cohort to general population normative data and (2) identify sociodemographic, lifestyle and SSc disease factors associated with satisfaction with social roles and activities.Methods Participants in the Scleroderma Patient-centered Intervention Network Cohort completed the Patient Reported Outcomes Information System Version 2 satisfaction with social roles and activities domain questionnaire. Multivariable regression was used to assess associations with sociodemographic, lifestyle and disease factors.Results Among 2385 participants, mean satisfaction with social roles and activities T-score (48.1, SD=9.9) was slightly lower than the US general population (mean=50, SD=10). Factors independently associated with satisfaction were years of education (0.54 per SD, 95% CI 0.14 to 0.93); non-White race or ethnicity (−1.13, 95% CI −2.18 to –0.08); living in Canada (−1.33, 95% CI −2.40 to –0.26 (reference USA)) or the UK (−2.49, 95% CI −3.92 to –1.06); body mass index (−1.08 per SD, 95% CI −1.47 to –0.69); gastrointestinal involvement (−3.16, 95% CI −4.27 to –2.05); digital ulcers (−1.90, 95% CI −3.05 to –0.76); moderate (−1.62, 95% CI −2.78 to –0.45) or severe (−2.26, 95% CI −3.99 to –0.52) small joint contractures; interstitial lung disease (−1.11, 95% CI −1.97 to –0.25); pulmonary arterial hypertension (−2.69, 95% CI −4.08 to –1.30); rheumatoid arthritis (−2.51, 95% CI −4.28 to –0.73); and Sjogren’s syndrome (−2.42, 95% CI −3.96 to –0.88).Conclusion Mean satisfaction with social roles and activities is slightly lower in SSc than the general population and associated with multiple sociodemographic and disease factors.
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- 2024
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4. Disease patterns and specific trajectories of anti-MDA5-related disease: a multicentre retrospective study of 70 adult patients
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Hubert de Boysson, Marie Cuchet, Charles Cassius, Pierre Cuchet, Christian Agard, Alexandra Audemard-Verger, Sylvain Marchand-Adam, Raphaëlla Cohen-Sors, Laure Gallay, Julie Graveleau, Cécile Lesort, Kim Ly, Alain Meyer, Grégoire Monseau, Antoine Néel, Bernard Bonnotte, Laurent Pérard, Nicolas Schleinitz, Delphine Mariotte, Brigitte Le Mauff, Gwladys Bourdenet, Wafa Masmoudi, Samuel Deshayes, Anaël Dumont, Anne Dompmartin, Diane Kottler, and Achille Aouba
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anti-MDA5 dermatomyositis ,prognosis ,rapidly progressive interstitial lung disease ,thromboembolic events ,malignancy ,Immunologic diseases. Allergy ,RC581-607 - Abstract
IntroductionThis study aimed to provide an updated analysis of the different prognostic trajectories of patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibodies.MethodsAmong a cohort of 70 patients, baseline characteristics and phenotypes, treatments and outcomes were analyzed. A Cox proportional hazards model was used to identify factors associated with poor outcomes, i.e., death or progressive disease at the last follow-up.ResultsAmong the 70 patients, 45 were women, and 54 were Caucasian. A dermatologic involvement was observed in 58 (83%) patients, including 40 with MDA5 vasculopathy-related skin lesions. Muscular involvement was observed in 39 (56%) patients. Interstitial lung disease (ILD) was observed at baseline in 52 (74%) patients, including 23 (44%) who developed rapidly progressive (RP) ILD. Seven (10%) patients showed thromboembolic complications within the first weeks of diagnosis, and eight (11%) other patients developed a malignancy (4 before the diagnosis of anti-MDA5 disease). Poor outcomes were observed in 28 (40%) patients, including 13 (19%) deaths. Among the 23 patients with RP-ILD, 19 (79%) showed poor outcomes, including 12 (63%) who died. In multivariate analyses, RP-ILD (hazard ratio (HR), 95% CI: 8.24 [3.21–22], p
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- 2024
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5. Patterns of patient-reported symptoms and association with sociodemographic and systemic sclerosis disease characteristics: a scleroderma Patient-centered Intervention Network (SPIN) Cohort cross-sectional studyResearch in context
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Robyn K. Wojeck, Mitchell R. Knisely, Donald E. Bailey, Tamara J. Somers, Linda Kwakkenbos, Marie-Eve Carrier, Warren R. Nielson, Susan J. Bartlett, Vanessa L. Malcarne, Marie Hudson, Brooke Levis, Andrea Benedetti, Luc Mouthon, Brett D. Thombs, Susan G. Silva, Claire E. Adams, Richard S. Henry, Catherine Fortuné, Karen Gottesman, Geneviève Guillot, Laura K. Hummers, Amanda Lawrie-Jones, Maureen D. Mayes, Michelle Richard, Maureen Sauvé, Shervin Assassi, Ghassan El-Baalbaki, Kim Fligelstone, Tracy Frech, Amy Gietzen, Daphna Harel, Monique Hinchcliff, Sindhu R. Johnson, Maggie Larche, Catarina Leite, Christelle Nguyen, Karen Nielsen, Janet Pope, François Rannou, Tatiana Sofia Rodriguez-Reyna, Anne A. Schouffoer, Maria E. Suarez-Almazor, Christian Agard, Nassim Ait Abdallah, Marc André, Elana J. Bernstein, Sabine Berthier, Lyne Bissonnette, Alessandra Bruns, Patricia Carreira, Marion Casadevall, Benjamin Chaigne, Lorinda Chung, Benjamin Crichi, Christopher Denton, Robyn Domsic, James V. Dunne, Bertrand Dunogue, Regina Fare, Dominique Farge-Bancel, Paul R. Fortin, Jessica Gordon, Brigitte Granel-Rey, Aurélien Guffroy, Genevieve Gyger, Eric Hachulla, Sabrina Hoa, Alena Ikic, Suzanne Kafaja, Nader Khalidi, Kimberly Lakin, Marc Lambert, David Launay, Yvonne C. Lee, Hélène Maillard, Nancy Maltez, Joanne Manning, Isabelle Marie, Maria Martin Lopez, Thierry Martin, Ariel Masetto, François Maurier, Arsene Mekinian, Sheila Melchor Díaz, Mandana Nikpour, Louis Olagne, Vincent Poindron, Susanna Proudman, Alexis Régent, Sébastien Rivière, David Robinson, Esther Rodríguez Almazar, Sophie Roux, Perrine Smets, Vincent Sobanski, Robert Spiera, Virginia Steen, Evelyn Sutton, Carter Thorne, John Varga, Pearce Wilcox, Mara Cañedo Ayala, Vanessa Cook, Sophie Hu, Bianca Matthews, Elsa-Lynn Nassar, Marieke Alexandra Neyer, Julia Nordlund, and Sabrina Provencher
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Systemic sclerosis ,Patient-reported symptoms ,Symptom cluster ,Medicine (General) ,R5-920 - Abstract
Summary: Background: Systemic sclerosis is a heterogenous disease in which little is known about patterns of patient-reported symptom clusters. We aimed to identify classes of individuals with similar anxiety, depression, fatigue, sleep disturbance, and pain symptoms and to evaluate associated sociodemographic and disease-related characteristics. Methods: This multi-centre cross-sectional study used baseline data from Scleroderma Patient-centered Intervention Network Cohort participants enrolled from 2014 to 2020. Eligible participants completed the PROMIS-29 v2.0 measure. Latent profile analysis was used to identify homogeneous classes of participants based on patterns of anxiety, depression, fatigue, sleep disturbance, and pain scores. Sociodemographic and disease-related characteristics were compared across classes. Findings: Among 2212 participants, we identified five classes, including four classes with “Low” (565 participants, 26%), “Normal” (651 participants, 29%), “High” (569 participants, 26%), or “Very High” (193 participants, 9%) symptom levels across all symptoms. Participants in a fifth class, “High Fatigue/Sleep/Pain and Low Anxiety/Depression” (234 participants, 11%) had similar levels of fatigue, sleep disturbance, and pain as in the “High” class but low anxiety and depression symptoms. There were significant and substantive trends in sociodemographic characteristics (age, education, race or ethnicity, marital or partner status) and increasing disease severity (diffuse disease, tendon friction rubs, joint contractures, gastrointestinal symptoms) across severity-based classes. Disease severity and sociodemographic characteristics of “High Fatigue/Sleep/Pain and Low Anxiety/Depression” class participants were similar to the “High” severity class. Interpretation: Most people with systemic sclerosis can be classified by levels of patient-reported symptoms, which are consistent across symptoms and highly associated with sociodemographic and disease-related variables, except for one group which reports low mental health symptoms despite high levels of other symptoms and substantial disease burden. Studies are needed to better understand resilience in systemic sclerosis and to identify and facilitate implementation of cognitive and behavioural strategies to improve coping and overall quality of life. Funding: National Institute of Nursing Research (F31NR019007), Canadian Institutes of Health Research, Arthritis Society Canada, the Lady Davis Institute for Medical Research, the Jewish General Hospital Foundation, McGill University, Scleroderma Society of Ontario, Scleroderma Canada, Sclérodermie Québec, Scleroderma Manitoba, Scleroderma Atlantic, Scleroderma Association of BC, Scleroderma SASK, Scleroderma Australia, Scleroderma New South Wales, Scleroderma Victoria, and Scleroderma Queensland.
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- 2023
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6. Thoracic lymphadenopathies in diffuse systemic sclerosis: an observational study on 48 patients using computed tomography
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Arthur Renaud, Raphael Pautre, Olivier Morla, Aurélie Achille, Cécile Durant, Olivier Espitia, Eric Frampas, and Christian Agard
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Systemic sclerosis ,Scleroderma ,Thoracic lymphadenopathies ,Interstitial lung disease ,Diseases of the respiratory system ,RC705-779 - Abstract
Abstract Background Thoracic multidetector computed tomography (MDCT) is essential for the detection of interstitial lung disease (ILD) in patients with systemic sclerosis (SSc). Thoracic MDCT assessment can reveal the presence of thoracic lymphadenopathies (LAP) whose signification remains uncertain. The purpose of the study was to describe the characteristics and to assess the significance of thoracic LAP in patients with diffuse SSc. Methods We conducted a monocentric observational study on adult patients with diffuse SSc, and collected general patient and first thoracic MDCT characteristics, PET-CT and outcome data. Comparisons were made between patients with and without thoracic LAP. Results Forty-eight patients were included. There were 30 patients (62.5%) with an ILD and 23 (48%) with at least one thoracic LAP on the first MDCT assessment. Median number per patient of thoracic LAP was 3 [1–8], with a mean size of 11.7 ± 1.7 mm, mainly located in right para-tracheal area (22.8% of the total number of LAP), right hilar area (20.3%), left hilar area (6.5%), and sub-carinal area (15.2%). PET-CT showed lymph node hypermetabolism in 11/15 patients (73.3%) with mean SUVmax at 4 ± 1.3. There were significantly more males (p = 0.002) and more patients exposed to silica (p = 0.001) in patients with thoracic LAP. ILD was significantly more extended according to Goh score (p = 0.03), and using semi-quantitative score for mixed ground-glass reticulation (p = 0.01) and global abnormalities (p = 0.03) in patients with thoracic LAP and ILD. Thirteen patients (27.1%) died during follow-up without significant difference according to the presence or not of thoracic LAP (p = 0.15). There was also no significant difference concerning immunosuppressive treatment initiation (p = 0.17). Conclusions Thoracic LAP are common in diffuse SSc and are generally multiple, not bulky, moderately hypermetabolic, and located at the base of the mediastinum lymph node chains. Their presence correlates with the extent of ILD. In absence of ILD, thoracic LAP presence seems to be often explained by silica exposure. Trial Registration: NA.
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- 2022
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7. Specific features to differentiate Giant cell arteritis aortitis from aortic atheroma using FDG-PET/CT
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Olivier Espitia, Jérémy Schanus, Christian Agard, Françoise Kraeber-Bodéré, Jeanne Hersant, Jean-Michel Serfaty, and Bastien Jamet
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Medicine ,Science - Abstract
Abstract Aortic wall 18F-fluorodeoxyglucose (FDG)-uptake does not allow differentiation of aortitis from atheroma, which is problematic in clinical practice for diagnosing large vessel vasculitis giant-cell arteritis (GCA) in elderly patients. The purpose of this study was to compare the FDG uptake characteristics of GCA aortitis and aortic atheroma using positron emission tomography/FDG computed tomography (FDG-PET/CT). This study compared FDG aortic uptake between patients with GCA aortitis and patients with aortic atheroma; previously defined by contrast enhanced CT. Visual grading according to standardized FDG-PET/CT interpretation criteria and semi-quantitative analyses (maximum standardized uptake value (SUVmax), delta SUV (∆SUV), target to background ratios (TBR)) of FDG aortic uptake were conducted. The aorta was divided into 5 segments for analysis. 29 GCA aortitis and 66 aortic atheroma patients were included. A grade 3 FDG uptake of the aortic wall was identified for 23 (79.3%) GCA aortitis patients and none in the atheroma patient group (p
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- 2021
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8. French recommendations for the management of systemic sclerosis
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Eric Hachulla, Christian Agard, Yannick Allanore, Jerome Avouac, Brigitte Bader-Meunier, Alexandre Belot, Alice Berezne, Anne-Sophie Bouthors, Geraldine Condette-Wojtasik, Joël Constans, Pascal De Groote, Elisabeth Diot, Florence Dumas, Patrick Jego, Francisca Joly, David Launay, Veronique Le Guern, Janine-Sophie Le Quintrec, Geraldine Lescaille, Christophe Meune, Bruno Moulin, Christelle Nguyen, Nadine Omeish, Frederic Pene, Marie-Aleth Richard, Juliette Rochefort, Alexandra Roren, Olivier Sitbon, Vincent Sobanski, Marie-Elise Truchetet, Luc Mouthon, and Collaborators
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Systemic sclerosis ,Recommendations ,Treatment ,Medicine - Abstract
Abstract Systemic sclerosis (SSc) is a generalized disease of the connective tissue, arterioles, and microvessels, characterized by the appearance of fibrosis and vascular obliteration. There are two main phenotypical forms of SSc: a diffuse cutaneous form that extends towards the proximal region of the limbs and/or torso, and a limited cutaneous form where the cutaneous sclerosis only affects the extremities of the limbs (without passing beyond the elbows and knees). There also exists in less than 10% of cases forms that never involve the skin. This is called SSc sine scleroderma. The prognosis depends essentially on the occurrence of visceral damage and more particularly interstitial lung disease (which is sometimes severe), pulmonary arterial hypertension, or primary cardiac damage, which represent the three commonest causes of mortality in SSc. Another type of involvement with poor prognosis, scleroderma renal crisis, is rare (less than 5% of cases). Cutaneous extension is also an important parameter, with the diffuse cutaneous forms having less favorable prognosis.
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- 2021
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9. Symptomatic aortitis at giant cell arteritis diagnosis: a prognostic factor of aortic event
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Olivier Espitia, Gauthier Blonz, Geoffrey Urbanski, Cédric Landron, Jérôme Connault, Christian Lavigne, Pascal Roblot, François Maillot, Alexandra Audemard-Verger, Mathieu Artifoni, Cécile Durant, Béatrice Guyomarch, Mohamed Hamidou, Julie Magnant, Christian Agard, and French Study Group for Large Vessel Vasculitis (GEFA)
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Giant cell arteritis ,Aortitis ,Aneurysm ,Aortic dissection ,Prognosis ,Diseases of the musculoskeletal system ,RC925-935 - Abstract
Abstract Background Giant cell arteritis (GCA) is frequently associated with aortic involvement that is likely to cause life-threatening structural complications (aneurysm, dissection). Few studies have investigated the occurrence of these complications, and no predictive factor has been identified so far. The aim of this study was to investigate factors associated with the risk of aortic complications in a cohort of GCA aortitis. Methods Data of all patients managed with aortitis (CT or 18 FDG PET) at the diagnosis of GCA in five hospitals from May 1998 and April 2019 were retrospectively collected. Clinical features were compared according to the presence of aortitis symptoms. The predictive factors of occurrence or aggravation of aortic structural abnormalities were investigated. Results One hundred and seventy-one patients with GCA aortitis were included; 55 patients (32%) had symptoms of aortitis (dorsal/lumbar/abdominal pain, aortic insufficiency) at diagnosis. The median follow-up was 38 months. Aortic complications occurred after a median time of 32 months. There were 19 new aortic aneurysms or complications of aneurysm and 5 dissections. Survival without aortic complication was significantly different between the symptomatic and non-symptomatic groups (Log rank, p = 0.0003). In multivariate analysis the presence of aortitis symptoms at diagnosis (HR 6.64 [1.95, 22.6] p = 0.002) and GCA relapse (HR 3.62 [1.2, 10.9] p = 0.02) were factors associated with the occurrence of aortic complications. Conclusion In this study, the presence of aortitis symptoms at the diagnosis of GCA aortitis and GCA relapse were independent predictive factors of occurrence of aortic complications during follow-up.
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- 2021
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10. Rheumatic involvement and bone scan features in Schnitzler syndrome: initial and follow-up data from a single-center cohort of 25 patients
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Christelle Darrieutort-Laffite, Catherine Ansquer, Hélène Aubert, Françoise Kraeber-Bodéré, Agathe Masseau, Christian Agard, Mohamed Hamidou, Claire Bernier, Jean-Marie Berthelot, Benoit Le Goff, Sébastien Barbarot, and Antoine Néel
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Bone scan ,Bone lesions ,Interleukin 1 receptor antagonist ,Schnitzler syndrome ,Diseases of the musculoskeletal system ,RC925-935 - Abstract
Abstract Objective To report on the characteristics and long-term course of rheumatic manifestations in Schnitzler syndrome (SchS). Methods A retrospective cohort study of patients with SchS followed between 2000 and 2020. Inclusion criteria included a diagnosis of SchS (Strasbourg criteria). All available bone scans were reviewed and scored according to the intensity and number of pathological sites. The scintigraphic score was compared with the clinical activity score, CRP level, and treatments. Results Twenty-five patients were included. Median age at diagnosis was 68 years. Eighty patients (72%) had SchS-related rheumatic pain. Most patients had a long-standing isolated rash before constitutional and/or rheumatic symptoms appeared. The monoclonal component level was usually very low (IgMκ in 22/25). Rheumatic pain predominated around the knees. Bone scans revealed abnormal tracer uptake in 15/18 (85%). The scintigraphic score correlated with clinical activity (r = 0.4, p
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- 2020
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11. Early trajectories of skin thickening are associated with severity and mortality in systemic sclerosis
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Emmanuel Ledoult, David Launay, Hélène Béhal, Luc Mouthon, Grégory Pugnet, Jean-Christophe Lega, Christian Agard, Yannick Allanore, Patrick Jego, Anne-Laure Fauchais, Jean-Robert Harlé, Sabine Berthier, Achille Aouba, Arsène Mekinian, Elisabeth Diot, Marie-Elise Truchetet, Carine Boulon, Alain Duhamel, Eric Hachulla, Vincent Sobanski, and the French National Scleroderma Cohort Network
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Systemic sclerosis ,Modified Rodnan skin score ,Skin thickening trajectories ,Clinical heterogeneity ,Diseases of the musculoskeletal system ,RC925-935 - Abstract
Abstract Background Systemic sclerosis (SSc) is a severe and highly heterogeneous disease. The modified Rodnan skin score (mRSS) is a widely used tool for the assessment of the extent and degree of skin thickness. This study aimed to identify the classes of patients with early similar skin thickening trajectories without any a priori assumptions and study their associations with organ involvement and survival. Methods From the French SSc national cohort, patients with a disease duration of less than 2 years at inclusion and with at least 2 mRSS available within the first 4 years of follow-up were enrolled. Classes of patients with similar mRSS trajectories were identified based on a latent class mixed model. The clinical characteristics and survival rate were compared between the obtained classes. Results A total of 198 patients fulfilled the inclusion criteria, with a total of 641 mRSS available. The median disease duration and follow-up were 0.8 (interquartile range 0.4; 1.2) and 6.3 (3.8; 8.9) years, respectively. Individual trajectories of mRSS were highly heterogeneous between patients. Models with 1–6 latent classes of trajectories were sequentially assessed, and the 5-class model represented the best fit to data. Each class was characterized by a unique global trajectory of mRSS. The median disease duration did not differ significantly between classes. Baseline organ involvement was more frequent in classes with significant change over time (classes 2–5) than in class 1 (low baseline mRSS without significant change over time). Using Cox regression, we observed a progressively increasing risk of death from classes 1 to 5. Conclusions Early identification of clinical phenotype based on skin thickening trajectories could predict morbi-mortality in SSc. This study suggested that mRSS trajectories characterization might be pivotal for clinical practice and future trial designs.
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- 2020
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12. Semi-Quantitative [18F]FDG-PET/CT ROC-Analysis-Based Cut-Offs for Aortitis Definition in Giant Cell Arteritis
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Olivier Espitia, Jérémy Schanus, Christian Agard, Françoise Kraeber-Bodéré, Alexis F. Guédon, Antoine Bénichou, Jean-Michel Serfaty, Sandrine Coudol, Matilde Karakachoff, and Bastien Jamet
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[18F]FDG-PET/CT ,aortitis ,giant cell arteritis ,aortic atheroma ,large vessel vasculitis ,diagnostic semi-quantitative thresholds ,Biology (General) ,QH301-705.5 ,Chemistry ,QD1-999 - Abstract
[18F]fluorodeoxyglucose-positron emission tomography/computed tomography ([18F]FDG-PET/CT) is used to diagnose large vessel vasculitis in giant cell arteritis (GCA). We aimed to define a semi-quantitative threshold for identifying GCA aortitis from aortic atheroma or the control. Contrast enhanced computed tomography (CECT) was used as the reference imaging for aortic evaluation and to define aortitis, aortic atheroma and control aortas. [18F]FDG-PET/CT was performed on 35 GCA patients and in two different control groups (aortic atheroma (n = 70) and normal control (n = 35)). Aortic semi-quantitative features were compared between the three groups. GCA patients without aortitis on CECT were excluded. Of the GCA patients, 19 (54.3%) were not on glucocorticoids (GC) prior to [18F]FDG-PET/CT. The SUVmax, TBRblood and TBRliver aortic values were significantly higher in the GCA aortitis group than in the aortic atheroma and control groups (p < 0.001). Receiver operating characteristic curve analyses brought to light quantitative cut-off values allowing GCA aortitis diagnosis with optimal sensitivity and specificity versus control or aortic atheroma patients for each PET-based feature analyzed. Considering the overall aorta, a SUVmax threshold of 3.25 and a TBRblood threshold of 1.75 had a specificity of 83% and 75%, respectively, a sensitivity of 81% and 81%, respectively, and the area under the ROC curve (AUC) was 0.86 and 0.83, respectively, for aortitis detection compared to control groups in GCA cases with GC. A SUVmax threshold of 3.45 and a TBRblood threshold of 1.97 had a specificity of 90% and 93%, respectively, a sensitivity of 89% and 89%, respectively, with an AUC of 0.89 and 0.96, respectively, for aortitis detection compared to the control in GC-free GCA cases. Discriminative thresholds of SUVmax and TBRblood for the diagnosis of GCA aortitis were established using CECT as the reference imaging.
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- 2022
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13. Gamma heavy chain disease associated with rheumatoid arthritis: a case report
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Gwenvaël Danic, Thomas Dejoie, Hélène Caillon, Aurélie Achille, Pierre Pottier, and Christian Agard
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γ-Heavy chain ,Capillary electrophoresis ,Rheumatoid arthritis ,Case report ,Medicine - Abstract
Abstract Background Gamma heavy chain disease (γ-HCD) is a monoclonal gammopathy defined by an abnormal clonal and isolated production of incomplete heavy chain gamma (γ), unable to bind with light chains kappa or lambda. This disease is rare and remains poorly described. Its association to lymphoid neoplasm is well established, but exceptional forms of γ-HCD may also accompany auto-immune diseases. We report here a new case of γ-HCD characterized by an indolent course with a 4-year follow-up, and its association with quiescent rheumatoid arthritis (RA). Case presentation We report the case of a 85-year old French white man followed for quiescent anti-CCP+ rheumatoid arthritis treated by prednisolone 4 mg/day and hydroxychloroquine 200 mg/day since 10 years, and a monoclonal gammopathy of undetermined significance for 6 years, who was hospitalized for costal fractures after a fall. Serum protein electrophoresis showed a stable small monoclonal peak, and capillary electrophoresis/immunosubtraction technique identified an isolated clonal γ-heavy chain (HC). Bone marrow aspiration was normal and he had no other lymphoproliferation. The monoclonal peak remained stable after 4 years of follow-up. Conclusions In case of monoclonal peak without complete monoclonal Ig on serum protein electrophoresis, the diagnosis of γ-HCD should be discussed and capillary electrophoresis/immune-subtraction is a mean to detect isolated monoclonal heavy chain (HC). Gamma-HC disease is rare, may be associated to RA, and may have an indolent course.
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- 2021
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14. Risk factors for symptomatic vascular events in giant cell arteritis: a study of 254 patients with large-vessel imaging at diagnosis
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Donatienne de Mornac, Christian Agard, Jean-Benoit Hardouin, Mohamed Hamidou, Jérôme Connault, Agathe Masseau, Alexandra Espitia-Thibault, Mathieu Artifoni, Chan Ngohou, François Perrin, Julie Graveleau, Cécile Durant, Pierre Pottier, Antoine Néel, and Olivier Espitia
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Diseases of the musculoskeletal system ,RC925-935 - Abstract
Aims: To identify factors associated with vascular events in patients with giant cell arteritis (GCA). Methods: We performed a retrospective study of GCA patients diagnosed over a 20-year-period, who all underwent vascular imaging evaluation at diagnosis. Symptomatic vascular events were defined as the occurrence of any aortic event (aortic dissection or symptomatic aortic aneurysm), stroke, myocardial infarction, limb or mesenteric ischemia and de novo lower limbs arteritis stage 3 or 4. Patients with symptomatic vascular event (VE+) and without were compared, and risk factors were identified in a multivariable analysis. Results: Thirty-nine (15.4%) of the 254 included patients experienced at least one symptomatic vascular event during follow-up, with a median time of 21.5 months. Arterial hypertension, diabetes, lower limbs arteritis or vascular complication at diagnosis were more frequent in VE+ patients ( p
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- 2021
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15. Large-vessel involvement is predictive of multiple relapses in giant cell arteritis
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Donatienne de Mornac, Olivier Espitia, Antoine Néel, Jérôme Connault, Agathe Masseau, Alexandra Espitia-Thibault, Mathieu Artifoni, Aurélie Achille, Anaïs Wahbi, Mathieu Lacou, Cécile Durant, Pierre Pottier, François Perrin, Julie Graveleau, Mohamed Hamidou, Jean-Benoit Hardouin, and Christian Agard
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Diseases of the musculoskeletal system ,RC925-935 - Abstract
Background: Giant cell arteritis (GCA) is the most common systemic vasculitis. Relapses are frequent. The aim of this study was to identify relapse risk factors in patients with GCA with complete large-vessel imaging at diagnosis. Methods: Patients with GCA followed in our institution between April 1998 and April 2018 were included retrospectively. We included only patients who had undergone large vascular imaging investigations at diagnosis by computed tomography (CT)-scan and/or positron emission tomography (PET)-scan and/or angio-magnetic resonance imaging (MRI). Clinical, biological, and radiological data were collected. Relapse was defined as the reappearance of GCA symptoms, with concomitant increase in inflammatory markers, requiring treatment adjustment. Relapsing patients (R) and non-relapsing patients (NR) were compared. Relapse and multiple relapses (>2) risk factors were identified in multivariable Cox analyses. Results: This study included 254 patients (73.2% women), with a median age of 72 years at diagnosis and a median follow up of 32.5 months. At diagnosis, 160 patients (63%) had an inflammatory large-vessel involvement on imaging, 46.1% (117 patients) relapsed at least once, and 21.3% (54 patients) had multiple relapses. The median delay of first relapse after diagnosis was 9 months. The second relapse delay was 21.5 months. NR patients had more stroke at diagnosis than R ( p = 0.03) and the brachiocephalic trunk was involved more frequently on CT-scan ( p = 0.046), as carotids ( p = 0.02) in R patients. Multivariate Cox model identified male gender [hazard ratio (HR): 0.51, confidence interval (CI) (0.27–0.96), p = 0.04] as a relapse protective factor, and peripheral musculoskeletal manifestations [HR: 1.74 (1.03–2.94), p = 0.004] as a relapse risk factor. Peripheral musculoskeletal manifestations [HR: 2.78 (1.23–6.28), p = 0.014], negative temporal artery biopsy [HR: 2.29 (1.18–4.45), p = 0.015], large-vessel involvement like upper limb ischemia [HR: 8.84 (2.48–31.56), p = 0.001] and inflammation of arm arteries on CT-scan [HR: 2.39 (1.02–5.58), p = 0.04] at diagnosis were risk factors of multiple relapses. Conclusion: Male gender was a protective factor for GCA relapse and peripheral musculoskeletal manifestations appeared as a relapsing risk factor. Moreover, this study identified a particular clinical phenotype of multi-relapsing patients with GCA, characterized by peripheral musculoskeletal manifestations, negative temporal artery biopsy, and large-vessel involvement with upper limb ischemia or inflammation of arm arteries. Plain language Summary At giant cell arteritis diagnosis, large-vessel inflammatory involvement is predictive of multiple relapses 46.1% of patients with GCA relapse, and 21.3% undergo multiple relapses; Male gender appears as a protective factor for relapsing in GCA; Peripheral musculoskeletal manifestations are a relapse and multiple relapses risk factor; A negative temporal artery biopsy is predictive of multiple relapses; Large-vessel involvement is predictive of multiple relapses.
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- 2021
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16. Deleterious neurological impact of diagnostic delay in immune-mediated thrombotic thrombocytopenic purpura
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Arthur Renaud, Aurélie Caristan, Amélie Seguin, Christian Agard, Gauthier Blonz, Emmanuel Canet, Marion Eveillard, Pascal Godmer, Julie Graveleau, Marie Lecouffe-Desprets, Hervé Maisonneuve, François Perrin, Mohamed Hamidou, and Antoine Néel
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Medicine ,Science - Abstract
Background Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare life-threatening thrombotic microangiopathy requiring urgent therapeutic plasma exchange (TPE). However, the exact impact of a slight delay in TPE initiation on the subsequent patients’ outcome is still controversial. Aim We aimed to study the frequency, short-term neurological consequences, and determinants of diagnostic delay in iTTP. Methods We conducted a retrospective monocentric study including patients with a first acute episode of iTTP (2005–2020) classified into 2 groups: delayed (>24h from first hospital visit, group 1) and immediate diagnosis (≤24h, group 2). Results Among 42 evaluated patients, 38 were included. Eighteen cases (47%) had a delayed diagnosis (median: 5 days). The main misdiagnosis was immune thrombocytopenia (67%). The mortality rate was 5% (1 death in each group). Neurological events (stroke/TIA, seizure, altered mental status) occurred in 67% vs 30% patients in group 1 and 2, respectively (p = 0.04). Two patients in group 1 exhibited neurological sequelae. The hospital length of stay was longer in group 1 (p = 0.02). At the first hospital evaluation, potential alternative causes of thrombocytopenia were more prevalent in group 1 (33% vs 5%, p = 0.04). Anemia was less frequent in group 1 (67% vs 95%, p = 0.04). All patients had undetectable haptoglobin levels. By contrast, 26% of schistocytes counts were Conclusion Diagnostic delay is highly prevalent in iTTP, with a significant impact on short-term neurological outcome. In patients with profound thrombocytopenia, the thorough search for signs of incipient organ dysfunction, systematic hemolysis workup, and proper interpretation of schistocytes count are the key elements of early diagnosis of TTP.
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- 2021
17. Risk Factors of Venous Thromboembolic Disease in Cancer Patients Treated with Immune Checkpoint Inhibitor
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Julien Denis le Sève, Alexis F. Guédon, Stéphanie Bordenave, Christian Agard, Jérôme Connault, Marc-Antoine Pistorius, Gaelle Quéreux, and Olivier Espitia
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Hematology - Abstract
Background Immune checkpoint inhibitors (ICIs) have revolutionized the management of cancers. The risk factors and pathophysiological mechanisms of venous thromboembolic events (VTEs) of this new therapeutic class are still to be specified. Methods The included patients had to have cancer and should be treated with ICI. Data analyzed included demographic data, biological data, and immune-related adverse events (IRAEs). We studied the prevalence of VTEs and the factors associated with VTEs. Results Of 374 patients on ICI, over a median follow-up period of 15.2 months, the number of VTE was 50 (13.4%). The majority of patients were treated for metastatic melanoma or nonsmall cell lung cancer. There was no difference in prevalence or survival between cancer types. Patients with combined therapy composed of nivolumab and ipilimumab had higher 1-year cumulative VTE occurrence (29.3% [95% confidence interval [CI]: 9.7; 44.6]) than patients with pembrolizumab (14.9%, [95%CI: 2.5; 25.8], p = 0.03) or nivolumab (9.1%, [95% CI: 5.0; 12.9], p Conclusion The prevalence of VTE was 14.2% under ICIs. IRAE and combine treatment of nivolumab and ipilimumab were associated with VTE. The pathophysiological mechanisms are multiple and complex with a possible link to aberrant activation of the immune system.
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- 2023
18. In-depth characterization of pulmonary arterial hypertension in mixed connective tissue disease: a French national multicenter study
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Benjamin Chaigne, Kevin Chevalier, Athenaïs Boucly, Christian Agard, Antoine Baudet, Arnaud Bourdin, Céline Chabanne, Vincent Cottin, Pierre Fesler, François Goupil, Patrick Jego, David Launay, Hervé Lévesque, Arnaud Maurac, Shirine Mohamed, Cécile Tromeur, Laurence Rottat, Olivier Sitbon, Marc Humbert, Luc Mouthon, Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre hospitalier universitaire de Nantes (CHU Nantes), Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Montpellier (UM), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), CHU Pontchaillou [Rennes], Laboratoire Traitement du Signal et de l'Image (LTSI), Université de Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM), Infections Virales et Pathologie Comparée - UMR 754 (IVPC), École Pratique des Hautes Études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Institute for Translational Research in Inflammation - U 1286 (INFINITE (Ex-Liric)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Institut Fédératif de Recherches Multidisciplinaires sur les Peptides (IFRMP 23), CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université Le Havre Normandie (ULH), Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre de Lutte Contre le Cancer Henri Becquerel Normandie Rouen (CLCC Henri Becquerel)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Groupe d'Etude de la Thrombose de Bretagne Occidentale (GETBO), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO)-Université de Brest (UBO), and Hôpital Bicêtre
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Rheumatology ,systemic lupus erythematosus ,[SDV.MHEP.RSOA]Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system ,systemic sclerosis ,pulmonary arterial hypertension ,[SDV]Life Sciences [q-bio] ,mixed connective tissue disease ,Pharmacology (medical) ,survival - Abstract
International audience; Abstract Objective pulmonary arterial hypertension (PAH) is a leading cause of death in mixed connective tissue disease (MCTD). We aimed to describe PAH in well-characterized MCTD patients. Methods MCTD patients enrolled in the French Pulmonary Hypertension Registry with a PAH diagnosis confirmed by right heart catheterization were included in the study and compared with matched controls: MCTD patients without PAH, systemic lupus erythematous (SLE) patients with PAH, and systemic sclerosis (SSc) patients with PAH. Survival rates were estimated by the Kaplan-Meier method and risk factors for PAH in MCTD patients and risk factors for mortality in MCTD-PAH were sought using multivariate analyses. Results thirty-six patients with MCTD-PAH were included in the study. Comparison with MCTD patients without PAH and multivariate analysis revealed that pericarditis, polyarthritis, thrombocytopenia, interstitial lung disease (ILD), and anti-Sm antibodies were independent predictive factors of PAH/PH in MCTD. Estimated survival rates at 1 year, 5 years, and 10 years, following PAH diagnosis were 83%, 67%, and 56%, respectively. MCTD-PAH presentation and survival did not differ from SLE-PAH and SSc-PAH. Multivariate analysis revealed that tobacco exposure was an independent factor predictive of mortality in MCTD-PAH. Conclusion PAH is a rare and severe complication of MCTD, associated with a 56% 10-year survival. We identified ILD, pericarditis, thrombocytopenia, and anti-Sm antibodies as risk factors for PAH in MCTD and tobacco exposure as predictor of mortality in MCTD-PAH.
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- 2023
19. Osteitis in Systemic Sclerosis: A Nationwide Case–Control Retrospective Study
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Benjamin Chaigne, Mickael Martin, Bénédicte Watelet, Elisabeth Diot, Claire Le Jeunne, François Maurier, Jérémie Dion, Luc Mouthon, Cloé Giret, Jean François Viallard, Sabine Berthier, Pascal Priollet, Marie-Elise Truchetet, Alain Lescoat, Olivier Cerles, Dorothée Fagedet, Cyril Cosse, Loic Raffray, Brigitte Granel, Christelle Nguyen, J. Bertolino, Ségolène Toquet, Solen Kernéis, David Luque Paz, Wendy Jourde, Christian Agard, and Grégory Pugnet
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medicine.medical_specialty ,Erythema ,medicine.drug_class ,Antibiotics ,Gastroenterology ,03 medical and health sciences ,0302 clinical medicine ,Rheumatology ,Internal medicine ,Skin Ulcer ,medicine ,Humans ,030212 general & internal medicine ,Abscess ,Osteitis ,Ulcer ,Retrospective Studies ,030203 arthritis & rheumatology ,Scleroderma, Systemic ,business.industry ,Septic shock ,Amoxicillin ,Soft tissue ,Retrospective cohort study ,medicine.disease ,3. Good health ,Case-Control Studies ,medicine.symptom ,beta-Lactamase Inhibitors ,business ,medicine.drug - Abstract
Systemic sclerosis (SSc) is an autoimmune connective tissue disorder characterized by skin fibrosis, vasculopathy, and dysimmunity. Data regarding osteitis in SSc are scarce.We performed a nationwide multicenter, retrospective, case-control study including patients with SSc, according to the 2013 American College of Rheumatology/European Alliance of Associations for Rheumatology classification, with a diagnosis of osteitis. The objectives of the study were to describe, to characterize, and to identify associated factors for osteitis in patients with SSc.Forty-eight patients were included. Twenty-six patients (54.1%) had osteitis beneath digital tip ulcers. Physical symptoms included pain (36 of 48, 75%), erythema (35 of 48, 73%), and local warmth (35 of 48, 73%). Thirty-one (65%) patients had median (interquartile range) C-reactive protein levels2 mg/liter of 8 (2.7-44.3) mg/liter. On radiography, computed tomography, or magnetic resonance imaging, osteitis was characterized by swelling or abscess of soft tissues, with acro-osteolysis or lysis in 28 patients (58%). Microbiological sampling was performed in 45 (94%) patients. Most pathogens were Staphylococcus aureus (43.8%), anaerobes and Enterobacteriaceae (29.1%), and Pseudomonas aeruginosa (10.4%). Management comprised antibiotics in 37 (77.1%) patients and/or surgery in 26 (54.2%). Fluoroquinolones were used in 22 (45.8%) patients, and amoxicillin plus β-lactamase inhibitor in 7 (14.6%). Six (12.6%) patients relapsed, 6 (12.6%) patients had osteitis recurrence, 15 (32%) sequelae, and 2 patients had septic shock and died.This study confirmed digital tip ulcers as an associated factor for osteitis and revealed a high rate of functional sequelae. Antimicrobial therapy with oral fluoroquinolone or intravenous amoxicillin and β-lactamase inhibitor are used as first-line antibiotic therapy in SSc patients with osteitis.
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- 2022
20. A qualitative interview study exploring the psychological health impacts of the SPIN-CHAT program among people with systemic sclerosis at the onset of COVID-19: Perceptions of trial participants and research team members
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Amanda Wurz, Delaney Duchek, Kelsey Ellis, Mannat Bansal, Marie-Eve Carrier, Lydia Tao, Laura Dyas, Linda Kwakkenbos, Brooke Levis, Ghassan El-Baalbaki, Danielle B. Rice, Yin Wu, Richard S. Henry, Laura Bustamante, Sami Harb, Shannon Hebblethwaite, Scott B. Patten, Susan J. Bartlett, John Varga, Luc Mouthon, Sarah Markham, Brett D. Thombs, S. Nicole Culos-Reed, Catherine Fortuné, Amy Gietzen, Geneviève Guillot, Karen Nielsen, Nancy Lewis, Michelle Richard, Maureen Sauvé, Joep Welling, Lacey Battaglio, Tina Burger, Adrienne Burleigh, Peggy Collins, Jacob Davila, Louise Inglese, Franny Kaplan, Violet Konrad, Silvia Petrella, Audrey Potvin, Natalie Puccio, Karen Gottesman, Marie Hudson, Laura K. Hummers, Amanda Lawrie-Jones, Vanessa L. Malcarne, Maureen D. Mayes, Warren R. Nielson, Shervin Assassi, Carolyn Ells, Kim Fligelstone, Tracy Frech, Daphna Harel, Monique Hinchcliff, Sindhu R. Johnson, Maggie Larche, Catarina Leite, Christelle Nguyen, Janet Pope, François Rannou, Tatiana Sofia Rodriguez-Reyna, Anne A. Schouffoer, Maria E. Suarez-Almazor, Christian Agard, Nassim Ait Abdallah, Marc André, Elana J. Bernstein, Sabine Berthier, Lyne Bissonnette, Alessandra Bruns, Patricia Carreira, Marion Casadevall, Benjamin Chaigne, Lorinda Chung, Benjamin Crichi, Christopher Denton, Robyn Domsic, James V. Dunne, Bertrand Dunogue, Regina Fare, Dominique Farge-Bancel, Paul R. Fortin, Jessica Gordon, Brigitte Granel-Rey, Aurélien Guffroy, Genevieve Gyger, Eric Hachulla, Ariane L. Herrick, Sabrina Hoa, Alena Ikic, Niall Jones, Nader Khalidi, Marc Lambert, David Launay, Yvonne C. Lee, Hélène Maillard, Nancy Maltez, Joanne Manning, Isabelle Marie, Maria Martin Lopez, Thierry Martin, Ariel Masetto, François Maurier, Arsene Mekinian, Sheila Melchor Díaz, Mandana Nikpour, Louis Olagne, Vincent Poindron, Susanna Proudman, Alexis Régent, Sébastien Rivière, David Robinson, Esther Rodríguez Almazar, Sophie Roux, Perrine Smets, Vincent Sobanski, Robert Spiera, Virginia Steen, Evelyn Sutton, Carter Thorne, Pearce Wilcox, Mara Cañedo Ayala, Marie-Nicole Discepola, Laury Montemurro, Elsa Lynn Nassar, Marieke Alexandra Neyer, Julia Nordlund, Nora Østbø, and Sabrina Provencher
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Experimental Psychopathology and Treatment ,Rehabilitation - Abstract
Explore trial participants’ and research team members’ perceptions of the impact of the videoconference-based, supportive care program (SPIN-CHAT Program) during early COVID-19 for individuals with systemic sclerosis (SSc). Data were collected cross-sectionally. A social constructivist paradigm was adopted, and one-on-one videoconference-based, semi-structured interviews were conducted with SPIN-CHAT Trial participants and research team members. A hybrid inductive-deductive approach and reflexive thematic analysis were used. Of the 40 SPIN-CHAT Trial participants and 28 research team members approached, 30 trial participants (Mean age = 54.9; SD = 13.0 years) and 22 research team members agreed to participate. Those who took part in interviews had similar characteristics to those who declined. Five themes were identified: (1) The SPIN-CHAT Program conferred a range of positive psychological health outcomes, (2) People who don’t have SSc don’t get it: The importance of SSc-specific programming, (3) The group-based format of the SPIN-CHAT Program created a safe space to connect and meet similar others, (4) The structure and schedule of the SPIN-CHAT Program reduced feelings of boredom and contributed to enhanced psychological health, (5) The necessity of knowledge, skills, and tools to self-manage SSc and navigate COVID-19. Participants’ and research team members’ perspectives elucidated SPIN-CHAT Program benefits and how these benefits may have been realized. Results underscore the importance of social support from similar others, structure, and self-management to enhance psychological health during COVID-19. clinicaltrials.gov (NCT04335279)IMPLICATIONS FOR REHABILITATIONThe videoconference-based, supportive care SPIN-CHAT Program enhanced psychological health amongst individuals affected by systemic sclerosis.SPIN-CHAT Program participants and research team members shared that being around similar others, program structure, and self-management support were important and may have contributed to enhanced psychological health.Further efforts are required to explore experiences within supportive care programs to better understand if and how psychological health is impacted. The videoconference-based, supportive care SPIN-CHAT Program enhanced psychological health amongst individuals affected by systemic sclerosis. SPIN-CHAT Program participants and research team members shared that being around similar others, program structure, and self-management support were important and may have contributed to enhanced psychological health. Further efforts are required to explore experiences within supportive care programs to better understand if and how psychological health is impacted.
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- 2023
21. Caractéristiques des patients traités par prostanoïdes pour une hypertension artérielle pulmonaire associée à une connectivite : étude multicentrique rétrospective
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Christian Lavigne, D. Horeau-Langlard, E. Diot, Frédéric Gagnadoux, M. Fournet, Christian Agard, V. Génin, and Cécile Durant
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Gynecology ,medicine.medical_specialty ,business.industry ,Gastroenterology ,Internal Medicine ,medicine ,medicine.disease ,business ,Connective tissue disease ,Associated Pulmonary Arterial Hypertension - Abstract
Resume Introduction L’hypertension arterielle pulmonaire (HTAP) est une complication grave des connectivites. Les donnees d’utilisation des prostanoides dans cette population sont peu nombreuses. L’objectif de cette etude etait de decrire les caracteristiques des patients traites par prostanoides dans cette indication, et de rapporter les donnees d’efficacite et de tolerance. Methodes Il s’agit d’une etude retrospective multicentrique. Les patients traites depuis 2006 ont ete identifies dans 5 centres hospitaliers. Les donnees concernant l’HTAP et la connectivite sous-jacente ont ete recueillies avant introduction des prostanoides et sous traitement. Resultats Vingt-et-un patients ont ete inclus, dont 20 (95 %) avaient une sclerodermie systemique cutanee limitee. Dix-neuf patients etaient traites par mono- ou bitherapie orale avant adjonction du prostanoide. Le treprostinil etait la molecule la plus utilisee (57 % des patients). A l’introduction du prostanoide, 19 patients (90 %) etaient consideres a haut risque de mortalite. Parmi les patients ayant eu un catheterisme cardiaque droit de reevaluation, il n’y avait pas de difference significative des parametres hemodynamiques par rapport a l’evaluation avant prostanoide. Aucune aggravation extrarespiratoire de la connectivite n’a ete constatee sous traitement. La survie a 1 an de l’introduction de prostanoide etait de 62 %. En analyse univariee, la classe fonctionnelle NYHA etait correlee a la survie sous traitement. Conclusion Cette etude presente des donnees originales d’utilisation des prostanoides dans une cohorte composee majoritairement de patients avec une sclerodermie systemique sous-jacente. Elle souligne la difficulte d’effectuer un suivi standardise sous traitement, chez des patients fragiles. La tolerance des prostanoides est superposable a celle rapportee dans l’HTAP idiopathique.
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- 2021
22. The Scleroderma Patient-centered Intervention Network Self-Management (SPIN-SELF) Program: protocol for a two-arm parallel partially nested randomized controlled feasibility trial with progression to full-scale trial
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Evelyn Sutton, Lorinda Chung, Sophie Roux, Robert Spiera, Maria E. Suarez-Almazor, Thierry Martin, Shadi Gholizadeh, Carolyn Ells, Isabelle Marie, Louis Olagne, Monique Hinchcliff, Karen Nielsen, Geneviève Guillot, Alena Ikic, Jessica K. Gordon, Christian Agard, Maria Gagarine, Hélène Maillard, Julie Cumin, Angelica Bourgeault, David Robinson, Susanna Proudman, Amy Gietzen, Maureen D. Mayes, François Rannou, Dan Bilsker, Joanne Manning, Richard S. Henry, Ariel Masetto, Isabelle Boutron, Catherine Fortune, Elana J. Bernstein, Carter Thorne, Cornelia H. M. van den Ende, Christopher P. Denton, Sindhu R. Johnson, Regina Fare, Nassim Ait Abdallah, Alexander W. Levis, Maria Martin, Sabrina Hoa, Eric Hachulla, Anne A. Schouffoer, Susan J. Bartlett, Marie Hudson, Sébastien Rivière, Pearce G. Wilcox, Mara Cañedo Ayala, Sheila Melchor, Ariane L. Herrick, Tracy M. Frech, Andrea Benedetti, Laura Dyas, Janet E. Pope, Dominique Farge-Bancel, Andrea Carboni Jiménez, Maggie Larché, Perrine Smets, Vanessa L. Malcarne, Julia Nordlund, Marie-Nicole Discepola, Lyne Bissonnette, Maureen Sauve, Christelle Nguyen, Marion Casadevall, Brett D. Thombs, Karen Gottesman, Patricia Carreira, Marie-Eve Carrier, Sabine Berthier, Mandana Nikpour, Alexandra Albert, Luc Mouthon, Alessandra Bruns, Claire Fedoruk, John Varga, Linda Kwakkenbos, Vincent Poindron, Brooke Levis, Shervin Assassi, Amanda Wurz, Benjamin Crichi, Daphna Harel, Suzanne Kafaja, Esther Rodriguez, Nancy Maltez, Vincent Sobanski, Catarina Leite, Marc André, François Maurier, Ghassan El-Baalbaki, Lisa R. Jewett, Nora Østbø, Marc Lambert, Michelle Richard, James V. Dunne, Niall Jones, Robyn T. Domsic, Kimberly A. Turner, Chase Correia, Joep Welling, Nicole Culos-Reed, Benjamin Chaigne, Kim Fligelstone, Tatiana Sofia Rodriguez-Reyna, Paul R. Fortin, Bertrand Dunogue, Virginia D. Steen, Warren R. Nielson, Ward van Breda, Arsene Mekinian, Nader Khalidi, Brigitte Granel-Rey, David Launay, Pamela Piotrowski, Alexis Régent, Genevieve Gyger, Robert Riggs, Lydia Tao, and Organizational Psychology
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medicine.medical_specialty ,Medicine (General) ,Medicine (miscellaneous) ,law.invention ,Scleroderma ,Experimental Psychopathology and Treatment ,Study Protocol ,03 medical and health sciences ,All institutes and research themes of the Radboud University Medical Center ,0302 clinical medicine ,R5-920 ,Randomized controlled trial ,law ,Patient-Centered Care ,Intervention (counseling) ,Self-management ,Humans ,Medicine ,Pharmacology (medical) ,030212 general & internal medicine ,Disease management (health) ,Randomized Controlled Trials as Topic ,030203 arthritis & rheumatology ,Self-efficacy ,Protocol (science) ,Scleroderma, Systemic ,business.industry ,COVID-19 ,Patient activation ,Women's cancers Radboud Institute for Health Sciences [Radboudumc 17] ,3. Good health ,Cohort ,e-Health ,Physical therapy ,Feasibility Studies ,Anxiety ,Systemic sclerosis ,medicine.symptom ,business - Abstract
Background Systemic sclerosis (scleroderma; SSc) is a rare autoimmune connective tissue disease. We completed an initial feasibility trial of an online self-administered version of the Scleroderma Patient-centered Intervention Network Self-Management (SPIN-SELF) Program using the cohort multiple randomized controlled trial (RCT) design. Due to low intervention offer uptake, we will conduct a new feasibility trial with progression to full-scale trial, using a two-arm parallel, partially nested RCT design. The SPIN-SELF Program has also been revised to include facilitator-led videoconference group sessions in addition to online material. We will test the group-based intervention delivery format, then evaluate the effect of the SPIN-SELF Program on disease management self-efficacy (primary) and patient activation, social appearance anxiety, and functional health outcomes (secondary). Methods This study is a feasibility trial with progression to full-scale RCT, pending meeting pre-defined criteria, of the SPIN-SELF Program. Participants will be recruited from the ongoing SPIN Cohort (http://www.spinsclero.com/en/cohort) and via social media and partner patient organizations. Eligible participants must have SSc and low to moderate disease management self-efficacy (Self-Efficacy for Managing Chronic Disease (SEMCD) Scale score ≤ 7.0). Participants will be randomized (1:1 allocation) to the group-based SPIN-SELF Program or usual care for 3 months. The primary outcome in the full-scale trial will be disease management self-efficacy based on SEMCD Scale scores at 3 months post-randomization. Secondary outcomes include SEMCD scores 6 months post-randomization plus patient activation, social appearance anxiety, and functional health outcomes at 3 and 6 months post-randomization. We will include 40 participants to assess feasibility. At the end of the feasibility portion, stoppage criteria will be used to determine if the trial procedures or SPIN-SELF Program need important modifications, thereby requiring a re-set for the full-scale trial. Otherwise, the full-scale RCT will proceed, and outcome data from the feasibility portion will be utilized in the full-scale trial. In the full-scale RCT, 524 participants will be recruited. Discussion The SPIN-SELF Program may improve disease management self-efficacy, patient activation, social appearance anxiety, and functional health outcomes in people with SSc. SPIN works with partner patient organizations around the world to disseminate its programs free-of-charge. Trial registration ClinicalTrials.govNCT04246528. Registered on 27 January 2020
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- 2021
23. IgA vasculitis in patients with inflammatory bowel disease: new insights into the role of TNF-α blockers
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Cécile Audrey Durel, Guillaume Moulis, Mathurin Fumery, Vered Abitbol, Stéphane Nancey, Groupe français d’étude des vascularites, Marie-Christine Martinez Vinson, Khalil El Karoui, Viviane Queyrel-Moranne, Christian Agard, Alexis Régent, Stéphane Koch, Benjamin Terrier, Didier Ducloux, Anne-Gaëlle Kervegant, Laurent Peyrin Biroulet, David Laharie, Anne Bourrier, Michael T. Collins, Lucine Vuitton, Cédric Rafat, Loïc Guillevin, Romain Paule, Mickaela Voicu, Camille Rasmussen, François Aubin, François Maurier, Caroline Morbieu, Nizar Joher, Tali Anne Szwebel, and Bénédicte Pigneur
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Vasculitis ,medicine.medical_specialty ,IgA Vasculitis ,Cyclophosphamide ,Antineoplastic Agents ,Gastroenterology ,Inflammatory bowel disease ,Rheumatology ,Recurrence ,Internal medicine ,medicine ,Humans ,Pharmacology (medical) ,Retrospective Studies ,Crohn's disease ,Tumor Necrosis Factor-alpha ,business.industry ,Inflammatory Bowel Diseases ,medicine.disease ,Ulcerative colitis ,Immunoglobulin A ,Discontinuation ,IgA vasculitis ,Tumor Necrosis Factor Inhibitors ,Complication ,business ,medicine.drug - Abstract
Objective The association of IgA vasculitis (IgAV) and IBD is rarely described, mainly during anti-TNF-α therapy. We aimed to describe the association of IgAV and IBD. Methods We retrospectively analysed the association of IgAV and IBD through the implication of the GETAID and FVSG networks. Characteristics of IBD and IgAV were collected using a standardized case report form. Results Forty-three cases were included. IBD [mainly Crohn’s disease (CD) in 58%] preceded IgAV in 38 (88%), with median interval of 9.2 (IQR 5.4–15.4) years. In these 38 patients, at IgAV diagnosis, five (13%) had active IBD and 28 (74%) were treated with anti-TNF-α for a median duration of 31.5 (IQR 19–56) months. Main IgAV manifestations were purpura all patients (100%), joints in 20/35 (57%), renal in 15/35 (43%) and gastrointestinal in 11/35 (31%) involvement. IgAV was treated with glucocorticoids in 25 (66%), colchicine in six (16%), CYC in six (16%) and anti-TNF-α were discontinued in 15/28 (54%). No IgAV relapse occurred when TNF-α blockers were stopped, vs 23% in patients pursuing it. Conversely, five (33%) had IBD flare or complication after anti-TNF-α cessation vs one (8%) in those continuing biologics. Anti-TNF-α were resumed in six (40%), with subsequent IgAV relapse in four (67%). Conclusions This large cohort suggests that TNF-α blockers may promote the onset of IgAV in IBD. Discontinuation of anti-TNF-α was associated with vasculitis remission but increased risk of IBD relapses, whereas continuation of anti-TNF-α was associated with IBD remission but vasculitis relapse.
- Published
- 2021
24. Semi-Quantitative [
- Author
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Olivier, Espitia, Jérémy, Schanus, Christian, Agard, Françoise, Kraeber-Bodéré, Alexis F, Guédon, Antoine, Bénichou, Jean-Michel, Serfaty, Sandrine, Coudol, Matilde, Karakachoff, and Bastien, Jamet
- Abstract
[
- Published
- 2022
25. Antiphospholipid syndrome in patients over 65 years: A comparative study of clinical and biological features and thrombotic relapses
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Colombe Masson, Thi T. An Nguyen, Virginie Dufrost, Marie Audrain, Caroline Hémont, Christian Agard, Mathieu Artifoni, Jérôme Connault, Marc Fouassier, Mohamed Hamidou, Alexis F Guedon, Denis Wahl, Stéphane Zuily, and Olivier Espitia
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Male ,Venous Thrombosis ,Rheumatology ,Immunoglobulin M ,Recurrence ,Antibodies, Anticardiolipin ,Lupus Coagulation Inhibitor ,Humans ,Lupus Erythematosus, Systemic ,Female ,Thrombosis ,Antiphospholipid Syndrome ,Aged - Abstract
Objective The aim of the study was to describe clinical and biological characteristics and thrombotic relapses of patients diagnosed with antiphospholipid syndrome (APS) after the age of 65 years, in comparison with patients diagnosed with APS before 65. Methods This retrospective multicenter study was performed to 2005 from 2017 and included patients diagnosed with APS after the age of 65 years, in accordance with Sydney criteria. We compared these patients with APS patients diagnosed before the age of 65 years, and with control thrombotic patients older than 65 years. Results Fifty-eight APS patients over the age of 65 years were compared to 127 APS patients aged less than 65 and to 58 controls. In elderly APS versus younger APS, there was a male predominance (58.6% vs 36.2% p = .001); myocardial infarction and lower limb deep vein thrombosis (LLDVT) were more frequent in elderly, respectively, 12.1% versus 1.6% ( p = .005), and 44.8% versus 29.9% ( p = .048). Anticardiolipin antibody (aCL) IgM was more frequently found in old patients compared to younger patients (33.9% vs 18.1%, p = .02), contrary to lupus anticoagulant (LAC) (52.8% vs 66.9%, p = .02). Older patients were more often diagnosed with single positive APS (82.8% vs 59.8% p = .002). The thrombotic relapse free survival was lower in elderly APS patients ( p = .044) compared to younger APS. Elderly APS patients had more recurrent arterial and venous thrombosis ( p = .03) and had poorer overall survival ( p = .004) than elderly controls. Conclusion In this study, APS was different in patients aged more than 65 years, with a male predominance and more myocardial infarctions and LLDVT at diagnosis. Single antiphopholipid positivity and aCL IgM were more frequent in older patients. Older patient with APS had more thrombotic recurrence during follow-up. Compared to elderly controls, elderly APS patients had more thrombosis recurrences and poorer survival.
- Published
- 2022
26. Antineutrophil Cytoplasm Antibody-Associated Vasculitides Valvular Impairment: Multicenter Retrospective Study and Systematic Review of the Literature
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Lina Jeantin, Tiphaine Lenfant, Pierre Bataille, Hubert de Boysson, Pascal Cathébras, Christian Agard, Stanislas Faguer, Vincent Poindron, Marc Ruivard, Nicolas Martin Silva, Matthieu Monge, Loic Guillevin, Xavier Puéchal, Benjamin Terrier, Agnès Dechartres, and Pierre Charles
- Subjects
Cytoplasm ,Rheumatology ,Endocarditis ,Immunology ,Granulomatosis with Polyangiitis ,Immunology and Allergy ,Humans ,Microscopic Polyangiitis ,Multicenter Studies as Topic ,Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ,Retrospective Studies ,Antibodies, Antineutrophil Cytoplasmic - Abstract
ObjectiveWhile myocardial impairment is a predictor of poor prognosis in antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV), little is known about valvular involvement. This study aims at describing the clinical presentation, management, and outcome of endocarditis associated with AAV.MethodsWe conducted a multicenter retrospective study in centers affiliated with the French Vasculitis Study Group. We included patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), or eosinophilic GPA with endocardial impairment. A systematic review was then performed through PubMed, Embase, and Cochrane Library from inception up to September 2020.ResultsThe retrospective cohort included 9 patients (82%) with GPA, 1 (9%) with MPA, and 1 (9%) with unclassified AAV. Clinical presentation included acute valvular insufficiency (n = 7, 64%), cardiac failure (n = 3, 27%), dyspnea (n = 3, 27%), and no symptoms (n = 2, 18%). The aortic valve was the most frequently affected (n = 8/10, 80%), and vegetations were noted in 4 of 10 patients (40%). Six patients (55%) underwent surgical valvular replacement. No death from endocarditis was reported. The systematic review retrieved 42 patients from 40 references: 30 (71%) had GPA, 21 (50%) presented with vegetations, the aortic valve (n = 26, 62%) was the most frequently involved. Valvular replacement was required in 20 cases (48%) and 5 patients (13%) died from the endocarditic impairment.ConclusionEndocarditis is a rare and potentially life-threatening manifestation of AAV. Acute valvular insufficiency may lead to urgent surgery. Implementing transthoracic echocardiography in standard assessment at baseline and follow-up of AAV might reduce the delay to diagnosis and allow earlier immunosuppressive treatment before surgery is needed.
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- 2022
27. Myeloproliferative neoplasms and clonal haematopoiesis in patients with giant cell arteritis: a case–control and exploratory study
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Aurélie Foucher, Pierre Duffau, L. Delaval, Olivier Kosmider, Thomas Sené, Maxime Samson, Benjamin Terrier, Hubert de Boysson, Sébastien Humbert, Chloé Friedrich, Anne Contis, Christian Agard, Anne-Sophie Alary, Alexis Régent, Claude Bachmeyer, Mathieu Puyade, Bruno Gombert, Luc Mouthon, Jean-François Viallard, Matthias Papo, Loïc Guillevin, and François-Xavier Danlos
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Male ,medicine.medical_specialty ,Giant Cell Arteritis ,Inflammation ,Gastroenterology ,Proinflammatory cytokine ,03 medical and health sciences ,0302 clinical medicine ,Rheumatology ,Internal medicine ,medicine ,Humans ,Pharmacology (medical) ,Platelet ,030212 general & internal medicine ,Myeloproliferative neoplasm ,Aged ,Retrospective Studies ,Aged, 80 and over ,Platelet Count ,Essential thrombocythemia ,business.industry ,High-Throughput Nucleotide Sequencing ,Janus Kinase 2 ,medicine.disease ,Myelodysplastic-Myeloproliferative Diseases ,Survival Analysis ,Pathophysiology ,Giant cell arteritis ,Case-Control Studies ,030220 oncology & carcinogenesis ,Cohort ,Female ,Clonal Hematopoiesis ,medicine.symptom ,business - Abstract
Objectives GCA is a large vessel vasculitis for which triggering factors remain unknown. Clonal haematopoiesis (CH) was associated with atherosclerosis through the induction of inflammation in myeloid cells, and data suggest that CH expansion and inflammation may support each other to induce a pro-inflammatory loop. Our objective was to describe the impact of JAK2p.V617F-mutated myeloproliferative neoplasms (MPNs) on GCA and to screen MPN-free patients for CH mutations. Methods We performed a retrospective case–control study comparing the characteristics of 21 GCA patients with MPN and 42 age- and gender-matched GCA patients without MPN. Also, 18 GCA patients were screened for CH through next-generation sequencing (NGS). Results The most frequent associated MPN was essential thrombocythaemia (ET; n = 11). Compared with controls, GCA patients with MPN had less-frequent cephalic symptoms (71.4 vs 97.6%; P = 0.004) and higher platelet counts at baseline [485 × 109/l (interquartile range 346–586) vs 346 (296–418); P = 0.02]. There was no difference between groups for other clinical features. Overall survival was significantly shorter in patients with MPN compared with controls [hazard ratio 8.2 (95% CI 1.2, 56.6); P = 0.03]. Finally, screening for CH using NGS in 15 GCA patients without MPN revealed CH in 33%. Conclusion GCA patients with MPN display higher platelet counts and shorter overall survival than controls. This association is not fortuitous, given the possible pathophysiological relationship between the two diseases. CH was found in one-third of GCA patients, which may be higher than the expected prevalence for a similar age, and should be confirmed in a larger cohort.
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- 2021
28. Étude monocentrique sur les médicaments pris par les patients pour le traitement de la sclérodermie systémique
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A. Renaud, Christian Agard, M. Artifoni, A. Achille, Olivier Espitia, and Cécile Durant
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030203 arthritis & rheumatology ,Gynecology ,03 medical and health sciences ,medicine.medical_specialty ,0302 clinical medicine ,business.industry ,Gastroenterology ,Internal Medicine ,medicine ,030212 general & internal medicine ,business - Abstract
Resume Introduction Le choix d’un traitement medicamenteux pour la sclerodermie systemique est oriente par des recommandations nationales et internationales. Le but principal de l’etude etait de decrire et d’analyser ces traitements chez nos patients. Nous avons egalement souhaite evaluer leur observance medicamenteuse. Methode Il s’agit d’une etude observationnelle monocentrique sur deux cohortes de patients atteints de sclerodermie systemique ; une cohorte principale comprenant des patients ambulatoires, inclus prospectivement, avec un recueil exhaustif des traitements ; et une cohorte secondaire avec recueil des donnees d’observance par autoquestionnaire. Resultats La cohorte principale a inclus 157 patients dont 31 cas de sclerodermie systemique diffuse. Un medicament vasodilatateur pour le phenomene de Raynaud etait prescrit chez 75 patients (47,9 %) et un traitement specifique de l’hypertension arterielle pulmonaire chez 10 patients (6,4 %). Les « immunomodulateurs/immunosuppresseurs » concernaient 62 patients (39,5 %), qui recevaient de la prednisone (n = 37, 23,6 %), du mycophenolate mofetil (n = 14, 8,9 %), de l’hydroxychloroquine (n = 12, 7,6 %) et de la colchicine (n = 22, 14 %). Un traitement pour l’atteinte digestive etait prescrit pour 106 patients (67,5 %) et un traitement d’une crise renale sclerodermique par inhibiteur de l’enzyme de conversion pour 6 patients (3,8 %). Parmi les 42 patients de la cohorte secondaire, 21,4 % rapportaient une bonne observance, notamment les plus âges (p = 0,045) ou ceux n’ayant pas experimente d’effets indesirables (p = 0,009). Conclusion Notre etude apporte des donnees originales de vraie vie qui illustre l’heterogeneite des habitudes de prescription dans la sclerodermie systemique. Comme cela a deja ete rapporte, l’observance medicamenteuse n’est pas satisfaisante.
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- 2021
29. Prognostic factors in giant cell arteritis associated aortitis with PET/CT and CT angiography at diagnosis
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Victor Genin, Jean-François Alexandra, Hubert de Boysson, Laurent Sailler, Maxime Samson, Brigitte Granel, Karim Sacre, Thomas Quéméneur, Clémentine Rousselin, Geoffrey Urbanski, Julie Magnant, Valérie Devauchelle-Pensec, Viviane Queyrel-Moranne, Mickaël Martin, Emmanuel Héron, Aurélie Daumas, Quentin Gomes de Pinho, Bastien Jamet, Jean-Michel Serfaty, Christian Agard, and Olivier Espitia
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Anesthesiology and Pain Medicine ,Rheumatology - Published
- 2023
30. Rheumatic involvement and bone scan features in Schnitzler syndrome: initial and follow-up data from a single-center cohort of 25 patients
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Claire Bernier, Hélène Aubert, Jean-Marie Berthelot, Antoine Néel, Christian Agard, Sébastien Barbarot, Benoit Le Goff, Mohamed Hamidou, Agathe Masseau, Françoise Kraeber-Bodéré, Christelle Darrieutort-Laffite, and Catherine Ansquer
- Subjects
Male ,medicine.medical_specialty ,lcsh:Diseases of the musculoskeletal system ,Interleukin 1 receptor antagonist ,Single Center ,Cohort Studies ,030207 dermatology & venereal diseases ,03 medical and health sciences ,0302 clinical medicine ,Schnitzler syndrome ,Musculoskeletal Pain ,Internal medicine ,medicine ,Humans ,Bone pain ,Retrospective Studies ,business.industry ,Waldenstrom macroglobulinemia ,Retrospective cohort study ,Bone scan ,medicine.disease ,Rash ,Rheumatology ,Bone lesions ,Cohort ,Female ,medicine.symptom ,lcsh:RC925-935 ,business ,030215 immunology ,Research Article ,Follow-Up Studies - Abstract
Objective To report on the characteristics and long-term course of rheumatic manifestations in Schnitzler syndrome (SchS). Methods A retrospective cohort study of patients with SchS followed between 2000 and 2020. Inclusion criteria included a diagnosis of SchS (Strasbourg criteria). All available bone scans were reviewed and scored according to the intensity and number of pathological sites. The scintigraphic score was compared with the clinical activity score, CRP level, and treatments. Results Twenty-five patients were included. Median age at diagnosis was 68 years. Eighty patients (72%) had SchS-related rheumatic pain. Most patients had a long-standing isolated rash before constitutional and/or rheumatic symptoms appeared. The monoclonal component level was usually very low (IgMκ in 22/25). Rheumatic pain predominated around the knees. Bone scans revealed abnormal tracer uptake in 15/18 (85%). The scintigraphic score correlated with clinical activity (r = 0.4, p r = 0.47, p p Conclusions Rheumatic manifestations are very prevalent in SchS. However, bone pain can be misleading and contribute to misdiagnosis. Bone scan abnormalities are very prevalent and correlate with disease activity and treatments. IL1-Ra has a dramatic and durable efficacy but may not be required in every patient early on.
- Published
- 2020
31. Use of Biologics to Treat Relapsing and/or Refractory Polyarteritis Nodosa: Data from a European Collaborative Study
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Jérome Hadjadj, Alice Canzian, Omer Karadag, Anne Contis, François Maurier, Sébastien Sanges, Silvia Sartorelli, Laure Denis, Claire de Moreuil, Cécile-Audrey Durel, Stéphane Durupt, Marie Jachiet, Diane Rouzaud, Carlo Salvarani, Roberto Padoan, Lorenzo Dagna, Fabrice Bonnet, Christian Agard, Thomas Moulinet, Marion Hermet, Raluca Sterpu, Alexandre Thibault Jacques Maria, Jérémy Keraen, Loic Guillevin, David Jayne, and Benjamin Terrier
- Subjects
Rheumatology ,Pharmacology (medical) - Abstract
Objectives To describe the effectiveness and safety of biologics for the treatment of relapsing and/or refractory polyarteritis nodosa (PAN). Methods A retrospective European collaborative study was conducted in patients with PAN who received biologics for relapsing and/or refractory disease. Results Forty-two patients with PAN received a total of 53 biologic courses, including TNF-α blockers in 15 cases, rituximab (RTX) in 18 cases, tocilizumab (TCZ) in 10 cases and other biologics in 10 cases. TNF-α blockers and TCZ were mainly used for refractory diseases whereas RTX was mainly initiated for relapsing disease. After a median follow-up of 29 (8–50) months, remission, partial response, treatment failure and treatment discontinuation due to severe adverse events occurred in, respectively, 40%, 13%, 40% and 7% of patients receiving TNF-α blockers, 50%, none, 30% and 20% of TCZ recipients, and 33%, 11%, 56% and none of the RTX recipients. No remission was noted in patients treated with other biologics. Severe adverse events were observed in 14 (28%) patients without significant differences between the three biologics, leading to early biologics discontinuation in only three cases. Conclusion These results suggest that TCZ may be effective in relapsing and/or refractory PAN. Our data warrant further study to confirm these findings.
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- 2022
32. Renal involvement in eosinophilic granulomatosis with polyangiitis (EGPA): a multicentric retrospective study of 63 biopsy-proven cases
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Marie Essig, Cécile-Audrey Durel, David Jayne, Christelle Barbet, Sandrine Hirschi-Santelmo, Thomas Le Gallou, Antoine Bardy, Jean-Jacques Boffa, Sylvain Marchand-Adam, Dimitri Titeca-Beauport, Grégory Pugnet, Xavier Belenfant, Camille Taillé, Xavier Puéchal, Cédric Rafat, Pascal Godmer, Vincent Cottin, Vítor Teixeira, Alexandre Karras, Julien Bouet, Renato Alberto Sinico, Jacques Gaultier, Philippe Guilpain, Daniel Engelbert Blockmans, Yoann Crabol, Christian Agard, Christophe Deligny, Durel, C, Sinico, R, Teixeira, V, Jayne, D, Belenfant, X, Marchand-Adam, S, Pugnet, G, Gaultier, J, Le Gallou, T, Titeca-Beauport, D, Agard, C, Barbet, C, Bardy, A, Blockmans, D, Boffa, J, Bouet, J, Cottin, V, Crabol, Y, Deligny, C, Essig, M, Godmer, P, Guilpain, P, Hirschi-Santelmo, S, Rafat, C, Puéchal, X, Taillé, C, and Karras, A
- Subjects
Adult ,Male ,0301 basic medicine ,medicine.medical_specialty ,Adolescent ,030232 urology & nephrology ,Churg-Strauss Syndrome ,Kidney ,Gastroenterology ,Nephropathy ,Young Adult ,03 medical and health sciences ,0302 clinical medicine ,renal biopsy ,Rheumatology ,Membranous nephropathy ,Internal medicine ,Eosinophilic ,medicine ,Humans ,Pharmacology (medical) ,Kidney transplantation ,Aged ,Retrospective Studies ,Aged, 80 and over ,medicine.diagnostic_test ,business.industry ,Acute Kidney Injury ,Middle Aged ,medicine.disease ,renal involvement ,EGPA ,030104 developmental biology ,medicine.anatomical_structure ,vasculiti ,glomerulonephriti ,Female ,Renal biopsy ,Granulomatosis with polyangiitis ,business ,Vasculitis - Abstract
Objective Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small-vessel vasculitis characterized by asthma, hypereosinophilia and ANCA positivity in 40% of patients. Renal involvement is rare and poorly described, leading to this renal biopsy-proven based study in a large EGPA cohort. Methods We conducted a retrospective multicentre study including patients fulfilling the 1990 ACR criteria and/or the 2012 revised Chapel Hill Consensus Conference criteria for EGPA and/or the modified criteria of the MIRRA trial, with biopsy-proven nephropathy. Results Sixty-three patients [27 women, median age 60 years (18–83)] were included. Renal disease was present at vasculitis diagnosis in 54 patients (86%). ANCA were positive in 53 cases (84%) with anti-MPO specificity in 44 (83%). All patients had late-onset asthma. Peripheral neuropathy was present in 29 cases (46%), alveolar haemorrhage in 10 (16%). The most common renal presentation was acute renal failure (75%). Renal biopsy revealed pauci-immune necrotizing GN in 49 cases (78%). Membranous nephropathy (10%) and membranoproliferative GN (3%) were mostly observed in ANCA-negative patients. Pure acute interstitial nephritis was found in six cases (10%); important interstitial inflammation was observed in 28 (44%). All patients received steroids with adjunctive immunosuppression in 54 cases (86%). After a median follow-up of 51 months (1–296), 58 patients (92%) were alive, nine (14%) were on chronic dialysis and two (3%) had undergone kidney transplantation. Conclusion Necrotizing pauci-immune GN is the most common renal presentation in ANCA-positive EGPA. ANCA-negative patients had frequent atypical renal presentation with other glomerulopathies such as membranous nephropathy. An important eosinophilic interstitial infiltration was observed in almost 50% of cases.
- Published
- 2020
33. Muscle biopsy in anti-neutrophil cytoplasmic antibody–associated vasculitis: diagnostic yield depends on anti-neutrophil cytoplasmic antibody type, sex and neutrophil count
- Author
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Christian Agard, Mohamed Hamidou, Maxime Leroy, Julie Graveleau, Alexandra Espitia-Thibault, Nowenn Le Lan, Claire Toquet, Antoine Néel, Mathieu Lacou, Agathe Masseau, Jean-Marie Mussini, and Christelle Volteau
- Subjects
Male ,0301 basic medicine ,medicine.medical_specialty ,Neutrophils ,Biopsy ,Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ,Gastroenterology ,Antibodies, Antineutrophil Cytoplasmic ,Leukocyte Count ,03 medical and health sciences ,Sex Factors ,0302 clinical medicine ,Rheumatology ,Recurrence ,Internal medicine ,medicine ,Humans ,Pharmacology (medical) ,Muscle, Skeletal ,Survival analysis ,Aged ,Retrospective Studies ,Anti-neutrophil cytoplasmic antibody ,030203 arthritis & rheumatology ,medicine.diagnostic_test ,business.industry ,Incidence ,Middle Aged ,Prognosis ,medicine.disease ,030104 developmental biology ,Absolute neutrophil count ,Female ,France ,Renal biopsy ,Granulomatosis with polyangiitis ,Microscopic polyangiitis ,Vasculitis ,business ,Algorithms - Abstract
Objectives This study aimed to examine the sensitivity of muscle biopsy (MB) in ANCA-associated vasculitis (AAV), identify factors predicting MB positivity and assess the prognostic value of a positive MB. Methods We conducted a single-centre retrospective study of AAV with an MB performed at diagnosis. AAV classification [granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA)] followed the European Medicines Agency algorithm. A logistic regression model was used to identify the factors associated with MB positivity. Survival curves were generated using the Kaplan–Meier method. Results Among 276 AAV patients (1995–2018), 101 had an MB. Seventy-eight patients were included: 33 with GPA, 25 with MPA and 20 with EGPA. MB samples were positive in 45 cases (58%): 17 GPA, 16 MPA and 12 EGPA. Univariate analysis focussed on GPA and MPA, revealed that the MB yield was higher in females [22/31 (71%) vs 11/27 (41%); P = 0.02] and in anti-MPO patients [25/37 (68%) vs 6/19 (32%) for anti-PR3; P = 0.01]. By multivariate analysis, three factors predicted MB positivity: anti-MPO ANCA [odds ratio (OR) 10.67 (CI 2.09, 81.68)], female sex [OR 5.3 (CI 1.16, 32.35)] and neutrophil count [OR 1.33 (CI 1.07, 1.8)]. MB positivity had no impact on relapse, death or end-stage renal disease–free survival. Conclusions MB is a safe and efficient diagnostic tool for AAV. Predictors of MB yield include ANCA type, sex and neutrophil count. MB cannot substitute for kidney biopsy when indicated, but should be considered in other cases.
- Published
- 2020
34. Long-Term Rituximab Use to Maintain Remission of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis
- Author
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Alexandre Karras, Stéphane Vinzio, Catherine Hanrotel-Saliou, Grégory Pugnet, Benjamin Terrier, Nadine Meaux-Ruault, Eric Hachulla, Antoine Huart, Elodie Perrodeau, Christian Agard, Bernard Bonnotte, Thomas Le Gallou, Philippe Ravaud, Maxime Samson, Nicolas Martin-Silva, Christine Vinter, Xavier Puéchal, Pierre Charles, Jean Sibilia, Antoine Néel, Jean-François Viallard, Loïc Guillevin, P. Gobert, François Maurier, François Lifermann, Luc Mouthon, Pascal Godmer, Pascal Cohen, and Pierre-Louis Carron
- Subjects
medicine.medical_specialty ,business.industry ,010102 general mathematics ,General Medicine ,medicine.disease ,Placebo ,01 natural sciences ,law.invention ,03 medical and health sciences ,Regimen ,0302 clinical medicine ,Randomized controlled trial ,Maintenance therapy ,law ,Internal medicine ,Internal Medicine ,medicine ,Rituximab ,030212 general & internal medicine ,0101 mathematics ,Microscopic polyangiitis ,business ,Granulomatosis with polyangiitis ,medicine.drug ,Anti-neutrophil cytoplasmic antibody - Abstract
Background Biannual rituximab infusions over 18 months effectively maintain remission after a "standard" remission induction regimen for patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Objective To evaluate the efficacy of prolonged rituximab therapy in preventing AAV relapses in patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) who have achieved complete remission after completing an 18-month maintenance regimen. Design Randomized controlled trial. (ClinicalTrials.gov: NCT02433522). Setting 39 clinical centers in France. Patients 68 patients with GPA and 29 with MPA who achieved complete remission after the first phase of maintenance therapy. Intervention Rituximab or placebo infusion every 6 months for 18 months (4 infusions). Measurements The primary end point was relapse-free survival at month 28. Relapse was defined as new or reappearing symptoms or worsening disease, with a Birmingham Vasculitis Activity Score greater than 0. Results From March 2015 to April 2016, 97 patients (mean age, 63.9 years; 35% women) were randomly assigned, 50 to the rituximab and 47 to the placebo group. Relapse-free survival estimates at month 28 were 96% (95% CI, 91% to 100%) and 74% (CI, 63% to 88%) in the rituximab and placebo groups, respectively, an absolute difference of 22% (CI, 9% to 36%) with a hazard ratio of 7.5 (CI, 1.67 to 33.7) (P = 0.008). Major relapse-free survival estimates at month 28 were 100% (CI, 93% to 100%) versus 87% (CI, 78% to 97%) (P = 0.009), respectively. At least 1 serious adverse event developed in 12 patients (24%) in the rituximab group (with 9 infectious serious adverse events occurring among 6 patients [12%]) versus 14 patients (30%) in the placebo group (with 6 infectious serious adverse events developing among 4 patients [9%]). No deaths occurred in either group. Limitation Potential selection bias based on previous rituximab response and tolerance. Conclusion Extended therapy with biannual rituximab infusions over 18 months was associated with a lower incidence of AAV relapse compared with standard maintenance therapy. Primary funding source French Ministry of Health and Hoffmann-La Roche.
- Published
- 2020
35. Reducing the initial number of rituximab maintenance-therapy infusions for ANCA-associated vasculitides: randomized-trial post-hoc analysis
- Author
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Olivier Aumaître, Xavier Puéchal, Grégory Pugnet, Pascal Godmer, François Maurier, P. Gobert, Stanislas Faguer, Mohamed Hamidou, François Lifermann, Sophie Rivière, Luc Mouthon, Noémie Jourde-Chiche, Bernard Bonnotte, Benjamin Terrier, Christian Agard, Nadine Meaux-Ruault, Loïc Guillevin, Agnès Dechartres, Antoine Huart, Nicolas Martin-Silva, Jean Sibilia, Alexandre Karras, Pierre Charles, Maxime Samson, Marie Matignon, Pascal Cohen, Jean-François Viallard, and Catherine Hanrotel-Saliou
- Subjects
medicine.medical_specialty ,Randomization ,Antigens, CD19 ,Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ,Gastroenterology ,Disease-Free Survival ,Drug Administration Schedule ,Antibodies, Antineutrophil Cytoplasmic ,Maintenance Chemotherapy ,law.invention ,03 medical and health sciences ,0302 clinical medicine ,Rheumatology ,Maintenance therapy ,Randomized controlled trial ,law ,Internal medicine ,Post-hoc analysis ,medicine ,Humans ,Pharmacology (medical) ,030212 general & internal medicine ,Survival rate ,030203 arthritis & rheumatology ,business.industry ,medicine.disease ,Antirheumatic Agents ,Rituximab ,Microscopic polyangiitis ,Granulomatosis with polyangiitis ,business ,medicine.drug - Abstract
ObjectiveThe randomized, controlled MAINRITSAN2 trial was designed to compare the capacity of an individually tailored therapy [randomization day 0 (D0)], with reinfusion only when CD19+ lymphocytes or ANCA had reappeared, or if the latter’s titre rose markedly, with that of five fixed-schedule 500-mg rituximab infusions [D0 + D14, then months (M) 6, 12 and 18] to maintain ANCA-associated vasculitis (AAV) remissions. Relapse rates did not differ at M28. This ancillary study was undertaken to evaluate the effect of omitting the D14 rituximab infusion on AAV relapse rates at M12.MethodsMAINRITSAN2 trial data were subjected to post-hoc analyses of M3, M6, M9 and M12 relapse-free survival rates in each arm as primary end points. Exploratory subgroup analyses were run according to CYC or rituximab induction and newly diagnosed or relapsing AAV.ResultsAt M3, M6, M9 and M12, respectively, among the 161 patients included, 79/80 (98.8%), 76/80 (95%), 74/80 (92.5%) and 73/80 (91.3%) from D0, and 80/81 (98.8%), 78/81 (96.3%), 76/81 (93.8%) and 76/81 (93.8%) from D0+D14 groups were alive and relapse-free. No between-group differences were observed. Results were not affected by CYC or rituximab induction, or newly diagnosed or relapsing AAV.ConclusionsWe were not able to detect a difference between the relapse-free survival rates for up to M12 for the D0 and D0+D14 rituximab-infusion groups, which could suggest that omitting the D14 rituximab remission-maintenance dose did not modify the short-term relapse-free rate. Nevertheless, results at M12 may also have been influenced by the rituximab-infusion strategies for both groups.
- Published
- 2020
36. Intravenous Immunoglobulins Tapering and Withdrawal in Systemic Capillary Leak Syndrome (Clarkson Disease)
- Author
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Quentin Moyon, Marc Pineton de Chambrun, Marie Gousseff, Alexis Mathian, Miguel Hie, Geoffrey Urbanski, Franco Verlicchi, Stanislas Faguer, Antoine Dossier, Jean-Christophe Lega, Sophie Riviere, David Saadoun, Julie Graveleau, Marie-Josée Lucchini-Lecomte, Christine Christides, Sylvie Le Moal, Béatrice Bibes, Giuseppe Malizia, Marc Ruivard, Gilles Blaison, Laurent Alric, Christian Agard, Martin Soubrier, Jean-François Viallard, Hervé Levesque, Georges-Etienne Rivard, Nathalie Tieulie, Arnaud Hot, Pierre-Yves Lovey, Thomas Hanslik, François Lhote, Vincent Eble, Jorge Álvarez Troncoso, Avinash Aujayeb, Paul Quentric, Dov Taieb, Fleur Cohen-Aubart, Marc Lambert, Zahir Amoura, CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Research Unit on Cardiovascular and Metabolic Diseases (ICAN), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Institut de Cardiométabolisme et Nutrition = Institute of Cardiometabolism and Nutrition [CHU Pitié Salpêtrière] (IHU ICAN), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Pitié-Salpêtrière [AP-HP], Centre d'Immunologie et des Maladies Infectieuses (CIMI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Centre hospitalier Bretagne Atlantique (Morbihan) (CHBA), Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Institut des Maladies Métaboliques et Casdiovasculaires (UPS/Inserm U1297 - I2MC), Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT)-Université de Toulouse (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), CIC - CHU Bichat, Institut National de la Santé et de la Recherche Médicale (INSERM), Laboratoire de Biométrie et Biologie Evolutive - UMR 5558 (LBBE), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche en Informatique et en Automatique (Inria)-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS), Hôpital Saint Eloi (CHRU Montpellier), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Immunologie - Immunopathologie - Immunothérapie [CHU Pitié Salpêtrière] (I3), CHU Charles Foix [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Centre hospitalier de Saint-Nazaire, Centre Hospitalier Henri Duffaut (Avignon), CHU Estaing [Clermont-Ferrand], CHU Clermont-Ferrand, Hôpital pasteur [Colmar], Université de Toulouse (UT), Centre hospitalier universitaire de Nantes (CHU Nantes), Hôpital Haut-Lévêque [CHU Bordeaux], CHU Bordeaux [Bordeaux], Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Centre de recherche du CHU Sainte-Justine / Research Center of the Sainte-Justine University Hospital [Montreal, Canada], Université de Montréal (UdeM)-CHU Sainte Justine [Montréal], Hôpital Pasteur [Nice] (CHU), Hôpital Edouard Herriot [CHU - HCL], Hospices Civils de Lyon (HCL), Université de Versailles Saint-Quentin-en-Yvelines - UFR Sciences de la santé Simone Veil (UVSQ Santé), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ), Service de médecine interne [CHU Ambroise Paré], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Ambroise Paré [AP-HP], Hôpital Delafontaine, Centre Hospitalier de Saint-Denis [Ile-de-France], Centre Hospitalier Eure-Seine - Hôpital d'Evreux - Vernon (Evreux), CHU Lille, SNFMI-2021, This clinical research was supported by a grant from Octapharma to the Association pour la Recherche, l'Enseignement et la Thérapeutique en Médecine Interne (ARTHEMI) but all treatment decisions, including intravenous immunoglobulin (IVIG) type, were left at physician's discretion. Conflicts of interest: M. Pineton de Chambrun was supported for this study by a grant from La Société Française Nationale de Médecine Interne (SNFMI-2021). The rest of the authors declare that they have no relevant conflicts of interest., Conflicts of interest: M. Pineton de Chambrun was supported for this study by a grant from La Société Française Nationale de Médecine Interne (SNFMI-2021). The rest of the authors declare that they have no relevant conflicts of interest., and This clinical research was supported by a grant from Octapharma to the Association pour la Recherche, l’Enseignement et la Thérapeutique en Médecine Interne (ARTHEMI) but all treatment decisions, including intravenous immunoglobulin (IVIG) type, were left at physician’s discretion.
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Adult ,Incidence ,[SDV]Life Sciences [q-bio] ,Paraproteinemias ,Immunoglobulins, Intravenous ,Tapering ,Intravenous immunoglobulins ,Middle Aged ,Withdrawal ,Systemic capillary leak syndrome ,Humans ,Immunology and Allergy ,Capillary Leak Syndrome ,Clarkson disease ,Retrospective Studies - Abstract
International audience; Background: The systemic capillary leak syndrome (SCLS), also known as Clarkson disease, is a very rare condition characterized by recurrent life-threatening episodes of vascular hyperpermeability in the presence of a monoclonal gammopathy. Extended intravenous immunoglobulin (IVIG) treatment is associated with fewer recurrences and improved survival, but the optimal treatment dosage and duration remain unknown. Objective: We aim to evaluate the safety of IVIG tapering and withdrawal in patients with SCLS. Methods: We conducted a retrospective multicenter study including all adult patients with monoclonal gammopathy–associated SCLS from the EurêClark registry who received at least 1 course of IVIG. The primary end point was overall survival according to IVIG withdrawal. Results: Fifty-nine patients of mean ± SD age 51 ± 13 years were included. Overall cumulative probabilities of 2-, 5-, 10- and 15-year survival were 100%, 85%, 72%, 44%, respectively. The IVIG was withdrawn at least once in 18 patients (31%; W+ group) and never in 41 patients (69%; W– group). Cumulative probabilities of 10-year survival in W+ versus W– groups were 50% and 83% (log rank test, P = .02), respectively. Relapse rate and the median number of relapses in the W+ versus the W– groups were 72% versus 58% (P = 0.3) and 2.5 (0.3–4) versus 1 (0–2) (P = .03), respectively. The IVIG tapering was not statistically associated with increased person-year incidence of attacks using a mixed linear model. Conclusions: The IVIG withdrawal was associated with increased mortality and higher rate of recurrence in SCLS patients. The IVIG tapering might be cautiously considered in stable SCLS patients.
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- 2022
37. Specific features to differentiate Giant cell arteritis aortitis from aortic atheroma using FDG-PET/CT
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Jérémy Schanus, J.M. Serfaty, Christian Agard, Olivier Espitia, Bastien Jamet, Jeanne Hersant, Françoise Kraeber-Bodéré, Centre hospitalier universitaire de Nantes (CHU Nantes), Université de Nantes (UN), Nuclear Oncology (CRCINA-ÉQUIPE 13), Centre de Recherche en Cancérologie et Immunologie Nantes-Angers (CRCINA), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN)-Centre hospitalier universitaire de Nantes (CHU Nantes)-Centre National de la Recherche Scientifique (CNRS)-Université d'Angers (UA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN)-Centre hospitalier universitaire de Nantes (CHU Nantes)-Centre National de la Recherche Scientifique (CNRS)-Université d'Angers (UA), Université d'Angers (UA)-Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Centre hospitalier universitaire de Nantes (CHU Nantes)-Université d'Angers (UA)-Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Centre hospitalier universitaire de Nantes (CHU Nantes), and Bernardo, Elizabeth
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Male ,medicine.medical_specialty ,Science ,Giant Cell Arteritis ,Aorta, Thoracic ,[SDV.CAN]Life Sciences [q-bio]/Cancer ,Standardized uptake value ,Article ,030218 nuclear medicine & medical imaging ,03 medical and health sciences ,Rheumatic diseases ,0302 clinical medicine ,[SDV.CAN] Life Sciences [q-bio]/Cancer ,Fluorodeoxyglucose F18 ,Positron Emission Tomography Computed Tomography ,Large vessel vasculitis ,medicine.artery ,medicine ,Humans ,cardiovascular diseases ,Arteritis ,neoplasms ,Aortitis ,Aged ,Aorta ,Multidisciplinary ,medicine.diagnostic_test ,business.industry ,Diagnostic markers ,Middle Aged ,medicine.disease ,Plaque, Atherosclerotic ,3. Good health ,carbohydrates (lipids) ,Giant cell arteritis ,Atheroma ,Positron emission tomography ,030220 oncology & carcinogenesis ,cardiovascular system ,Medicine ,Female ,Radiology ,business - Abstract
Aortic wall 18F-fluorodeoxyglucose (FDG)-uptake does not allow differentiation of aortitis from atheroma, which is problematic in clinical practice for diagnosing large vessel vasculitis giant-cell arteritis (GCA) in elderly patients. The purpose of this study was to compare the FDG uptake characteristics of GCA aortitis and aortic atheroma using positron emission tomography/FDG computed tomography (FDG-PET/CT). This study compared FDG aortic uptake between patients with GCA aortitis and patients with aortic atheroma; previously defined by contrast enhanced CT. Visual grading according to standardized FDG-PET/CT interpretation criteria and semi-quantitative analyses (maximum standardized uptake value (SUVmax), delta SUV (∆SUV), target to background ratios (TBR)) of FDG aortic uptake were conducted. The aorta was divided into 5 segments for analysis. 29 GCA aortitis and 66 aortic atheroma patients were included. A grade 3 FDG uptake of the aortic wall was identified for 23 (79.3%) GCA aortitis patients and none in the atheroma patient group (p max, ∆SUV and TBRs) were significantly higher in the aortitis group. ∆SUV was the feature with the largest differential between aortitis and aortic atheroma. In this study, GCA aortitis could be distinguished from an aortic atheroma by the presence of an aortic wall FDG uptake grade 3, an FDG uptake of the 5 aortic segments, and FDG uptake of the peripheral arteries.
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- 2021
38. The consequences of COVID-19 pandemic on patients with monoclonal gammopathy-associated systemic capillary leak syndrome (Clarkson disease)
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Marc Pineton de Chambrun, Quentin Moyon, Stanislas Faguer, Geoffrey Urbanski, Alexis Mathian, Noémie Zucman, Marie Werner, Charles-Edouard Luyt, Franco Verlicchi, Zahir Amoura, Marie Gousseff, Wladimir Mauhin, Arnaud Hot, Jean-Christophe Lega, Marc Lambert, Sophie Riviere, Antoine Dossier, Marc Ruivard, François Lhote, Gilles Blaison, Sybille Merceron, Nathalie Zapella, Laurent Alric, Christian Agard, Mathieu Lacout, David Saadoun, Julie Graveleau, Martin Soubrier, Julien Haroche, Julien Boileau, Marie-Josee Lucchini-Lecomte, Thomas Hanslik, Christine Christides, Hervé Levesque, Aline Talasczka, Caroline Bulte, Eric Hachulla, Olivier Decaux, Romain Sonneville, Florent Ibouanga, Bertrand Arnulf, Marcel Benedit, Jean François Viallard, Nathalie Tieulie, Fadi Haddad, Bruno Moulin, Fleur Cohen-Aubert, Pierre-Yves Lovey, Sylvie le Moal, Béatrice Bibes, Georges-Etienne Rivard, Eric Rondeau, Giuseppe Malizia, Philippe Debourdeau, Pierre Abgueguen, Annick Bosseray, Jérôme Devaquet, Claire Presne, François Liferman, Nicolas Limal, Laurent Argaud, Romain Hernu, Sylvie de la Salle, Jorge Álvarez Troncoso, John Harty, Pascal Godmer, Miguel Hie, Thomas Papo, and Pierre-Yves Hatron
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SARS-CoV-2 ,systemic capillary-leak syndrome ,Paraproteinemias ,Immunology and Allergy ,COVID-19 ,Humans ,Clinical Communications ,Clarkson’s disease ,vaccination ,Pandemics ,Capillary Leak Syndrome - Published
- 2021
39. Large-vessel involvement is predictive of multiple relapses in giant cell arteritis
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Mohamed Hamidou, Christian Agard, Alexandra Espitia-Thibault, Jean-Benoit Hardouin, Mathieu Lacou, Olivier Espitia, Donatienne de Mornac, Aurélie Achille, Agathe Masseau, Julie Graveleau, F. Perrin, Jérôme Connault, Anaïs Wahbi, Mathieu Artifoni, Pierre Pottier, Antoine Néel, and Cécile Durant
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medicine.medical_specialty ,large-vessel imaging ,Large vessel ,Diseases of the musculoskeletal system ,Gastroenterology ,03 medical and health sciences ,0302 clinical medicine ,Rheumatology ,Internal medicine ,medicine ,protective factors ,risk factors ,Orthopedics and Sports Medicine ,In patient ,030212 general & internal medicine ,Relapse risk ,Original Research ,030203 arthritis & rheumatology ,relapse ,business.industry ,giant cell arteritis ,medicine.disease ,Giant cell arteritis ,RC925-935 ,large-vessel involvement ,business ,Systemic vasculitis - Abstract
Background: Giant cell arteritis (GCA) is the most common systemic vasculitis. Relapses are frequent. The aim of this study was to identify relapse risk factors in patients with GCA with complete large-vessel imaging at diagnosis. Methods: Patients with GCA followed in our institution between April 1998 and April 2018 were included retrospectively. We included only patients who had undergone large vascular imaging investigations at diagnosis by computed tomography (CT)-scan and/or positron emission tomography (PET)-scan and/or angio-magnetic resonance imaging (MRI). Clinical, biological, and radiological data were collected. Relapse was defined as the reappearance of GCA symptoms, with concomitant increase in inflammatory markers, requiring treatment adjustment. Relapsing patients (R) and non-relapsing patients (NR) were compared. Relapse and multiple relapses (>2) risk factors were identified in multivariable Cox analyses. Results: This study included 254 patients (73.2% women), with a median age of 72 years at diagnosis and a median follow up of 32.5 months. At diagnosis, 160 patients (63%) had an inflammatory large-vessel involvement on imaging, 46.1% (117 patients) relapsed at least once, and 21.3% (54 patients) had multiple relapses. The median delay of first relapse after diagnosis was 9 months. The second relapse delay was 21.5 months. NR patients had more stroke at diagnosis than R ( p = 0.03) and the brachiocephalic trunk was involved more frequently on CT-scan ( p = 0.046), as carotids ( p = 0.02) in R patients. Multivariate Cox model identified male gender [hazard ratio (HR): 0.51, confidence interval (CI) (0.27–0.96), p = 0.04] as a relapse protective factor, and peripheral musculoskeletal manifestations [HR: 1.74 (1.03–2.94), p = 0.004] as a relapse risk factor. Peripheral musculoskeletal manifestations [HR: 2.78 (1.23–6.28), p = 0.014], negative temporal artery biopsy [HR: 2.29 (1.18–4.45), p = 0.015], large-vessel involvement like upper limb ischemia [HR: 8.84 (2.48–31.56), p = 0.001] and inflammation of arm arteries on CT-scan [HR: 2.39 (1.02–5.58), p = 0.04] at diagnosis were risk factors of multiple relapses. Conclusion: Male gender was a protective factor for GCA relapse and peripheral musculoskeletal manifestations appeared as a relapsing risk factor. Moreover, this study identified a particular clinical phenotype of multi-relapsing patients with GCA, characterized by peripheral musculoskeletal manifestations, negative temporal artery biopsy, and large-vessel involvement with upper limb ischemia or inflammation of arm arteries. Plain language Summary At giant cell arteritis diagnosis, large-vessel inflammatory involvement is predictive of multiple relapses 46.1% of patients with GCA relapse, and 21.3% undergo multiple relapses; Male gender appears as a protective factor for relapsing in GCA; Peripheral musculoskeletal manifestations are a relapse and multiple relapses risk factor; A negative temporal artery biopsy is predictive of multiple relapses; Large-vessel involvement is predictive of multiple relapses.
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- 2021
40. Aortite de l’artérite à cellules géantes : diagnostic, pronostic et traitement
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Christian Agard, Guillaume Bonnard, Antoine Enfrein, and Olivier Espitia
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Aneurysm ,medicine.diagnostic_test ,business.industry ,Guideline adherence ,medicine ,General Medicine ,Ultrasonography ,Nuclear medicine ,business ,medicine.disease ,Computed tomography angiography ,Positron Emission Tomography-Computed Tomography - Abstract
Points essentiels Une aortite, definie par un epaississement parietal de l’aorte, est observee chez environ 50 % des patients ayant une arterite a cellules geantes (ACG). Des lesions structurales de l’aorte, ectasie ou anevrisme, peuvent survenir, avec ou sans aspect inflammatoire de l’aorte, parfois des le diagnostic d’ACG, mais le plus souvent au cours du suivi. La mise en evidence de l’atteinte aortique, a rechercher chez tout patient des le diagnostic d’ACG, repose sur une imagerie de l’aorte, angio-TDM, angio-IRM, ou TEP-TDM. Le pronostic de l’aortite et de ses complications reste mal connu, mais la mortalite liee aux evenements graves, rupture d’anevrisme ou dissection de l’aorte, pourrait potentiellement etre reduite par un diagnostic plus precoce et une surveillance reguliere. Le traitement de l’aortite de l’ACG repose sur la corticotherapie a forte dose, avec une dose d’attaque de prednisone de 0,7 mg/kg/j selon les recommandations du Groupe d’etude francais de l’arterite a cellules geantes (GEFA). Les ectasies et anevrismes de l’aorte sont a surveiller afin de poser l’indication d’une chirurgie aortique dans les meilleures conditions possibles.
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- 2019
41. Maladies hémolytiques et thrombose veineuse : mise au point
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Christian Agard, Julie Graveleau, M. Hamidou, M. Lecouffe-Desprets, Antoine Néel, M. Artifoni, Jérôme Connault, and Pierre Pottier
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Hemolytic anemia ,medicine.medical_specialty ,business.industry ,Gastroenterology ,Disease ,medicine.disease ,Hemolysis ,Venous thrombosis ,Current practice ,Internal Medicine ,medicine ,Paroxysmal nocturnal hemoglobinuria ,Thrombotic Microangiopathies ,Intensive care medicine ,business - Abstract
Many factors can contribute to the risk of venous thrombosis observed in hemolytic diseases. Some mechanisms are related to hemolysis by itself, while others seem more specific to each disease. Despite recent advances in the quantification of this risk and in understanding its physiopathology, the association of hemolysis with venous thrombosis is often unknown. The purpose of this general review is to clarify the main pro-thrombotic mechanisms during hemolysis and to synthesize the clinical data currently available. We will focus on the main types of hemolytic pathologies encountered in current practice, namely paroxysmal nocturnal hemoglobinuria, hemoglobinopathies, auto-immune hemolytic anemia and thrombotic microangiopathies.
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- 2019
42. COVID-19 outcomes in patients with inflammatory rheumatic and musculoskeletal diseases treated with rituximab: a cohort study
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Jérôme Avouac, Elodie Drumez, Eric Hachulla, Raphaèle Seror, Sophie Georgin-Lavialle, Soumaya El Mahou, Edouard Pertuiset, Thao Pham, Hubert Marotte, Amélie Servettaz, Fanny Domont, Pascal Chazerain, Mathilde Devaux, Pascal Claudepierre, Vincent Langlois, Arsène Mekinian, Alexandre Thibault Jacques Maria, Béatrice Banneville, Bruno Fautrel, Jacques Pouchot, Thierry Thomas, René-Marc Flipo, Christophe Richez, Florence Aeschlimann, Christian Agard, Nassim Ait-Abdallah, Jean-David Albert, Didier Alcais, Jean-Sébastien Allain, Yannick Allanore, Blanca Amador-Borreiro, Zahir Amoura, Emma Andre, Anaïs Arbault, Jean-Benoît Arlet, Laurent Arnaud, Denis Arniaud, Herliette Arty-Hue, Lucie Atlan, Alexandra Audemard-Verger, Christine Audoin-Pajot, Victor Audren, Maxime Bach-Bunner, Hélène Bacquet-Deschryver, Brigitte Bader-Meunier, Nathalie Balandraud, Jean-Charles Balblanc, Stéphane Bally, Frédéric Banal, Pierre Barbery, Thomas Barnetche, Audre Barrelet, André Basch, Vincent Baumier, Guillaume Bayer, Sophie Bayle, Catherine Beauvais, Rudie Beinat, Véronique Belin, Rakiba Belkhir, Ruben Benainous, Alexandre Belot, Mohammed Benammar, Mathilde Benhamou, Ygal Benhamou, Ahmed Benmansour, Pascal Bennet, Brigitte Bernoux-Manat, Elise Berthet, Emilie Berthoux, Ewa Bertolini, Adrien Bigot, Aurélia Bisson-Vaivre, Gilles Blaison, Gilles Bolla, Olivier Bonidan, Christine Bonnet, Raphaël Borie, Marie Bossert, Laurence Boudou, Françoise Bouhour, Kévin Bouiller, Bastien Bouldoires, Karima Boussoualim, Eric Bouvard, Regine Brondino, Pierre Buchlin, Laurence Cabantous, Patrice Cacoub, Simon Cadiou, Maurizio Carteni, Aurélia Carbasse, Brice Castel, Pascal Cathebras, Hervé Caumont, Annalisa Celant, Benjamin Chaigne, Benoît Chaillous, Romuald Champy, Agnès Charcot, Pierre Charles, Isabelle Charlot-Lambrecht, Caroline Charpin, Emmanuel Chatelus, Bernard Chaudier, Pascale Chertok, Xavier Chevalier, Maxime Chevreau, Emilie Chotard, Delphine Chu Miow Lin, Gaëlle Clavel, Cyril Clavel-Osorio, Fleur Cohen, Gregory Cohen, Marie-Eve Colette-Cedoz, Nived Collercandy, Antoine Colombey, Chloé Comarmond, Bernard Combe, Céline Comparon, Elodie Constant, Pascal Coquerelle, Justine Corli, Clémence Corre, Nathalie Costedoat-Chalumeau, Marie Couret, Natacha Courvoisier, Fabienne Coury-Lucas, Cécile Coutarel, Fabrice Coutier, Richard Damade, Laurence Daver-Malaterre, Sarahe Dehimat, Michel Delahousse, Emilie Barrois-Delattre, Delphine Denarie, Camille Deprouw, Emanuelle Dernis, Alban Deroux, Renaud Desbarbieux, Elise Descamps, Chantal Deslandre, Marie Desmurs, Jacques Despaux, Marie Desplats, Frédérick Detree, Valérie Devauchelle-Pensec, Robin Dhote, Philippe Dieude, Yannick Dieudonne, Elisabeth Diot, Guillaume Direz, Djamal-Dine Djeddi, Sarah Douvier, Béatrice Drouet, Catherine Duc, Angélique Ducornet, Carine Dufauret-Lombard, Alain Duhamel, Cécile Dumaine, Anne-Elisabeth Dumel, Chantal Dumoulin-Richez, Agnès Duquesne, Géraldine Durand, Mariane Durandin-Truffinet, Pierre-Marie Duret, Maïka Duval, Mikaël Ebbo, Esther Ebstein, Andra Economu-Dubosc, Stéphanie Emilie, Romain Euvrard, Philippe Evon, Sylvie Fabre, Dorothée Fagedet, Meryem Farhat, Marion Fauconier, Jacques Fechtenbaum, Renaud Felten, Fanny Fernandes, Nicole Ferreira-Maldent, Elodie Feurer, Amandine Fichet, Françoise Flaisler, Nans Florens, Violaine Foltz, Elisabeth Fontanges, Jennifer Foret, Anne-Claire Fougerousse, Anne Fouque-Aubert, Catherine Foutrier-Morello, Hélène Francois-Pradier, Léa Frantzen, Pierre Fritz, Antoine Froissart, Jean Fulpin, Piera Fuzibet, Francis Gaches, Laurence Gagneux-Lemoussu, Mélanie Penhoat-Gahier, Joris Galland, Frédérique Gandjbakhch, Nicole Garnier, Thomas Garraud, Jean-François Garrot, Romain Gastaldi, Véronique Gaud-Listrat, Maud Gauthier-Prieur, Dana Georgescu, Nathalie Gerard, Elisabeth Gervais, Christelle Gibert, Eric Gibert, Ghislaine Gill, Jérôme Gillard, Mélanie Gilson, Pauline Gimonnet, Jeanine-Sophie Giraudet-Le Quintrec, Aude Giraud-Morelet, Baptiste Glace, Camille Glanowski, Bertrand Godeau, Bruno Gombert, Camille Gonnet-Gracia, Tiphaine Goulenok, Philippe Goupille, Olivier Gourmelen, Sophie Govindaraju-Audouard, Franck Grados, Martine Grall-Lerosey, Bruno Grardel, Anne Grasland, Gilles Grateau, Monica Groza, Constance Guillaud, Séverine Guillaume, Caroline Guillibert, Xavier Guillot, Philippe Guilpain, Aline Gury, Marie-Hélène Guyot, Cécile Hacquard-Bouder, Marie-Noelle Havard, Jean-Pierre Hellier, Pascal Hennequin, Basile Henriot, Julien Henry, Véronique Hentgen, Marion Hermet, Muriel Herasse, Julie Hernandez, Miguel Hie, Pascal Hilliquin, Olivier Hinschberger, Ambre Hittinger-Roux, Jan Holubar, Christophe Hudry, Serge Huguenel, Clara Jaccard, Jean-Michel, Jacquemier, Bénédicte Jamard, Catherine Jan, Sylvie Jean, Mathieu Jouvray, Pierre-Antoine Juge, Laurent Juillard, Denis Jullien, Anna Kabala, Abdelkrim Kabchou, Ludovic Karkowski, Françoise Karman, Farid Kemiche, Jérémy Keraen, Pierre Kieffer, Isabelle Kone-Paut, Abdeldajallil Koreichi, Marie Kostine, Sylvain La Batide Alanore, Pierre Lafforgue, Sophie Lahalle, Marc Lambert, Isabelle Lambrecht, Sylvain Lanot, Aurélia Lanteri, Jean-Paul Larbre, Augustin Latourte, Christian Lavigne, Sophie Le Guen Guegan, Guillaume, Le Guenno, Diane Leguy, Agnès Lebrun, Emmanuel Ledoult, Nathalie Legoupil, Erick Legrand, Charlotte Lejeune, Olivier Leloire, Christophe Leroux, Rémi Leroy, Marie Leroy-Gouix, Tifenn Leturcq, Amélie Leurs, Céline Leveque-Michaud, François-Xavier Limbach, Frédéric Liote, Anne Lohse, Pierre Lozac'h, Virginie Lucas, Aurélie Madelon, Nadine Magy-Bertrand, Matthieu Mahevas, Hélène Maillard, Thibault Maillet, Sandrine Malochet-Guinamand, Quentin Mangon, Sylvie Marchou-Lopez, Nathalie Margarit, Thierry Marhadour, Xavier Mariette, Claire Martin, Alexis Mathian, François Maurier, Frédéric Maury, Betty Mazet-Guillaume, Arnaud Mazouyez, Hassan Mazyad, Nadia Mehsen-Cetre, Ulrich Meinzer, Isabelle Melki, Laurent Messer, Corinne Miceli, Martin Michaud, Catherine Michel, Matthias Michel, Mathilde Michon, Anne-Marie Milesi-Lecat, Anna Molto, Marie Moly, Olivier Moranne, Gautier Morel, Hugo Morel, Jacques Morel, Franck Morin, Laurence Moulinier, Guillaume Moulis, Bertrand Moura, Minh Nguyen, Sabine Nicolas-Vullierme, Hubert Nielly, Gaétane Nocturne, Aurore Nottez, Henri-Olivier Ollagnon, Isabelle Pacaud-Vitoux, Anne Pagnier, Caroline Paris, Antoine Parrot, Tristan Pascart, Yasmina Pascaud-Mansour, Lætitia Paulin, Stephan Pavy, Laurent Perard, Yves-Marie Pers, Micheline Pha, Maud Pichon, Audrey Pierreisnard, Gabrielle Pizana, Sylvaine Poignant, Elsa Poix, Agnès Portier, Antoine Poulet, Samira Plassard, Grégory Pugnet, Déborah Puyraimond-Zemmour, Pierre Quartier-Dit-Maire, Marion Quenet, Viviane Queyrel, Loïc Raffray, Philippe Remy, Myriam Renard, Jessica Rene, Sabine Revuz, Bénédicte Rey, Gaëlle Richard-Colmant, Etienne Riviere, Sébastien Riviere, Sophie Robin, Julien Rohmer, Isabelle Roitg, Mélanie Romier, Michel Rolland, Mélanie Roriz, Carole Rosenberg, Linda Rossi, Olivier Roth, Sid-Ahmed Rouidi, Mathilde Roumier, Mickaël Rousiere, Clémentine Rousselin, Bénédicte Rouviere, Christian Roux, Fabienne Roux, Marielle Roux, Nicolas Roux, Diane Rouzaud, Sylvie Rozenberg, Isabelle Sacco, Fatiha Sadji, Laurent Sailler, Carine Salliot, Jean-Hugues Salmon, Alain Saraux, Jean Schmidt, Julie Seguier, Jérémie Sellam, Eric Senbel, Thomas Sene, Patricia Senet, Pascal Seve, Aurélie Sicaud, Perrine Smets, Vincent Sobanski, Christelle Sordet, Elisabeth Sornay-Rendu, Odile Souchaud-Debouverie, Lætitia Sparsa, Lionel Spielmann, Sarah Steib, Chloé Stavris, Catherine Straus, Victor Strotz, Paulina Szafors, Séverine Taffignon-Clave, Justine Simoens, Claire Theillac, Nora Tenenbaum, Benoît Thomachot, Nathalie Tieulie, Soizic Tiriau, Alice Tison, Eric Toussirot, Ludovic Trefond, Sophie Trijau, Sébastien Trouillier, Anne-Priscille Trouvin, Marie-Elise Truchetet, Marc Ulrich, Jacques Vaquier, Eric Veillard, Laurent Veillon, Guillaume Vial, Jean-François Viallard, Judith Victor, Claire Vidon, Mathias Vidon, Camille Vigne, Alexandre Virone, Ursula Warzocha, Daniel Wendling, Claude Werle, Cécile Wibaux, Alexandra Willems, Michel Wisniewski, Juliette Woessner, Bernadette Xerri-Campano, Service de rhumatologie [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Evaluation des technologies de santé et des pratiques médicales - ULR 2694 (METRICS), Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de médecine interne [Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Institute for Translational Research in Inflammation - U 1286 (INFINITE (Ex-Liric)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Immunologie des Maladies Virales et Autoimmunes (IMVA - U1184), Université Paris-Sud - Paris 11 (UP11)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre Hospitalier Tourcoing, Centre Hospitalier René Dubos [Pontoise], Assistance Publique - Hôpitaux de Marseille (APHM), Santé Ingénierie Biologie Saint-Etienne (SAINBIOSE), Centre Ingénierie et Santé (CIS-ENSMSE), École des Mines de Saint-Étienne (Mines Saint-Étienne MSE), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT)-École des Mines de Saint-Étienne (Mines Saint-Étienne MSE), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Biologie Intégrative du Tissu Osseux (LBTO), Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre d'Investigation Clinique - Epidémiologie Clinique Saint-Etienne (CIC-EC), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de Reims (CHU Reims), CHU Pitié-Salpêtrière [AP-HP], Groupe Hospitalier Diaconesses Croix Saint-Simon, Centre hospitalier intercommunal de Poissy/Saint-Germain-en-Laye - CHIPS [Poissy], Epidemiology in Dermatology and Evaluation in Therapeutics (EpiDermE), Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), CHU Saint-Antoine [AP-HP], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Cellules Souches, Plasticité Cellulaire, Médecine Régénératrice et Immunothérapies (IRMB), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), CHU Bordeaux [Bordeaux], Composantes innées de la réponse immunitaire et différenciation (CIRID), Université Bordeaux Segalen - Bordeaux 2-Centre National de la Recherche Scientifique (CNRS), Laboratoire Chrono-environnement (UMR 6249) (LCE), Centre National de la Recherche Scientifique (CNRS)-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Maladies génétiques d'expression pédiatrique (U933), Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de Médecine Interne = Hôpital de jour de médecine [CHU Tenon], INSERM U1059, SAINBIOSE - Santé, Ingénierie, Biologie, Saint-Etienne (SAINBIOSE-ENSMSE), Institut Mines-Télécom [Paris] (IMT)-Institut Mines-Télécom [Paris] (IMT)-Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Biologie intégrative du tissu osseux, Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM), CIC Saint Etienne, Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne)-Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hôpital Nord (Saint Etienne), centre hospitalier intercommunal de Poissy/Saint-Germain-en-Laye - CHIPS [Poissy], Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne), Laboratoire Chrono-environnement - CNRS - UBFC (UMR 6249) (LCE), Service de rhumatologie, Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre, Service de Médecine Interne [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de rhumatologie [CH Gustave Dron, Tourcoing], Centre Hospitalier Gustave Dron [Tourcoing], Hopital Réné Dubos, Université Paris 1 Panthéon-Sorbonne - UFR Science Politique (UP1 UFR11), and Université Paris 1 Panthéon-Sorbonne (UP1)
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medicine.medical_specialty ,Immunology ,law.invention ,03 medical and health sciences ,0302 clinical medicine ,Rheumatology ,immune system diseases ,law ,hemic and lymphatic diseases ,Internal medicine ,Clinical endpoint ,Immunology and Allergy ,Medicine ,030212 general & internal medicine ,030203 arthritis & rheumatology ,business.industry ,Hazard ratio ,Odds ratio ,Articles ,medicine.disease ,Intensive care unit ,3. Good health ,[SDV.MP]Life Sciences [q-bio]/Microbiology and Parasitology ,Rheumatoid arthritis ,Cohort ,Rituximab ,business ,[SDV.MHEP]Life Sciences [q-bio]/Human health and pathology ,Cohort study ,medicine.drug - Abstract
Summary Background Various observations have suggested that the course of COVID-19 might be less favourable in patients with inflammatory rheumatic and musculoskeletal diseases receiving rituximab compared with those not receiving rituximab. We aimed to investigate whether treatment with rituximab is associated with severe COVID-19 outcomes in patients with inflammatory rheumatic and musculoskeletal diseases. Methods In this cohort study, we analysed data from the French RMD COVID-19 cohort, which included patients aged 18 years or older with inflammatory rheumatic and musculoskeletal diseases and highly suspected or confirmed COVID-19. The primary endpoint was the severity of COVID-19 in patients treated with rituximab (rituximab group) compared with patients who did not receive rituximab (no rituximab group). Severe disease was defined as that requiring admission to an intensive care unit or leading to death. Secondary objectives were to analyse deaths and duration of hospital stay. The inverse probability of treatment weighting propensity score method was used to adjust for potential confounding factors (age, sex, arterial hypertension, diabetes, smoking status, body-mass index, interstitial lung disease, cardiovascular diseases, cancer, corticosteroid use, chronic renal failure, and the underlying disease [rheumatoid arthritis vs others]). Odds ratios and hazard ratios and their 95% CIs were calculated as effect size, by dividing the two population mean differences by their SD. This study is registered with ClinicalTrials.gov, NCT04353609. Findings Between April 15, 2020, and Nov 20, 2020, data were collected for 1090 patients (mean age 55·2 years [SD 16·4]); 734 (67%) were female and 356 (33%) were male. Of the 1090 patients, 137 (13%) developed severe COVID-19 and 89 (8%) died. After adjusting for potential confounding factors, severe disease was observed more frequently (effect size 3·26, 95% CI 1·66–6·40, p=0·0006) and the duration of hospital stay was markedly longer (0·62, 0·46–0·85, p=0·0024) in the 63 patients in the rituximab group than in the 1027 patients in the no rituximab group. 13 (21%) of 63 patients in the rituximab group died compared with 76 (7%) of 1027 patients in the no rituximab group, but the adjusted risk of death was not significantly increased in the rituximab group (effect size 1·32, 95% CI 0·55–3·19, p=0·53). Interpretation Rituximab therapy is associated with more severe COVID-19. Rituximab will have to be prescribed with particular caution in patients with inflammatory rheumatic and musculoskeletal diseases. Funding None.
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- 2021
43. Thoracic lymphadenopathies in diffuse systemic sclerosis: an observational study on 48 patients using computed tomography
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Arthur Renaud, Raphael Pautre, Olivier Morla, Aurélie Achille, Cécile Durant, Olivier Espitia, Eric Frampas, and Christian Agard
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Pulmonary and Respiratory Medicine ,Adult ,Male ,Scleroderma, Systemic ,RC705-779 ,Research ,digestive, oral, and skin physiology ,Lymphadenopathy ,Interstitial lung disease ,Middle Aged ,Thorax ,Thoracic lymphadenopathies ,Scleroderma ,Diseases of the respiratory system ,Multidetector Computed Tomography ,Humans ,Systemic sclerosis ,Female ,France ,Lung Diseases, Interstitial ,Aged - Abstract
Background Thoracic multidetector computed tomography (MDCT) is essential for the detection of interstitial lung disease (ILD) in patients with systemic sclerosis (SSc). Thoracic MDCT assessment can reveal the presence of thoracic lymphadenopathies (LAP) whose signification remains uncertain. The purpose of the study was to describe the characteristics and to assess the significance of thoracic LAP in patients with diffuse SSc. Methods We conducted a monocentric observational study on adult patients with diffuse SSc, and collected general patient and first thoracic MDCT characteristics, PET-CT and outcome data. Comparisons were made between patients with and without thoracic LAP. Results Forty-eight patients were included. There were 30 patients (62.5%) with an ILD and 23 (48%) with at least one thoracic LAP on the first MDCT assessment. Median number per patient of thoracic LAP was 3 [1–8], with a mean size of 11.7 ± 1.7 mm, mainly located in right para-tracheal area (22.8% of the total number of LAP), right hilar area (20.3%), left hilar area (6.5%), and sub-carinal area (15.2%). PET-CT showed lymph node hypermetabolism in 11/15 patients (73.3%) with mean SUVmax at 4 ± 1.3. There were significantly more males (p = 0.002) and more patients exposed to silica (p = 0.001) in patients with thoracic LAP. ILD was significantly more extended according to Goh score (p = 0.03), and using semi-quantitative score for mixed ground-glass reticulation (p = 0.01) and global abnormalities (p = 0.03) in patients with thoracic LAP and ILD. Thirteen patients (27.1%) died during follow-up without significant difference according to the presence or not of thoracic LAP (p = 0.15). There was also no significant difference concerning immunosuppressive treatment initiation (p = 0.17). Conclusions Thoracic LAP are common in diffuse SSc and are generally multiple, not bulky, moderately hypermetabolic, and located at the base of the mediastinum lymph node chains. Their presence correlates with the extent of ILD. In absence of ILD, thoracic LAP presence seems to be often explained by silica exposure. Trial Registration: NA.
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- 2021
44. [Giant cell arteritis]
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Christian, Agard, Donatienne, de Mornac, and Olivier, Espitia
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Giant Cell Arteritis ,Humans - Published
- 2021
45. [Vascular imaging in giant cell arteritis]
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Christian, Agard, Donatienne, de Mornac, and Olivier, Espitia
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Diagnostic Imaging ,Diagnostic Tests, Routine ,Giant Cell Arteritis ,Humans - Published
- 2021
46. Pain levels and associated factors in the Scleroderma Patient-centered Intervention Network (SPIN) cohort: A multicentre cross-sectional study
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Suzanne Kafaja, Jessica K. Gordon, Nader Khalidi, Esther Rodriguez, Ariel Masetto, Anne A. Schouffoer, Nancy Maltez, Geneviève Guillot, Virginia D. Steen, Joep Welling, Tatiana Sofia Rodriguez-Reyna, Paul R. Fortin, Bertrand Dunogue, Niall Jones, Robyn T. Domsic, Eric Hachulla, Arsene Mekinian, Warren R. Nielson, Joanne Manning, Sindhu R. Johnson, Chase Correia, Evelyn Sutton, Nassim Ait Abdallah, David Robinson, Benjamin Chaigne, Susanna Proudman, Geneviève Gyger, Karen Gottesman, Mara Cañedo Ayala, Christian Agard, Susan J. Bartlett, Yvonne C. Lee, Kim Fligelstone, Carter Thorne, Mandana Nikpour, Marie Hudson, Catherine Fortune, Thierry Martin, Maggie Larché, Laura K. Hummers, Benjamin Crichi, Carolyn Ells, Sabine Berthier, Angelica Bourgeault, Marie-Nicole Discepola, Ariane L. Herrick, Tracy M. Frech, Andrea Benedetti, Brett D. Thombs, Sébastien Rivière, Marion Casadevall, Sheila Melchor, Janet E. Pope, Maria E. Suarez-Almazor, Regina Fare, Alessandra Bruns, John Varga, Vincent Poindron, Rina S. Fox, Lyne Bissonnette, Vanessa L. Malcarne, Richard S. Henry, Hélène Maillard, Maureen Sauve, Maria Martin, Lorinda Chung, Marc Lambert, Marie-Eve Carrier, Louis Olagne, Sophie Roux, Maria Gagarine, François Rannou, Elana J. Bernstein, Andrea Carboni Jiménez, Monique Hinchcliff, Amy Gietzen, Alena Ikic, Christelle Nguyen, Julia Nordlund, Brigitte Granel-Rey, Alexis Régent, Robert Riggs, David Launay, Sabrina Hoa, Michelle Richard, Robert Spiera, Isabelle Marie, James V. Dunne, Brooke Levis, Shervin Assassi, Catarina Leite, Daphna Harel, Marc André, Ghassan El-Baalbaki, François Maurier, Nora Østbø, Vincent Sobanski, Karen Nielsen, Patricia Carreira, Alexandra Albert, Luc Mouthon, Linda Kwakkenbos, Christopher Denton, Maureen D. Mayes, Pearce G. Wilcox, Dominique Farge-Bancel, and Perrine Smets
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medicine.medical_specialty ,business.industry ,Cross-sectional study ,Immunology ,Disease ,medicine.disease ,Rheumatology ,Scleroderma ,Women's cancers Radboud Institute for Health Sciences [Radboudumc 17] ,Experimental Psychopathology and Treatment ,All institutes and research themes of the Radboud University Medical Center ,Quality of life ,Rheumatoid arthritis ,Internal medicine ,Cohort ,Immunology and Allergy ,Medicine ,business ,Rheumatism - Abstract
Summary Background Pain is an important and detrimental feature of systemic sclerosis but is often overlooked or deprioritised in research and clinical care. Raynaud's phenomenon, arthritis, and cutaneous ulcers are among the commonly reported disease manifestations of systemic sclerosis that could be associated with pain. We aimed to assess levels of pain intensity and interference and to evaluate disease factors associated with pain intensity and interference. Methods In this multicentre cross-sectional study, participants from the Scleroderma Patient-centered Intervention Network cohort who completed pain intensity and interference measures (Patient Reported Outcomes Information System-29 profile, version 2·0) as part of baseline assessments were included. Patients were recruited from 46 centres in Australia, Canada, France, Mexico, Spain, the UK, and the USA between April 15, 2014, and Jan 7, 2020. Eligible patients included those aged 18 years or older who met the criteria for systemic sclerosis devised by the American College of Rheumatology and the European League Against Rheumatism. Associations of pain intensity and pain interference with systemic sclerosis-related variables and overlap syndromes, controlling for sociodemographic variables, were assessed with multiple linear regression. Continuous independent variables were standardised. Findings Among 2157 participants with systemic sclerosis (268 [12%] males and 1889 [88%] females), 1870 (87%) reported mild, moderate, or severe pain (defined as ≥1 on a 0 to 10 scale), and 815 (38%) reported moderate or severe pain (defined as ≥5). Moreover, 757 (35%) participants reported moderate or severe pain interference. Greater pain intensity was independently associated with female sex (0·58 points [95% CI 0·26–0·90]), non-White race or ethnicity (0·50 points [0·21–0·79]), fewer years in formal education (0·30 points per SD [0·19–0·41]), country (reference: USA; Canada: 0·29 points [0·01–0·57] and UK: 0·58 points [0·21–0·95]), greater body-mass index (0·35 points per SD [0·24–0·45]); joint contractures (0·67 points [0·39–0·94]), digital ulcers (0·33 points [0·10–0·55]), gastrointestinal involvement (0·66 points [0·33–0·98]), skin involvement (measured using modified Rodnan skin score; 0·22 points per SD [0·10–0·35]), rheumatoid arthritis (0·96 points [0·50–1·43]), and Sjogren's syndrome (0·42 points [0·01–0·83]). Pain interference results were similar. Interpretation Pain is common among people with systemic sclerosis. Controlling for sociodemographic variables, greater pain was associated with multiple systemic sclerosis-related manifestations, including joint contractures, digital ulcers, gastrointestinal involvement, skin involvement, and the presence of overlap syndromes. Health-care providers should work with patients to address pain, including identifying and addressing systemic sclerosis manifestations associated with their pain, and supporting behavioural approaches to minimise impact on function and quality of life. Funding Canadian Institutes of Health Research, Arthritis Society, The Lady Davis Institute for Medical Research of the Jewish General Hospital, Jewish General Hospital Foundation, McGill University, Scleroderma Society of Ontario, Scleroderma Canada, Sclerodermie Quebec, Scleroderma Manitoba, Scleroderma Atlantic, Scleroderma Association of BC, Scleroderma SASK, Scleroderma Australia, Scleroderma New South Wales, Scleroderma Victoria, and Scleroderma Queensland.
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- 2021
47. Spectrum and Outcome of Noninfectious Aortitis
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Lucie Biard, Christian Agard, Cloé Comarmond, Matthieu Resche-Rigon, Fanny Domont, Olivier Espitia, Anne Claire Desbois, Mathieu Vautier, Yasmina Ferfar, Sarah Morinet, David Saadoun, Patrice Cacoub, Immunologie - Immunopathologie - Immunothérapie [CHU Pitié Salpêtrière] (I3), CHU Charles Foix [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Épidémiologie et statistiques cliniques pour les évaluations tumorales, respiratoires et de réanimation (ECSTRRA), Centre of Research in Epidemiology and StatisticS (CRESS), Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre hospitalier universitaire de Nantes (CHU Nantes), and CACOUB, Patrice
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Male ,medicine.medical_specialty ,Multivariate analysis ,[SDV.IMM] Life Sciences [q-bio]/Immunology ,Cogan syndrome ,Giant Cell Arteritis ,Immunology ,large vessel vasculitides ,Disease ,Gastroenterology ,Rheumatology ,Internal medicine ,medicine ,Humans ,Immunology and Allergy ,Cumulative incidence ,Polychondritis, Relapsing ,Aortitis ,Relapsing polychondritis ,Retrospective Studies ,Ankylosing spondylitis ,business.industry ,medicine.disease ,Takayasu Arteritis ,noninfectious aortitis ,Giant cell arteritis ,[SDV.IMM]Life Sciences [q-bio]/Immunology ,Female ,business ,aortitis - Abstract
ObjectiveTo assess the spectrum and long-term outcome of patients with noninfectious aortitis.MethodsWe performed a retrospective multicenter study of 353 patients (median age at diagnosis was 62 [IQR 46–71] yrs and 242 [68.6%] patients were women) with noninfectious aortitis. Factors associated with vascular complications were assessed in multivariate analysis.ResultsWe included 136 patients with giant cell arteritis (GCA), 96 with Takayasu arteritis (TA), 73 with clinically isolated aortitis (CIA), and 48 with aortitis secondary to inflammatory diseases (including Behçet disease, relapsing polychondritis, IgG4-related disease, Cogan syndrome, ankylosing spondylitis). After a median follow-up of 52 months, vascular complications were observed in 32.3%, revascularizations in 30% of patients, and death in 7.6%. The 5-year cumulative incidence of vascular complications was 58% (95% CI 41–71), 20% (95% CI 13–29), and 19% (95% CI 11–28) in CIA, GCA, and TA, respectively. In multivariate analysis, male sex (HR 2.10, 95% CI 1.45–3.05, P < 0.0001) and CIA (HR 1.76, 95% CI 1.11–2.81, P = 0.02) were independently associated with vascular complications.ConclusionNoninfectious aortitis accounts for significant morbidity and mortality. CIA seems to carry the highest rate of vascular complications.
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- 2021
48. Early trajectories of skin thickening are associated with severity and mortality in systemic sclerosis
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Luc Mouthon, Jean-Christophe Lega, Marie-Elise Truchetet, Arsène Mekinian, V. Sobanski, Alain Duhamel, Yannick Allanore, Carine Boulon, Anne-Laure Fauchais, Christian Agard, Elisabeth Diot, Achille Aouba, Emmanuel Ledoult, Jean-Robert Harlé, Hélène Behal, Sabine Berthier, Eric Hachulla, Patrick Jego, Grégory Pugnet, D. Launay, Université de Lille, Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de médecine interne [Lille], Centre de référence des maladies auto-immunes systémiques rares du Nord et Nord Ouest [CHRU Lille], Hôpital Claude Huriez [Lille], CHU Lille-CHU Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Evaluation des technologies de santé et des pratiques médicales - ULR 2694 (METRICS), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Université de Lille, Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), CHU Toulouse [Toulouse], Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL), Service de médecine interne [Nantes], Université de Nantes (UN)-Hôtel-Dieu-Centre hospitalier universitaire de Nantes (CHU Nantes), Service de rhumatologie [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], CHU Pontchaillou [Rennes], Service de Médecine interne A et polyclinique médicale [CHU Limoges], CHU Limoges, Hôpital de la Timone [CHU - APHM] (TIMONE), CHU Dijon, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Service de médecine interne [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Service de Médecine Interne [CHU Saint-Antoine], CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), CHU Bordeaux [Bordeaux], Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)
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Change over time ,Adult ,Male ,Clinical heterogeneity ,medicine.medical_specialty ,lcsh:Diseases of the musculoskeletal system ,Skin thickening trajectories ,[SDV]Life Sciences [q-bio] ,Disease ,03 medical and health sciences ,0302 clinical medicine ,Interquartile range ,Internal medicine ,medicine ,Humans ,10. No inequality ,Survival rate ,030304 developmental biology ,Aged ,Skin ,030203 arthritis & rheumatology ,0303 health sciences ,Scleroderma, Systemic ,business.industry ,Proportional hazards model ,Skin thickening ,Modified Rodnan skin score ,Middle Aged ,Rheumatology ,Disease Progression ,Organ involvement ,Systemic sclerosis ,Female ,lcsh:RC925-935 ,business ,[SDV.MHEP]Life Sciences [q-bio]/Human health and pathology ,Research Article - Abstract
Background Systemic sclerosis (SSc) is a severe and highly heterogeneous disease. The modified Rodnan skin score (mRSS) is a widely used tool for the assessment of the extent and degree of skin thickness. This study aimed to identify the classes of patients with early similar skin thickening trajectories without any a priori assumptions and study their associations with organ involvement and survival. Methods From the French SSc national cohort, patients with a disease duration of less than 2 years at inclusion and with at least 2 mRSS available within the first 4 years of follow-up were enrolled. Classes of patients with similar mRSS trajectories were identified based on a latent class mixed model. The clinical characteristics and survival rate were compared between the obtained classes. Results A total of 198 patients fulfilled the inclusion criteria, with a total of 641 mRSS available. The median disease duration and follow-up were 0.8 (interquartile range 0.4; 1.2) and 6.3 (3.8; 8.9) years, respectively. Individual trajectories of mRSS were highly heterogeneous between patients. Models with 1–6 latent classes of trajectories were sequentially assessed, and the 5-class model represented the best fit to data. Each class was characterized by a unique global trajectory of mRSS. The median disease duration did not differ significantly between classes. Baseline organ involvement was more frequent in classes with significant change over time (classes 2–5) than in class 1 (low baseline mRSS without significant change over time). Using Cox regression, we observed a progressively increasing risk of death from classes 1 to 5. Conclusions Early identification of clinical phenotype based on skin thickening trajectories could predict morbi-mortality in SSc. This study suggested that mRSS trajectories characterization might be pivotal for clinical practice and future trial designs.
- Published
- 2020
49. Scalp Necrosis in Giant Cell Arteritis
- Author
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Christian Agard and Olivier Espitia
- Subjects
Pathology ,medicine.medical_specialty ,Necrosis ,business.industry ,Prednisolone ,Treatment outcome ,Giant Cell Arteritis ,General Medicine ,medicine.disease ,Giant cell arteritis ,medicine.anatomical_structure ,Treatment Outcome ,Scalp Dermatoses ,Scalp ,medicine ,Humans ,Female ,medicine.symptom ,business ,Glucocorticoids ,Aged - Published
- 2020
50. Adénopathies médiastinales dans la sclérodermie systémique diffuse : une étude observationnelle sur 48 patients
- Author
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E. Frampas, O. Morla, R. Pautre, A. Renaud, Christian Agard, Olivier Espitia, and Cécile Durant
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Gastroenterology ,Internal Medicine - Abstract
Introduction La tomodensitometrie (TDM) thoracique est un examen essentiel pour la detection d’une pneumopathie interstitielle diffuse (PID) dans le cadre de la sclerodermie systemique (ScS). Cependant, cet examen peut reveler des adenopathies mediastinales dont la signification reste incertaine dans cette maladie. Le but de cette etude etait de decrire les adenopathies mediastinales au cours de la ScS diffuse, et d’evaluer leurs determinants et impacts pronostiques. Patients et methodes Nous avons mene une etude retrospective observationnelle monocentrique sur des patients atteints de ScS de forme cutanee diffuse ayant eu au moins une TDM thoracique au cours de leur suivi. Nous avons recueilli les caracteristiques generales des patients et de la premiere TDM thoracique disponible, ainsi que les donnees de TEP-TDM et d’histologie d’adenopathie mediastinale lorsqu’elles etaient realisees. Nous avons egalement analyse la derniere TDM thoracique disponible et recueilli le devenir des patients (deces et/ou introduction d’un immunosuppresseur). Enfin, des analyses comparatives ont ete realisees entre les patients avec ou sans adenopathies mediastinales. Resultats Parmi les 48 patients inclus, 30 (62,5 %) presentaient une PID et 23 (48 %) avaient au moins une adenopathie mediastinale. Le nombre median d’adenopathies mediastinales par patient etait de 3 [1-8], avec une taille moyenne de 11,7 ± 1,7 mm, principalement situee dans la zone para-tracheale droite (22,8 % du nombre total d’adenopathies), la zone hilaire droite (20,3 %), et la zone sous-carinaire (15,2 %). La TEP-TDM montrait un hypermetabolisme des adenopathies mediastinales chez 11/15 patients (73,3 %) avec une SUVmax moyenne de 4 ± 1,3. L’analyse histologique disponible chez 5 patients n’a jamais decrit de granulome ni de cellules tumorales. Il y avait davantage d’hommes (p = 0,002) et d’exposition a la silice (p = 0,001) chez les patients ayant au moins une adenopathie mediastinale. La PID etait significativement plus etendue selon le score de Goh (p = 0,03), et en utilisant un score semi-quantitatif pour le mixte verre depoli-reticulations (p = 0,01) et les anomalies globales (p = 0,03) chez les patients ayant au moins une adenopathie mediastinale. Il n’y avait pas de difference concernant le nombre et la taille maximale des adenopathies mediastinales, ainsi que le score d’extension globale des PID entre la premiere et la derniere TDM thoracique. Dix patients (20,3 %) sont decedes et 37 patients (77,1 %) ont eu au moins un immunosuppresseur debute au cours du suivi, sans difference significative entre les patients avec ou sans adenopathies mediastinales. Conclusion Les adenopathies mediastinales sont frequentes dans la ScS diffuse et sont generalement multiples, non volumineuses, moderement hypermetaboliques, et situees a la base des chaines de ganglions lymphatiques mediastinaux. Aucun diagnostic de neoplasie ou de granulomatose n’a ete pose au cours du suivi. Bien que correlee a l’etendue de la PID, leur presence ne semble pas avoir de consequence en termes de devenir chez ces patients. En l’absence de PID, la presence d’adenopathies mediastinales semble souvent s’expliquer par l’exposition a la silice.
- Published
- 2021
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