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Start Over Author "Rimar D." Topic scleroderma, systemic
20 results on '"Rimar D."'

1. Systemic sclerosis, silica exposure and cellular therapies: The sand in the gears?

Author

Lescoat A, Rimar D, and Farge D

Subjects
Humans, Hematopoietic Stem Cell Transplantation adverse effects, Hematopoietic Stem Cell Transplantation methods, Cell- and Tissue-Based Therapy methods, Cell- and Tissue-Based Therapy adverse effects, Lung Diseases, Interstitial therapy, Lung Diseases, Interstitial etiology, Occupational Diseases therapy, Occupational Diseases etiology, Silicosis therapy, Scleroderma, Systemic therapy, Silicon Dioxide adverse effects, Occupational Exposure adverse effects
Abstract

Systemic sclerosis (SSc) is a chronic orphan autoimmune disease with the highest mortality rate among rheumatic diseases. SSc-related interstitial-lung disease (ILD) remains among the leading causes of SSc-related mortality with still few therapeutic effective strategies. In patients with crystallin silica exposure, SSc is recognized as an occupational disease according to the French social security system (Table 25A of the general insurance regimen). Lympho-ablative or myeloablative immunosuppression followed by autologous hematopoietic stem-cell transplantation (aHSCT) is the only therapeutic approach with demonstrated efficacy, improved survival with disease modifying effects on SSc-fibrotic manifestations (skin disease and ILD) and quality of life. A documented past and/or present occupational silica exposure, with extensive exposure and/or silica-related ILD and/or with persistent silica content in the broncho-alveolar lavage fluid are contra-indications to aHSCT in SSc patients, due to the risk of silica-related malignancy or of SSc relapse. This article aims to discuss alternative options in SSc patients with a history of silica exposure, and how innovative cellular therapies (mesenchymal stromal cells, CAR cells) could represent new therapeutic options for these patients., (Copyright © 2024 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Published
2024
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2. Complete resolution of gastric antral vascular ectasia after autologous haematopoietic stem cell transplantation in systemic sclerosis.

Author

Keret S, Zuckerman T, Henig I, Rainis T, Odeh S, Artoul N, Shouval A, Slobodin G, and Rimar D

Subjects
Humans, Cyclophosphamide, Transplantation, Autologous, Gastric Antral Vascular Ectasia, Scleroderma, Systemic, Hematopoietic Stem Cell Transplantation
Abstract

Competing Interests: Competing interests: None declared.

Published
2023
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3. Differentially expressed genes in systemic sclerosis: Towards predictive medicine with new molecular tools for clinicians.

Author

Keret S, Rimar D, Lansiaux P, Feldman E, Lescoat A, Milman N, and Farge D

Subjects
Humans, Precision Medicine, Fibrosis, Autoantibodies therapeutic use, Scleroderma, Systemic therapy, Scleroderma, Systemic drug therapy, Autoimmune Diseases
Abstract

Systemic sclerosis (SSc) is a rare and chronic autoimmune disease characterized by a pathogenic triad of immune dysregulation, vasculopathy, and progressive fibrosis. Clinical tools commonly used to assess patients, including the modified Rodnan skin score, difference between limited or diffuse forms of skin involvement, presence of lung, heart or kidney involvement, or of various autoantibodies, are important prognostic factors, but still fail to reflect the large heterogeneity of the disease. SSc treatment options are diverse, ranging from conventional drugs to autologous hematopoietic stem cell transplantation, and predicting response is challenging. Genome-wide technologies, such as high throughput microarray analyses and RNA sequencing, allow accurate, unbiased, and broad assessment of alterations in expression levels of multiple genes. In recent years, many studies have shown robust changes in the gene expression profiles of SSc patients compared to healthy controls, mainly in skin tissues and peripheral blood cells. The objective analysis of molecular patterns in SSc is a powerful tool that can further classify SSc patients with similar clinical phenotypes and help predict response to therapy. In this review, we describe the journey from the first discovery of differentially expressed genes to the identification of enriched pathways and intrinsic subsets identified in SSc, using machine learning algorithms. Finally, we discuss the use of these new tools to predict the efficacy of various treatments, including stem cell transplantation. We suggest that the use of RNA gene expression-based classifications according to molecular subsets may bring us one step closer to precision medicine in Systemic Sclerosis., Competing Interests: Declaration of Competing Interest All authors declare no conflict of interest., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Published
2023
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4. Nailfold video capillaroscopy as a useful diagnostic tool in systemic vasculitis.

Author

Keret S, Mazzawi J, Slobodin G, Rimar O, Rosner I, Rozenbaum M, Kaly L, Boulman N, Awisat A, Shouval A, Ingegnoli F, and Rimar D

Subjects
Adult, Capillaries, Humans, Microscopic Angioscopy, Nails blood supply, Retrospective Studies, Raynaud Disease diagnosis, Scleroderma, Systemic, Systemic Vasculitis, Vasculitis
Abstract

Background: Nailfold video capillaroscopy (NVC) enables us a direct view of the microvasculature. Only several capillaroscopy studies in adult patients with vasculitis have been reported., Aim: To characterize NVC changes in vasculitis., Methods: Vasculitis patients and healthy controls were evaluated by NVC. NVC changes associated with vasculitis were assessed retrospectively in a cohort of 100 patients with Raynaud's phenomenon (RP)., Results: 17 patients with active vasculitis and 8 patients with vasculitis in remission were compared to 25 age and sex-matched healthy controls. Active vasculitis patients demonstrated higher rates of neoangiogenesis and capillary loss in comparison to other groups. Two novel NVC abnormalities were observed in patients with vasculitis: "Rolling" (slow capillary flow) and "peri-capillary stippling" (PCS), small deposits that may represent capillary leak. PCS was observed exclusively in 5 of 17 patients with active vasculitis. Retrospectively, we were able to detect PCS also in 14 % of 100 patients that were evaluated for RP, of whom 64 % were diagnosed with scleroderma or a related disorder., Conclusions: Patients with active vasculitis demonstrate frequent capillary abnormalities. Although these abnormalities are non-specific, we suggest that their combination may aid the diagnosis of vasculitis. Future studies are needed to validate our findings., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Published
2022
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6. SARS-COV-2 vaccination after stem cell transplantation for scleroderma.

Author

Rimar D, Slobodin G, Paz A, Henig I, and Zuckerman T

Subjects
Adult, Aged, Antibodies, Viral blood, Antibodies, Viral immunology, BNT162 Vaccine, CD4-Positive T-Lymphocytes immunology, Female, Hematopoietic Stem Cell Transplantation, Humans, Male, Middle Aged, SARS-CoV-2, Scleroderma, Systemic therapy, COVID-19 prevention & control, COVID-19 Vaccines immunology, Immunocompromised Host immunology, Scleroderma, Systemic immunology
Abstract

Competing Interests: Competing interests: None declared.

Published
2021
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8. Autologous Hematological Stem Cell Transplantation for Systemic Sclerosis in Israel.

Author

Rimar D, Butbul Aviel Y, Gefen A, Nevo N, Shen-Orr SS, Starosvetsky E, Rosner I, Rozenbaum M, Kaly L, Boulman N, Slobodin G, and Zuckerman T

Subjects
Adult, Autoantibodies blood, Autoantibodies classification, Child, Female, Humans, Immunosuppressive Agents administration & dosage, Immunosuppressive Agents adverse effects, Israel epidemiology, Lung pathology, Monitoring, Physiologic methods, Outcome and Process Assessment, Health Care, Respiratory Function Tests methods, Retrospective Studies, Skin pathology, Transplantation, Autologous, Cyclophosphamide administration & dosage, Cyclophosphamide adverse effects, Hematopoietic Stem Cell Transplantation adverse effects, Hematopoietic Stem Cell Transplantation methods, Scleroderma, Systemic diagnosis, Scleroderma, Systemic epidemiology, Scleroderma, Systemic immunology, Scleroderma, Systemic therapy
Abstract

Background: Autologous hematological stem cell transplantation (HSCT) is a novel therapy for systemic sclerosis (SSc) that has been validated in three randomized controlled trials., Objectives: To report the first Israeli experience with HSCT for progressive SSc and review the current literature., Methods: Five SSc patients who were evaluated in our department and were treated by HSCT were included. Medical records were evaluated retrospectively. Demographic, clinical, and laboratory data were recorded. Continuous data are presented as the mean ± standard deviation. Categorical variables are presented as frequencies and percentages., Results: Five SSc patients were treated with HSCT. Four patients were adults (mean age 53 ± 12 years) and one was a 12-year-old pediatric patient. All patients were female. HSCT was initiated 1.4 ± 0.8 years after diagnosis. Two patients were RNA POLIII positive, two were anti-topoisomerase 1 positive, and one only antinuclear antibodies positive. All patients had skin and lung involvement. The mean modified Rodnan Skin Score was 29 ± 4.7 before HSCT, which improved to 10.4 ± 9.6 after HSCT. The forced vital capacity improved from 68 ± 13% to 90 ± 28%. Diffusing capacity of the lungs for carbon monoxide increased by 6%. Among severe adverse events were cyclophosphamide-related congestive heart failure, antithymocyte globulin-related capillary leak syndrome, and scleroderma renal crisis. All symptoms completely resolved with treatment without sequela. No treatment related mortality was recorded., Conclusions: HSCT is an important step in the treatment of progressive SSc in Israel. Careful patient selection reduces treatment related morbidity and mortality.

Published
2020

9. Lysyl oxidase-a possible role in systemic sclerosis-associated pulmonary hypertension: a multicentre study.

Author

Vadasz Z, Balbir Gurman A, Meroni P, Farge D, Levi Y, Ingegnoli F, Braun-Moscovici Y, Rosner I, Slobodin G, Rozenbaum M, Jiries N, Kaly L, Boulman N, Zilber K, Ginsberg S, Awisat A, Goldberg Y, Lurie M, Ghigna MR, Guignabert C, Humbert M, and Rimar D

Subjects
Adult, Biopsy, Case-Control Studies, Female, Fibrosis, Humans, Hypertension, Pulmonary enzymology, Hypertension, Pulmonary pathology, Hypertension, Pulmonary physiopathology, Lung enzymology, Lung pathology, Male, Middle Aged, Protein-Lysine 6-Oxidase metabolism, Pulmonary Diffusing Capacity physiology, Scleroderma, Systemic enzymology, Scleroderma, Systemic pathology, Scleroderma, Systemic physiopathology, Skin enzymology, Skin pathology, Hypertension, Pulmonary etiology, Protein-Lysine 6-Oxidase physiology, Scleroderma, Systemic complications
Abstract

Objective: Lysyl oxidase (LOX) is an extracellular enzyme that cross-links collagen fibrils. LOX was found to be increased in serum of SSc patients and was suggested to be related to skin fibrosis, yet a vascular source of LOX has been demonstrated in idiopathic pulmonary arterial hypertension (iPAH). We aimed to validate elevated LOX serum levels in SSc and to study its correlation with clinical characteristics and investigate its main source at the tissue level., Methods: A total of 86 established SSc patients were compared with 86 patients with very early diagnosis of systemic sclerosis (VEDOSS), 110 patients with primary RP (PRP) and 80 healthy controls. LOX serum levels were determined by ELISA. Five lung and 12 skin biopsies from SSc patients were stained for LOX and compared with controls., Results: Serum levels of LOX in SSc were significantly higher than in VEDOSS, PRP and healthy controls (P < 0.001). LOX inversely correlated with the diffusing capacity of the lung for carbon monoxide diffusing capacity (DLCO) in diffuse SSc (r = -0.376, P = 0.02). Patients with moderate to severe estimated systolic PAH had higher LOX levels (P < 0.01). Lung biopsies demonstrated intense LOX staining in SSc patients with PAH that was predominantly located in the endothelium of the remodelled pulmonary vessels., Conclusion: Serum LOX levels are increased in established SSc and inversely correlate with the DLCO. LOX is elevated in patients with moderate to severe PAH and is located in the proliferating endothelium in lung arterioles, suggesting a possible role for LOX in SSc-associated PAH., (© The Author(s) 2019. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Published
2019
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10. Nailfold Video Capillaroscopy: Beyond Systemic Sclerosis.

Author

Rimar D, Rimar O, Rosner I, Rozenbaum M, Kaly L, Boulman N, and Slobodin G

Subjects
Humans, Raynaud Disease pathology, Scleroderma, Systemic pathology, Vasculitis diagnosis, Vasculitis pathology, Microscopic Angioscopy methods, Nails blood supply, Raynaud Disease diagnosis, Scleroderma, Systemic diagnosis
Published
2019

11. [THE ROLE OF SEMAPHORIN 7A IN SYSTEMIC SCLEROSIS].

Author

Rimar D, Slobodin G, Rosner I, Rozenbaum M, Kaly L, Boulman N, Jiries N, Awisat A, and Vadasz Z

Subjects
Animals, Fibroblasts metabolism, Humans, Integrin beta1 metabolism, Mice, Pilot Projects, Pulmonary Fibrosis etiology, Pulmonary Fibrosis metabolism, Scleroderma, Systemic complications, Scleroderma, Systemic metabolism, Skin, Scleroderma, Systemic drug therapy, Semaphorins metabolism, Semaphorins therapeutic use, Transforming Growth Factor beta1 metabolism
Abstract

Introduction: Semaphorins are a large group of membrane bound and secreted proteins. The semaphorins were first recognized for their important role in neurodevelopment and specifically their repulsive axonal growth guidance during embryonic development. Recently, semaphorins have also been found to have an important role in the regulation of the immune system, thus denoted as "immune semaphorins". Semaphorin 7A is a membrane bound protein which mediated its effect by two receptors: the β1 integrin subunit and plexin C1. Interactions between semaphorin 7A and its receptors contribute to inflammation and immunity by the stimulation of macrophage chemotaxis and cytokine production, regulation of dendritic cell migration and modulation of T cell function. Recently, semaphorin 7A has been found to have a role in the induction of fibrosis by tumor growth factor β1 (TGF β1). TGFβ1 activates semaphorin 7A and its receptors plexin C1 and β1 integrin subunit and induces proliferation of fibroblasts, lung fibrosis and remodeling in mice. A small study of 4 patients with systemic sclerosis (SSc) has recently demonstrated increased expression of semaphorin 7A mRNA on fibroblasts and B lymphocytes in peripheral blood., Aims: To evaluate the expression of semaphorin 7A on regulatory T cells and B cells from peripheral blood of patients with SSc compared to healthy controls and to try and correlate the expression of semaphorin 7A with pulmonary fibrosis, skin fibrosis and other clinical characteristics of SSc patients., Methods: Twenty six SSc patients were compared to 10 healthy controls. The expression of semaphorin 7A was evaluated by flow cytometry analysis of B cells using monoclonal antibodies to CD 108 and CD 19 and on peripheral regulatory T cells using monoclonal antibodies to CD 3 and CD 108. The analysis was conducted using flow-cytometry. Demographic, clinical and laboratory data were prospectively collected. Further data collection included: Systolic pulmonary artery pressure as assessed by echocardiography, lung function tests including diffusing capacity, nailfold video capillaroscopy pattern, modified Rodnan skin score (MRSS), Valentini activity index and Medsger severity score. Pulmonary involvement was determined by high resolution CT scan if it was suspected, according to impaired lung functions or auscultatory findings., Results: Ten patients with diffused SSC (8 of whom suffered from pulmonary fibrosis) and 16 patients with limited disease were compared with 10 healthy controls. There was no difference between the groups with regard to age, gender, BMI or smoking habits. Semaphorin 7A expression on regulatory T cells was not different between SSc patients and healthy controls 4.2±6.5 % vs. 2.3±1.1 % (p< 0.35) nor was a difference found between SSC patients with diffuse disease compared to limited disease 2.5±8 % vs. 5.1±14 % (p< 0.3). Comparing the expression of semaphorin 7A on B cells did not reveal a difference between SSc patients and healthy controls as well 9.7±9.4 % vs. 4.9±1.7% (p< 0.12). No correlation was found between skin score, activity score or severity score and levels of expression of sempahorin 7A on B cells or regulatory T cells., Conclusions: In this small scale study we were not able to validate the role of semaphorin 7A as a mediator of fibrosis in SSc, as was suggested by a previous pilot study. Larger scale studies and investigation of semaphorin 7A on other peripheral cells and in tissues are needed in order to delineate the exact role of semaphorin as a mediator of fibrosis in SSc.

Published
2017

12. Regulatory T Cells in Systemic Sclerosis: a Comprehensive Review.

Author

Slobodin G and Rimar D

Subjects
Humans, Scleroderma, Systemic immunology, T-Lymphocytes, Regulatory immunology
Abstract

Systemic sclerosis (SSc) is a chronic inflammatory disease with complex pathogenesis, based on the sophisticated interplay of injury to the vascular endothelium, exaggerated tissue regeneration and fibrosis, and extensive immune abnormalities. The role of regulatory T cells (Tregs) in the development of SSc has started being studied during the last decade with new aspects being disclosed continuously, in parallel with the better understanding of Tregs physiology. There is a general agreement in the medical literature regarding the decreased functional capacity of circulating Tregs in SSc. Some patients, particularly those with active disease, may have increased numbers of circulating Tregs, representing the inhibitory response of the immune system to its inappropriate activation or occurring as a compensatory move for Tregs' decreased suppressive ability. Decreased pool of circulating Tregs can be seen in other SSc patients, with even lower Treg percentages seen in patients with long-standing disease. Skin-resident Tregs are depleted in advanced SSc but can be active and have a role in earlier disease stages. In addition to diminished suppressive ability, Tregs can contribute to SSc evolution by their microenvironment-dependent transformation to pathogenic effector T cells of Th17 or Th2 lineages with respective pro-inflammatory or pro-fibrotic activity. The current data on the effects of existing treatment modalities, including autologous stem cell transplantation, on Tregs function in SSc, is controversial, not being sufficiently elaborated.

Published
2017
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13. Lysyl Oxidase in Systemic Sclerosis: Getting Under the Skin.

Author

Rimar D, Rosner I, Slobodin G, Rozenbaum M, Kaly L, Boulman N, and Vadasz Z

Subjects
Biomarkers metabolism, Humans, Scleroderma, Systemic enzymology, Scleroderma, Systemic immunology, Skin immunology, Protein-Lysine 6-Oxidase metabolism, Scleroderma, Systemic physiopathology, Skin pathology
Published
2016

14. Semaphorin 3A: an immunoregulator in systemic sclerosis.

Author

Rimar D, Nov Y, Rosner I, Slobodin G, Rozenbaum M, Halasz K, Haj T, Jiries N, Kaly L, Boulman N, and Vadasz Z

Subjects
Adult, Biomarkers blood, Female, Humans, Male, Middle Aged, Scleroderma, Systemic blood, Semaphorin-3A blood, Scleroderma, Systemic metabolism, Semaphorin-3A metabolism, T-Lymphocytes, Regulatory metabolism
Abstract

Abstarct: Semaphorin 3A (sema3A) plays a regulatory role in immune responses, mainly affecting the activation of regulatory T cells. It has been found to correlate with disease activity in rheumatoid arthritis and systemic lupus erythematosus (SLE). To investigate the expression of sema3A in patients with systemic sclerosis (SSc) compared to healthy controls and SLE disease controls and to correlate it with clinical characteristics, 27 SSc patients, 42 SLE patients and 28 healthy controls were enrolled. Serum level of sema3A was measured by ELISA, and expression of sema3A on regulatory T cells was evaluated by FACS analysis. SSc patients were evaluated for demographics, clinical manifestations, routine laboratory results, nailfold videocapillaroscopy, pulmonary function tests, echocardiograms, modified Rodnan skin score, and disease activity and severity scores. Serum levels of semaphorin 3A were lower in SSc compared to healthy controls 14.38 ± 5.7 versus 27.14 ± 8.4 ng/ml, p < 0.0001 and similar to SLE 15.7 ± 4.3 ng/ml. The expression of semaphorin 3A on regulatory T cells was also lower in SSc compared to healthy controls 61.7 ± 15.7 versus 88.7 ± 3. 7 % (p < 0.0001). Semaphorin 3A serum level inversely correlated with the duration of disease: r = -0.4, p = 0.036 and with low C4 level r = 0.66, p = 0.026. SCL-70 antibody positivity was associated with a lower semaphorin 3A level (difference in mean of 3.44, p = 0.06). Sema3A expression is low in SSc serum and more specifically on regulatory T cells. This may help explain the reduced activation of regulatory T cells in SSc.

Published
2015
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15. New potential biomarkers for disease activity and fibrosis in systemic sclerosis.

Author

Vadasz Z and Rimar D

Subjects
Biomarkers blood, Humans, Patient Acuity, T-Lymphocytes, Regulatory metabolism, Connective Tissue metabolism, Connective Tissue pathology, Fibrosis enzymology, Inflammation immunology, Protein-Lysine 6-Oxidase metabolism, Scleroderma, Systemic blood, Scleroderma, Systemic diagnosis, Semaphorin-3A blood
Published
2014

16. Brief report: lysyl oxidase is a potential biomarker of fibrosis in systemic sclerosis.

Author

Rimar D, Rosner I, Nov Y, Slobodin G, Rozenbaum M, Halasz K, Haj T, Jiries N, Kaly L, Boulman N, Daood R, and Vadasz Z

Subjects
Adult, Biomarkers blood, Female, Fibrosis blood, Fibrosis etiology, Humans, Lung physiopathology, Male, Middle Aged, Respiratory Function Tests, Scleroderma, Systemic blood, Scleroderma, Systemic physiopathology, Skin Diseases blood, Skin Diseases etiology, Fibrosis diagnosis, Protein-Lysine 6-Oxidase blood, Scleroderma, Systemic complications, Skin Diseases diagnosis
Abstract

Objective: Fibrosis is a major cause of morbidity and mortality in systemic sclerosis (SSc). Levels of lysyl oxidase (LOX), an extracellular enzyme that stabilizes collagen fibrils, have been found to be elevated in the skin of SSc patients, but have not been evaluated in the serum or correlated with the clinical parameters. We undertook this study to evaluate serum LOX levels in SSc patients and to correlate these levels with clinical parameters of SSc., Methods: SSc patients were evaluated for demographic features, clinical manifestations, routine laboratory tests, serum autoantibodies, serum LOX concentrations, and nailfold capillaroscopy patterns. They underwent pulmonary function testing, echocardiography, and high-resolution computed tomography scans of the lung, assessment of skin fibrosis by the modified Rodnan skin thickness score (MRSS), and assessment of disease severity and activity by the Medsger severity scale and the Valentini activity index., Results: Twenty-six SSc patients were evaluated and compared with 25 healthy controls and with 9 disease control patients with primary myelofibrosis. Almost 62% of the SSc patients (16 of 26) had limited cutaneous SSc (lcSSc), while 38% had diffuse cutaneous SSc (dcSSc) (10 of 26); 31% of the patients (8 of 26) had lung involvement. The LOX concentration in SSc patients was higher than that in healthy controls and similar to that in disease controls (P < 0.0001), and it was significantly higher in patients with dcSSc than in those with lcSSc (P = 0.006). The LOX concentration correlated with the MRSS in patients without lung fibrosis., Conclusion: This study is the first to demonstrate high serum LOX levels in SSc patients that correlate specifically with skin fibrosis. These correlations suggest that LOX levels may serve as a novel biomarker of fibrosis. Future studies are warranted to determine whether LOX is a potential therapeutic target in SSc., (Copyright © 2014 by the American College of Rheumatology.)

Published
2014
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17. Racial differences in systemic sclerosis disease presentation: A European Scleroderma Trials and Research group study

Author

Jaeger, Veronika K, Tikly, Mohammed, Dong, Xu, Siegert, Elise, Hachulla, Eric, Airò, Paolo, Valentini, Gabriele, Matucci Cerinic, Marco, Distler, Oliver, Cozzi, Franco, Carreira, Patricia, Allanore, Yannick, Müller-Ladner, Ulf, Ananieva, Lidia P, Balbir-Gurman, Alexandra, Distler, Jörg H W, Czirják, Laszlo, Mengtao, Li, Henes, Jörg, Jimenez, Sergio A, Smith, Vanessa, Damjanov, Nemanja, Denton, Christopher P, Delgaldo, Francesco, Saketkoo, Lesley Ann, Walker, Ulrich, A, Randone, Sb, Bannert, B, Iannone, F, Maurer, B, Jordan, S, Dobrota, R, Becker, M, Mihai, C, Becvarare, R, Tomčík, M, Bielecka, Ok, Gindzienska-Sieskiewicz, E, Karaszewska, K, Cutolo, M, Pizzorni, C, Paolino, S, Sulli, A, Ruaro, B, Alessandri, E, Riccardi, A, Giacco, V, Messitini, V, Irace, R, Kedor, C, Casteleyn, V, Hilger, J, Hoeppner, J, Rednic, S, Szabo, I, Petcu, A, Avouac, J, Camelia, F, Desbas, C, Vlachoyiannopoulos, P, Montecucco, C, Caporali, R, Cavagna, L, Stork, J, Inanc, M, Joven, Be, Novak, S, Anic, F, Varju, C, Minier, T, Chizzolini, C, Allai, D, Kucharz, Ej, Kotulska, A, Kopec-Medrek, M, Widuchowska, M, Dolnicar, As, Coleiro, B, Gabrielli, A, Manfredi, L, Benfaremo, D, Ferrarini, A, Bancel, Df, Hij, A, Lansiaux, P, Lazzaroni, Mg, Hesselstrand, R, Wuttge, D, Andréasson, R, Martinovic, D, Bozic, I, Radic, M, Braun-Moscovici, Y, Monaco, Al, Furini, F, Hunzelmann, N, Moinzadeh, P, Pellerito, R, Caimmi, C, Bertoldo, E, Morovic-Vergles, J, Culo, Im, Pecher, Ac, Santamaria, Vo, Heitmann, S, Codagnone, M, Pflugfelder, J, Krasowska, D, Michalska-Jakubus, M, Seidel, M, Hasler, P, Kretschmar, S, Kohm, M, Bajocchi, G, Salvador, Mj, Silva, Japd, Stamenkovic, B, Stankovic, A, Selmi, Cf, Santis, M, Ceribelli, A, Garzanova, L, Koneva, O, Starovoytova, M, Herrick, A, Puppo, F, Negrini, S, Murdaca, G, Engelhart, M, Szücs, G, Szamosi, S, de la Puente, C, Grande, Cs, Villanueva, Mjg, Midtvedt, Sø, Hoffmann-Vold, Am, Launay, D, Sobanski, V, Riccieri, V, Vasile, M, Ionescu, Rm, Opris, D, Sha, A, Woods, A, Gheorghiu, Am, Bojinca, M, Sunderkötter, C, Ehrchen, J, Ingegnoli, F, Mouthon, L, Dunogue, B, Chaigne, B, Legendre, P, Cantatore, Fp, Corrado, A, Ullman, S, Iversen, L, von Mühlen CA, Pozzi, Mr, Eyerich, K, Lauffer, F, Wiland, P, Szmyrka-Kaczmarek, M, Sokolik, R, Morgiel, E, Madej, M, Vanthuyne, M, Frédéric, H, Alegre-Sancho, Jj, Aringer, M, Herrmann, K, Günther, C, Westhovens, R, Langhe, E, Lenaerts, J, Anic, B, Baresic, M, Mayer, M, Üprus, M, Otsa, K, Yavuz, S, Granel, B, Radominski, Sc, De, C, Müller, S, Azevedo, Vf, Mendoza, F, Busquets, J, Popa, S, Agachi, S, Zenone, T, Pileckyte, M, Stebbings, S, Mathieu, A, Vacca, A, Sampaio-Barros, Pd, Stamp, L, Solanki, K, Silva, C, Schollum, J, Barns-Graham, H, Veale, D, O'Rourke, M, Loyo, E, Tineo, C, Paulino, G, Mohamed, Waaa, Rosato, E, Gigante, A, Oksel, F, Yargucu, F, Tanaseanu, Cm, Popescu, M, Dumitrascu, A, Tiglea, I, Foti, R, Visalli, E, Benenati, A, Amato, G, Ancuta, C, Villiger, P, Adler, S, Fröhlich, J, Kayser, C, Eduardo, Al, Fathi, N, Alii, S, Ahmed, M, Hasaneen, S, Hakeem, Ee, de la PG, Lefebvre, P, Martin, Jjg, Sibilia, J, Chatelus, E, Gottenberg, Je, Chifflot, H, Litinsky, I, Galdo, Fd, Abignano, G, Eng, S, Seskute, G, Butrimiene, I, Rugiene, R, Karpec, D, Pascal, M, Kerzberg, E, Bianchi, W, Bianchi, Bv, Bianchi, Dv, Barcellos, Y, Castellví, I, Millan, M, Limonta, M, Rimar, D, Rosner, I, Slobodin, G, Couto, M, Spertini, F, Ribi, C, Buss, G, Marcoccia, A, Bondanini, F, Ciani, A, Kahl, S, Hsu, Vm, Martin, T, Poindron, V, Meghit, K, Moiseev, S, Novikov, P, Chung, L, Kolstad, K, Stark, M, Schmeiser, T, Thiele, A, Majewski, D, Zdrojewski, Z, Zaneta, S, Wierzba, K, Martínez-Barrio, J, López-Longo, Fj, Bernardino, V, Moraes-Fontes, Mf, Rodrigues, Ac, Riemekasten, G, Sommerlatte, S, Jendreck, S, Arnold, S, Levy, Y, Rezus, E, Cardoneanu, A, Burlui, Am, Pamuk, On, Puttini, Ps, Talotta, R, Bongiovanni, S, Poormoghim, H, Andalib, E, Almasi, S, Kötter, I, Krusche, M, Cuomo, G, Danzo, F, Masini, F, Gaches, F, Michaud, M, Cartos, F, Belloli, L, Casu, C, Sfikakis, P, Tektonidou, M, Furst, D, Feldman, Gr, Ramazan, Am, Nurmambet, E, Miroto, A, Suta, C, Andronache, I, Huizinga, Twj, de Vries-Bouwstra, J., Chizzolini, Carlo, Jaeger, Veronika K, Tikly, Mohammed, Xu, Dong, Siegert, Elise, Hachulla, Eric, Airò, Paolo, Valentini, Gabriele, Matucci Cerinic, Marco, Distler, Oliver, Cozzi, Franco, Carreira, Patricia, Allanore, Yannick, Müller-Ladner, Ulf, Ananieva, Lidia P, Balbir-Gurman, Alexandra, Distler, Jörg H W, Czirják, Laszlo, Li, Mengtao, Henes, Jörg, Jimenez, Sergio A, Smith, Vanessa, Damjanov, Nemanja, Denton, Christopher P, Delgaldo, Francesco, Saketkoo, Lesley Ann, Walker, Ulrich A, University of Zurich, Cerinic, Marco Matucci, Walker Ulrich, A, Randone, Silvia Bellando, Bannert, Bettina, Iannone, Florenzoaa, Maurer, Brittaab, Jordan, Suzanaab, Dobrota, Rucsandraab, Becker, Mikeab, Mihai, Carinaa, Becvarare, Radima, Tomcik, Michala, Bielecka, Otylia Kowala, Gindzienska-Sieskiewicz, Ewaa, Karaszewska, Katarzynaa, Cutolo, Maurizioa, Pizzorni, Carmena, Paolino, Sabrinaae, Sulli, Albertoa, Ruaro, Barbara, Alessandri, Elisa, Riccardi, Antonella, Giacco, Veronica, Messitini, Valentina, Irace, Rosaria, Kedor, Claudia, Casteleyn, Vincent, Hilger, Julia, Hoeppner, Jakob, Rednic, Simona, Szabo, Iulia, Petcu, Ana, Avouac, Jérome, Camelia, Frantz, Desbas, Carole, Vlachoyiannopoulos, Panayioti, Montecucco, Carlo Maurizio, Caporali, Roberto, Cavagna, Lorenzo, Stork, Jiri, Inanc, Murat, Joven, Beatriz E., Novak, Srdan, Anic, Felina, Varju, Cecilia, Minier, Tunde, Allai, Daniela, Kucharz, Eugene J., Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Dolnicar, Alenka Sipek, Coleiro, Bernard, Gabrielli, Armando, Manfredi, Lucia, Benfaremo, Devi, Ferrarini, Alessia, Bancel, Dominique Farge, Hij, Adrian, Lazzaroni, Maria Grazia, Hesselstrand, Roger, Wuttge, Dirk, Andréasson, Kristofer, Martinovic, Duska, Bozic, Ivona, Radic, Mislav, Braun-Moscovici, Yolanda, Monaco, Andrea Lo, Furini, Federica, Hunzelmann, Nicola, Moinzadeh, Pia, Pellerito, Raffaele, Caimmi, Cristian, Bertoldo, Eugenia, Morovic-Vergles, Jadranka, Culo, Ivana Melanie, Pecher, Ann-Christian, Santamaria, Vera Ortiz, Heitmann, Stefan, Codagnone, Medeleine, Pflugfelder, Johanne, Krasowska, Dorota, Michalska-Jakubus, Malgorzata, Seidel, Matthia, Hasler, Paul, Kretschmar, Samuel, Kohm, Michaela, Bajocchi, Gianluigi, Salvador, Maria João, Da Silva, JoséAntonio Pereira, Stamenkovic, Bojana, Stankovic, Aleksandra, Selmi, Carlo Francesco, De Santis, Maria, Ceribelli, Angela, Garzanova, Ludmila, Koneva, Olga, Starovoytova, Maya, Herrick, Ariane, Puppo, Francesco, Negrini, Simone, Murdaca, Giuseppe, Engelhart, Merete, Szücs, Gabriela, Szamosi, Szilvia, De La Puente, Carlo, Grande, Cristina Sobrino, Villanueva, Maria Jesus Garcia, Midtve, Øyvindbw, Hoffmann-Vold, Anna-Mariabw, Launay, Davidbx, Sobanski, Vincentbx, Riccieri, Valeriaby, Vasile, Massimilianoby, Stefantoni, Katia, Ionescu, Ruxandra Maria, Opris, Daniela, Sha, Ami, Woods, Adrianne, Gheorghiu, Ana Maria, Bojinca, Mihai, Sunderkötter, Cord, Ehrchen, Jan, Ingegnoli, Francesca, Mouthon, Luc, Dunogue, Bertrand, Chaigne, Benjamin, Legendre, Paul, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Von Mühlen, Carlos Alberto, Pozzi, Maria Rosa, Eyerich, Kilian, Lauffer, Felix, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Vanthuyne, Marie, Frédéric, Houssiau, Alegre-Sancho, Juan Jose, Aringer, Martin, Herrmann, Kristine, Günther, Claudia, Westhovens, Rene, De Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Üprus, Maria, Otsa, Kati, Yavuz, Sule, Granel, Brigitte, Radominski, Sebastião Cezar, De Souza Müller, Carolina, Feijóazevedo, Valderílio, Mendoza, Fabian, Busquets, Joanna, Popa, Sergei, Agachi, Svetlana, Zenone, Thierry, Pileckyte, Margarita, Stebbings, Simon, Jordan, Sarah, Mathieu, Alessandro, Vacca, Alessandra, Sampaio-Barros, Percival D., Stamp, Lisa, Solanki, Kamal, Silva, Cherumi, Schollum, Joanne, Barns-Graham, Helen, Veale, Dougla, O'Rourke, Marie, Loyo, Esthela, Tineo, Carmen, Paulino, Glenny, Mohamed, Walid Ahmed Abdel Atty, Rosato, Edoardo, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Visalli, Elisa, Benenati, Alessia, Amato, Giorgio, Ancuta, Codrina, Villiger, Peter, Adler, Sabine, Fröhlich, Johanne, Kayser, Cristiane, Eduardo, Andrade Lui, Fathi, Nihal, Alii, Safa, Ahmed, Marrow, Hasaneen, Samar, El Hakeem, Eman, De La Peña Lefebvre, Paloma García, Martin, Jorge Juan Gonzalez, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Hélène, Litinsky, Ira, Del Galdo, Francesco, Abignano, Giuseppina, Eng, Sookho, Seskute, Goda, Butrimiene, Irena, Rugiene, Rita, Karpec, Diana, Pascal, Melanie, Kerzberg, Eduardo, Bianchi, Washington, Bianchi, Breno Valdetaro, Bianchi, Dante Valdetaro, Barcellos, Yeda, Castellví, Ivan, Millan, Milena, Limonta, Massimiliano, Rimar, Doron, Rosner, Itzhak, Slobodin, Gleb, Couto, Maura, Spertini, Françoi, Ribi, Camillo, Buss, Guillaume, Marcoccia, Antonella, Bondanini, Francesco, Ciani, Aldo, Kahl, Sarah, Hsu, Vivien M., Martin, Thierry, Poindron, Vincent, Meghit, Kilifa, Moiseev, Sergey, Novikov, Pavel, Chung, Lori, Kolstad, Kathleen, Stark, Marianna, Schmeiser, Tim, Thiele, Astrid, Majewski, Dominik, Zdrojewski, Zbigniew, Zaneta, Smolenska, Wierzba, Karol, Martínez-Barrio, Julia, López-Longo, Francisco Javier, Bernardino, Vera, Moraes-Fontes, Maria Francisca, Rodrigues, Ana Catarina, Riemekasten, Gabriela, Sommerlatte, Sabine, Jendreck, Sebastian, Arnold, Sabrina, Levy, Yair, Rezus, Elena, Cardoneanu, Anca, Burlui, Alexandra Maria, Pamuk, Omer Nuri, Puttini, Piercarlo Sarzi, Talotta, Rossella, Bongiovanni, Sara, Poormoghim, Hadi, Andalib, Elham, Almasi, Simin, Kötter, Ina, Krusche, Matrin, Cuomo, Giovanna, Danzo, Fiammetta, Masini, Francesco, Gaches, Franci, Michaud, Martin, Cartos, Florian, Belloli, Laura, Casu, Cinzia, Sfikakis, Petro, Tektonidou, Maria, Furst, Daniel, Feldman, Gary R., Ramazan, Ana-Maria, Nurmambet, Emel, Miroto, Amalia, Suta, Cristina, Andronache, Iulia, Huizinga, Tom W. J., De Vries-Bouwstra, Jeska, and Walker, Ulrich A.

Subjects
Male, Vital capacity, Organ manifestations, systemic sclerosis, Type I, race difference, Systemic scleroderma, Gastroenterology, Scleroderma, immunology, 0302 clinical medicine, Diffusing capacity, middle aged, pulmonary hypertension, Medicine, Pharmacology (medical), 030212 general & internal medicine, organ manifestations, races, skin and connective tissue diseases, Lung, race, pathophysiology, African Continental Ancestry Group, ddc:616, integumentary system, disease course, Hazard ratio, Races, 10051 Rheumatology Clinic and Institute of Physical Medicine, Pulmonary, Middle Aged, Blacks, cohort analysis, Autoantibodie, 3. Good health, Asians, female, priority journal, DNA Topoisomerases, Type I, Black, centromere, Cohort, Hypertension, organ manifestation, Systemic sclerosis, Female, systemic sclerosi, Human, Adult, Asian Continental Ancestry Group, medicine.medical_specialty, Hypertension, Pulmonary, European Continental Ancestry Group, Black People, 610 Medicine & health, complication, Caucasian, White People, Article, lung, 03 medical and health sciences, Black person, Rheumatology, Asian People, forced vital capacity, Internal medicine, geographic distribution, Humans, controlled study, human, DNA topoisomerase, Aged, Autoantibodies, 030203 arthritis & rheumatology, Scleroderma, Systemic, Asian, business.industry, Whites, Systemic, Odds ratio, medicine.disease, Pulmonary hypertension, major clinical study, mortality, clinical feature, business, DNA Topoisomerases, autoantibody
Abstract

Objectives Racial factors play a significant role in SSc. We evaluated differences in SSc presentations between white patients (WP), Asian patients (AP) and black patients (BP) and analysed the effects of geographical locations. Methods SSc characteristics of patients from the EUSTAR cohort were cross-sectionally compared across racial groups using survival and multiple logistic regression analyses. Results The study included 9162 WP, 341 AP and 181 BP. AP developed the first non-RP feature faster than WP but slower than BP. AP were less frequently anti-centromere (ACA; odds ratio (OR) = 0.4, P < 0.001) and more frequently anti-topoisomerase-I autoantibodies (ATA) positive (OR = 1.2, P = 0.068), while BP were less likely to be ACA and ATA positive than were WP [OR(ACA) = 0.3, P < 0.001; OR(ATA) = 0.5, P = 0.020]. AP had less often (OR = 0.7, P = 0.06) and BP more often (OR = 2.7, P < 0.001) diffuse skin involvement than had WP. AP and BP were more likely to have pulmonary hypertension [OR(AP) = 2.6, P < 0.001; OR(BP) = 2.7, P = 0.03 vs WP] and a reduced forced vital capacity [OR(AP) = 2.5, P < 0.001; OR(BP) = 2.4, P < 0.004] than were WP. AP more often had an impaired diffusing capacity of the lung than had BP and WP [OR(AP vs BP) = 1.9, P = 0.038; OR(AP vs WP) = 2.4, P < 0.001]. After RP onset, AP and BP had a higher hazard to die than had WP [hazard ratio (HR) (AP) = 1.6, P = 0.011; HR(BP) = 2.1, P < 0.001]. Conclusion Compared with WP, and mostly independent of geographical location, AP have a faster and earlier disease onset with high prevalences of ATA, pulmonary hypertension and forced vital capacity impairment and higher mortality. BP had the fastest disease onset, a high prevalence of diffuse skin involvement and nominally the highest mortality.

Published
2020

18. Incidence and risk factors for gangrene in patients with systemic sclerosis from the EUSTAR cohort

Author

Mihai, Carina, Distler, Oliver, Gheorghiu, Ana Maria, Constantin, Paul I, Dobrota, Rucsandra, Jordan, Suzana, Smith, Vanessa, Hachulla, Eric, Henes, Jörg, Siegert, Elise, Vettori, Serena, Müller-Ladner, Ulf, Matucci Cerinic, Marco, Allanore, Yannick, Lepri, G, Jaeger, Vk, Walker, Ua, Iannone, F, Cacciapaglia, F, Tomčík, M, Becvar, R, Rednic, S, Petcu, A, Szabo, I, Codullo, V, Caporali, R, Montecucco, C, Carreira, P, Ioven, B, Minier, T, Czirják, L, Chizzolini, C, Allali, D, Zanatta, E, Doria, A, Gabrielli, A, Airò, P, Lazzaroni, Mg, Radić, M, Martinovic, D, Braun-Moscovici, Y, Balbir-Gurman, A, Hunzelmann, N, Caramaschi, P, Morovic-Vergles, J, Denton, C, Santamaria, V, Heitmann, S, Krasowska, D, Michalska-Jakubus, M, Seidel, M, Foeldvari, I, Helmus, N, Salvador, M, Stamenkovic, B, Stankovic, A, Ananieva, L, Herrick, A, Engelhart, M, De La Puente, C, Hoffmann-Vold, Am, Midtvedt, Ø, Launay, D, Sobanski, V, Riccieri, V, Opris-Belinski, D, Groseanu, L, Ionescu, R, Bojinca, M, Sunderkötter, C, Distler, J, Ingegnoli, F, van der Haecke, A, Ullman, S, Pozzi, Mr, Eyerich, K, Vanthuyne, M, Erler, A, Aringer, M, De Langhe, E, Baresic, M, Mayer, M, Anic, B, Yavuz, S, Granel, B, Popa, S, Agachi, S, Zenone, T, Mathieu, A, Vacca, A, Solanki, K, Veale, D, Loyo, E, Tineo, C, Gigante, A, Rosato, E, Oksel, F, Yagurcu, F, Tănăseanu, Cm, Visalli, E, Benenati, A, Foti, R, Ancuta, C, Dan, D, Adler, S, Villiger, P, Fathi, N, de la Peña Lefebvre PG, González Martín, J, Chatelus, E, Sibilia, J, Litinsky, I, Del Galdo, F, Ann Sakettkoo, L, Kerzberg, E, Bianchi, Wa, Bianchi, Bv, Castellví, I, Limonta, M, Rimar, D, Couto, M, Ribi, C, Spertini, F, Kahl, S, Hsu, V, Poindron, V, Meghit, K, Martin, T, Kolstad, K, Chung, L, Thiele, A, Schmeiser, T, Zdrojewski, Z, Riemekasten, G, Levy, Y, Cardoneanu, A, Burlui, A, Rezus, E, Pamuk, On, Talotta, R, Bongiovanni, S, Puttini, Ps., Mihai, Carina, Distler, Oliver, Gheorghiu, Ana Maria, Constantin, Paul I, Dobrota, Rucsandra, Jordan, Suzana, Smith, Vanessa, Hachulla, Eric, Henes, Jörg, Siegert, Elise, Vettori, Serena, Müller-Ladner, Ulf, Matucci Cerinic, Marco, Allanore, Yannick, Giovanna, Cuomo, Chizzolini, Carlo, Allali, Danièle, and University of Zurich

Subjects
Adult, Male, medicine.medical_specialty, Databases, Factual, systemic sclerosis, digital ulcer, 610 Medicine & health, Disease, ddc:616.07, Logistic regression, Systemic scleroderma, Cohort Studies, Rheumatology, Risk Factors, Internal medicine, Cox proportional hazards regression, medicine, Humans, Pharmacology (medical), In patient, digital ulcers, gangrene, vasculopathy, Aged, Gangrene, Scleroderma, Systemic, business.industry, Incidence (epidemiology), Incidence, 10051 Rheumatology Clinic and Institute of Physical Medicine, food and beverages, Middle Aged, medicine.disease, Cross-Sectional Studies, Cohort, Female, business, systemic sclerosi
Abstract

Objective In patients with SSc, peripheral vasculopathy can promote critical ischaemia and gangrene. The aim of this study was to investigate the prevalence, incidence and risk factors for gangrene in the EUSTAR cohort. Methods We included patients from the EUSTAR database fulfilling the ACR 1980 or the ACR/EULAR 2013 classification criteria for SSc, with at least one visit recording data on gangrene. Centres were asked for supplementary data on traditional cardiovascular risk factors. We analysed the cross-sectional relationship between gangrene and its potential risk factors by univariable and multivariable logistic regression. Longitudinal data were analysed by Cox proportional hazards regression. Results 1757 patients were analysed (age 55.9 [14.5] years, disease duration 7.9 [10.3] years, male sex 16.7%, 24.6% diffuse cutaneous subset [dcSSc]). At inclusion, 8.9% of patients had current or previous digital gangrene, 16.1% had current digital ulcers (DUs) and 42.7% had ever had DUs (current or previous). Older age, DUs ever and dcSSc were statistically significant risk factors for gangrene in the cross-sectional multivariable model. During a median follow-up of 13.1 months, 16/771 (0.9%) patients developed gangrene. All 16 patients who developed gangrene had previously had DUs and gangrene. Further risk factors for incident gangrene were the dcSSc subset and longer disease duration. Conclusion In unselected SSc patients, gangrene occurs in about 9% of SSc patients. DUs ever and, to a lesser extent, the dcSSc subset are strongly and independently associated with gangrene, while traditional cardiovascular risk factors could not be identified as risk factors.

Published
2019

19. Outcomes of patients with systemic sclerosis treated with rituximab in contemporary practice: a prospective cohort study

Author

Elhai, M, Boubaya, M, Distler, O, Smith, V, Matucci-Cerinic, M, Sancho, JJ, Truchetet, ME, Braun-Moscovici, Y, Iannone, F, Novikov, PI, Lescoat, A, Siegert, E, Castellvi, I, Airo, P, Vettori, S, Langhe, E, Hachulla, E, Erler, A, Ananieva, L, Krusche, M, Lopez-Longo, FJ, Distler, JHW, Hunzelmann, N, Hoffmann-Vold, AM, Riccieri, V, Hsu, VM, Pozzi, MR, Ancuta, C, Rosato, E, Mihai, C, Kuwana, M, Saketkoo, LA, Chizzolini, C, Hesselstrand, R, Ullman, S, Yavuz, S, Rednic, S, Caimmi, C, Bloch-Queyrat, C, Allanore, Y, Guiducci, S, Walker, UA, Kyburz, D, Lapadula, G, Maurer, B, Jordan, S, Dobrota, R, Becvar, R, Sierakowsky, S, Bielecka, OK, Sulli, A, Cutolo, M, Cuomo, G, Nicoara, I, Kahan, A, Vlachoyiannopoulos, PG, Montecucco, CM, Caporali, R, Stork, J, Inanc, M, Carreira, PE, Novak, S, Czirjak, L, Varju, C, Kucharz, EJ, Kotulska, A, Kopec-Medrek, M, Widuchowska, M, Cozzi, F, Rozman, B, Mallia, C, Coleiro, B, Gabrielli, A, Farge, D, Wu, C, Marjanovic, Z, Faivre, H, Hij, D, Dhamadi, R, Wollheim, F, Scheja, A, Wuttge, DM, Andreasson, K, Martinovic, D, Balbir-Gurman, A, Trotta, F, Lo Monaco, A, Pellerito, R, Mauriziano, O, Caramaschi, P, Morovic-Vergles, J, Black, C, Denton, C, Damjanov, N, Henes, J, Santamaria, VO, Heitmann, S, Krasowska, D, Matthias, Hasler, P, Burkhardt, H, Himsel, A, Bajocchi, G, Da Silva, JAP, Salvador, MJ, Stamenkovic, B, Stankovic, A, Selmi, CF, De Santis, M, Tikly, M, Denisov, LN, Herrick, A, Muller-Ladner, U, Frerix, M, Tarner, I, Scorza, R, Puppo, F, Engelhart, M, Strauss, G, Nielsen, H, Damgaard, K, Szucs, G, Mendoza, AZ, de la Puente, C, Giraldo, WAS, Midtvedt, O, Reiseter, S, Garen, T, Launay, D, Valesini, G, Ionescu, RM, Groseanu, L, Opris, D, Cornateanu, RS, Ionitescu, R, Gherghe, AM, Soare, A, Gorga, M, Bojinca, M, Milicescu, M, Sunderkotter, C, Kuhn, A, Sandorfi, N, Schett, G, Beyer, C, Meroni, P, Ingegnoli, F, Mouthon, L, De Keyser, F, Melsens, K, Cantatore, FP, Corrado, A, Iversen, L, von Muhlen, CA, Bohn, JM, Lonzetti, LS, Eyerich, K, Hein, R, Knott, E, Wiland, P, Szmyrka-Kaczmarek, M, Sokolik, R, Morgiel, E, Madej, M, Houssiau, FA, Krummel-Lorenz, B, Saar, P, Aringer, M, Gunther, C, Westhovens, R, Lenaerts, J, Anic, B, Baresic, M, Mayer, M, Uprus, M, Otsa, K, Granel, B, Muller, CD, Radominski, SC, Azevedo, VF, Jimenez, S, Busquets, J, Agachi, S, Groppa, L, Chiaburu, L, Russu, E, Popa, S, Zenone, T, Pileckyte, M, Mathieu, A, Vacca, A, Sampaio-Barros, PD, Yoshinari, NH, Marangoni, RG, Martin, P, Fuocco, L, Stebbings, S, Highton, J, Chapman, P, O'Donnell, J, Stamp, L, Doube, A, Solanki, K, Veale, D, O'Rourke, M, Loyo, E, Li, MT, Mohamed, WAAA, Amoroso, A, Gigante, A, Oksel, F, Yargucu, F, Tanaseanu, CM, Popescu, M, Dumitrascu, A, Tiglea, I, Foti, R, Chirieac, R, Furst, D, Villiger, P, Adler, S, van Laar, J, Kayser, C, Fathi, N, Hassanien, M, Lefebvre, PGD, Rubio, SR, Exposito, MV, Chatelus, E, Sibilia, J, Gottenberg, JE, Chifflot, H, Litinsky, I, Emery, P, Buch, M, Del Galdo, F, Venalis, A, Butrimiene, I, Venalis, P, Rugiene, R, Karpec, D, Lasky, JA, Cosentino, V, Kerzberg, E, Montoya, F, Bianchi, W, Carneiro, S, Maretti, GB, Bianchi, DV, Limonta, M, Lupi, ALBE, Lupi, E, Rosner, I, Rozenbaum, M, Slobodin, G, Boulman, N, Rimar, D, Couto, M, Kahl, S, Chen, F, McCloskey, D, Malveaux, H, Spertini, F, Ribi, C, Buss, G, Martin, T, Guffroy, A, Poindron, V, Chotchaeva, F, Mukhin, NA, Moiseev, S, EUSTAR Network, Elhai, Muriel, Boubaya, Marouane, Distler, Oliver, Smith, Vanessa, Matucci-Cerinic, Marco, Alegre Sancho, Juan José, Truchetet, Marie-Elise, Braun-Moscovici, Yolanda, Iannone, Florenzo, Novikov, Pavel I, Lescoat, Alain, Siegert, Elise, Castellví, Ivan, Airó, Paolo, Vettori, Serena, De Langhe, Ellen, Hachulla, Eric, Erler, Anne, Ananieva, Lidia, Krusche, Martin, López-Longo, F. J., Distler, Jörg H W, Hunzelmann, Nicola, Hoffmann-Vold, Anna-Maria, Riccieri, Valeria, Hsu, Vivien M, Pozzi, Maria R, Ancuta, Codrina, Rosato, Edoardo, Mihai, Carina, Kuwana, Masataka, Saketkoo, Lesley Ann, Chizzolini, Carlo, Hesselstrand, Roger, Ullman, Susanne, Yavuz, Sule, Rednic, Simona, Caimmi, Cristian, Bloch-Queyrat, Coralie, Allanore, Yannick, and Cuomo, Giovanna

Subjects
Male, Vital capacity, systemic sclerosis, Pulmonary Fibrosis, Vital Capacity, Scleroderma, lung fibrosis, rituximab, skin fibrosis, immune system diseases, DLCO, hemic and lymphatic diseases, Immunology and Allergy, Medicine, Prospective Studies, Registries, skin and connective tissue diseases, Prospective cohort study, Lung, skin fibrosi, Skin, ddc:616, integumentary system, Orvostudományok, Middle Aged, Respiratory Function Tests, lung fibrosis, rituximab, skin fibrosis, systemic sclerosis, Treatment Outcome, lung fibrosi, Antirheumatic Agents, Systemic sclerosis, Rituximab, Female, systemic sclerosi, medicine.drug, Adult, medicine.medical_specialty, Immunology, Klinikai orvostudományok, General Biochemistry, Genetics and Molecular Biology, FEV1/FVC ratio, Rheumatology, Internal medicine, Humans, Adverse effect, Propensity Score, Aged, Biochemistry, Genetics and Molecular Biology (all), Scleroderma, Systemic, Skin fibrosis, business.industry, medicine.disease, Fibrosis, Lung fibrosis, business
Abstract

ObjectiveTo assess the safety and efficacy of rituximab in systemic sclerosis (SSc) in clinical practice.MethodsWe performed a prospective study including patients with SSc from the European Scleroderma Trials and Research (EUSTAR) network treated with rituximab and matched with untreated patients with SSc. The main outcomes measures were adverse events, skin fibrosis improvement, lung fibrosis worsening and steroids use among propensity score-matched patients treated or not with rituximab.Results254 patients were treated with rituximab, in 58% for lung and in 32% for skin involvement. After a median follow-up of 2 years, about 70% of the patients had no side effect. Comparison of treated patients with 9575 propensity-score matched patients showed that patients treated with rituximab were more likely to have skin fibrosis improvement (22.7 vs 14.03 events per 100 person-years; OR: 2.79 [1.47–5.32]; p=0.002). Treated patients did not have significantly different rates of decrease in forced vital capacity (FVC)>10% (OR: 1.03 [0.55–1.94]; p=0.93) nor in carbon monoxide diffusing capacity (DLCO) decrease. Patients having received rituximab were more prone to stop or decrease steroids (OR: 2.34 [1.56–3.53], pConclusionRituximab use was associated with a good safety profile in this large SSc-cohort. Significant change was observed on skin fibrosis, but not on lung. However, the limitation is the observational design. The potential stabilisation of lung fibrosis by rituximab has to be addressed by a randomised trial.

Published
2019

20. Phenotypes determined by cluster analysis and their survival in the prospective European Scleroderma Trials and Research cohort of patients with systemic sclerosis

Author

Sobanski, Vincent, Giovannelli, Jonathan, Allanore, Yannick, Riemekasten, Gabriela, Airo, Paolo, Vettori, Serena, Cozzi, Franco, Distler, Oliver, Matucci-Cerinic, Marco, Denton, Christopher, Launay, David, Hachulla, Eric, Cerinic, Marco Matucci, Guiducci, Serena, Walker, Ulrich, Kyburz, Diego, Lapadula, Giovanni, Iannone, Florenzo, Maurer, Britta, Jordan, Suzana, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Cutolo, Maurizio, Sulli, Alberto, Valentini, Gabriele, Cuomo, Giovanna, Siegert, Elise, Rednic, Simona, Nicoara, Ileana, Kahan, Andre, Vlachoyiannopoulos, Panayiotis, Montecucco, Carlo, Caporali, Roberto, Stork, Jiri, Inanc, Murat, Carreira, Patricia E, Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Kucharz, Eugene J, Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Wu, Chen, Marjanovic, Zora, Faivre, Helene, Hij, Darin, Dhamadi, Roza, Hesselstrand, Roger, Wollheim, Frank, Wuttge, Dirk M, Andreasson, Kristofer, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Trotta, Francesco, Lo Monaco, Andrea, Hunzelmann, Nicolas, Pellerito, Raffaele, Bambara, Lisa Maria, Caramaschi, Paola, Morovic-Vergles, Jadranka, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthias, Hasler, Paul, Burkhardt, Harald, Himsel, Andrea, Bajocchi, Gianluigi, Nuova, Arcispedale Santa Maria, Salvador, Maria Joao, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Stankovic, Aleksandra, Selmi, Carlo Francesco, De Santis, Maria, Marasini, Bianca, Tikly, Mohammed, Ananieva, Lidia P, Denisov, Lev N, Mueller-Ladner, Ulf, Frerix, Marc, Tarner, Ingo, Scorza, Raffaella, Puppo, Francesco, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szucs, Gabriella, Szamosi, Szilvia, Zea Mendoza, Antonio, de la Puente, Carlos, Sifuentes Giraldo, Walter Alberto, Midtvedt, Oyvind, Reiseter, Silje, Garen, Torhild, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Wigley, Fredrick M, Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Soare, Alina, Gorga, Marilena, Bojinca, Mihai, Mihai, Carina, Milicescu, Mihaela, Sunderkoetter, Cord, Kuhn, Annegret, Sandorfi, Nora, Schett, Georg, Distler, Joerg HW, Beyer, Christian, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, von Muehlen, Carlos Alberto, Bohn, Jussara Marilu, Lonzetti, Lilian Scussel, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Houssiau, Frederic A, Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Westhovens, Rene, de Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Granel, Brigitte, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Jimenez, Sergio, Busquets, Joanna, Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Popa, Sergei, Zenone, Thierry, Pileckyte, Margarita, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Sampaio-Barros, Percival D, Yoshinari, Natalino H, Marangoni, Roberta G, Martin, Patricia, Fuocco, Luiza, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Mohamed, Walid Ahmed Abdel Atty, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Chirieac, Rodica, Ancuta, Codrina, Furst, Daniel E, Villiger, Peter, Adler, Sabine, van Laar, Jacob, Kayser, Cristiane, Eduardo, Andrade Luis C, Fathi, Nihal, Hassanien, Manal, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Emery, Paul, Buch, Maya, Del Galdo, Francesco, Venalis, Algirdas, Butrimiene, Irena, Venalis, Paulius, Rugiene, Rita, Karpec, Diana, Saketkoo, Lesley Ann, Lasky, Joseph A, Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Rosner, Itzhak, Rozenbaum, Michael, Slobodin, Gleb, Boulman, Nina, Rimar, Doron, Couto, Maura, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Kahl, Sarah, Hsu, Vivien M, Chen, Fei, McCloskey, Deborah, Malveaux, Halina, Pasquali, Jean Louis, Martin, Thierry, Gorse, Audrey, Guffroy, Aurelien, Poindron, Vincent, EUSTAR Collaborators, Guiducci, S., Walker, U., Kyburz, D., Lapadula, G., Iannone, F., Maurer, B., Jordan, S., Becvar, R., Sierakowsky, S., Kowal Bielecka, O., Cutolo, M., Sulli, A., Valentini, G., Cuomo, G., Siegert, E., Rednic, S., Nicoara, I., Kahan, A., Vlachoyiannopoulos, P., Montecucco, C., Caporali, R., Stork, J., Inanc, M., Carreira, P.E., Novak, S., Czirják, L., Varju, C., Chizzolini, C., Kucharz, E.J., Kotulska, A., Kopec-Medrek, M., Widuchowska, M., Rozman, B., Mallia, C., Coleiro, B., Gabrielli, A., Farge, D., Wu, C., Marjanovic, Z., Faivre, H., Hij, D., Dhamadi, R., Airò, P., Hesselstrand, R., Wollheim, F., Wuttge, D.M., Andréasson, K., Martinovic, D., Balbir-Gurman, A., Braun-Moscovici, Y., Trotta, F., Lo Monaco, A., Hunzelmann, N., Pellerito, R., Mauriziano, O., Maria Bambara, L., Caramaschi, P., Morovic-Vergles, J., Black, C., Damjanov, N., Henes, J., Ortiz Santamaria, V., Heitmann, S., Krasowska, D., Seidel, M., Hasler, P., Burkhardt, H., Himsel, A., Bajocchi, G., Maria Nuova, A.S., João Salvador, M., Pereira Da Silva, J.A., Stamenkovic, B., Stankovic, A., Francesco Selmi, C., De Santis, M., Marasini, B., Tikly, M., Ananieva, L.P., Denisov, L.N., Müller-Ladner, U., Frerix, M., Tarner, I., Scorza, R., Puppo, F., Engelhart, M., Strauss, G., Nielsen, H., Damgaard, K., Szücs, G., Szamosi, S., Zea Mendoza, A., de la Puente, C., Sifuentes Giraldo, W.A., Midtvedt, Ø., Reiseter, S., Garen, T., Valesini, G., Riccieri, V., Maria Ionescu, R., Opris, D., Groseanu, L., Wigley, F.M., Sfrent Cornateanu, R., Ionitescu, R., Maria Gherghe, A., Soare, A., Gorga, M., Bojinca, M., Mihai, C., Milicescu, M., Sunderkötter, C., Kuhn, A., Sandorfi, N., Schett, G., Distler, J.H., Beyer, C., Meroni, P., Ingegnoli, F., Mouthon, L., De Keyser, F., Smith, V., Paolo Cantatore, F., Corrado, A., Ullman, S., Iversen, L., Alberto von Mühlen, C., Marilu Bohn, J., Scussel Lonzetti, L., Rosa Pozzi, M., Eyerich, K., Hein, R., Knott, E., Wiland, P., Szmyrka-Kaczmarek, M., Sokolik, R., Morgiel, E., Madej, M., Houssiau, F.A., Jose Alegre-Sancho, J., Krummel-Lorenz, B., Saar, P., Aringer, M., Günther, C., Westhovens, R., de Langhe, E., Lenaerts, J., Anic, B., Baresic, M., Mayer, M., Üprus, M., Otsa, K., Yavuz, S., Granel, B., Cezar Radominski, S., de Souza Müller, C., Azevedo, V.F., Jimenez, S., Busquets, J., Agachi, S., Groppa, L., Chiaburu, L., Russu, E., Popa, S., Zenone, T., Pileckyte, M., Stebbings, S., Highton, J., Mathieu, A., Vacca, A., Sampaio-Barros, P.D., Yoshinari, N.H., Marangoni, R.G., Martin, P., Fuocco, L., Stamp, L., Chapman, P., O'Donnell, J., Solanki, K., Doube, A., Veale, D., O'Rourke, M., Loyo, E., Li, M., Abdel Atty Mohamed, W.A., Rosato, E., Amoroso, A., Gigante, A., Oksel, F., Yargucu, F., Tanaseanu, C.M., Popescu, M., Dumitrascu, A., Tiglea, I., Foti, R., Chirieac, R., Ancuta, C., Furst, D.E., Villiger, P., Adler, S., van Laar, J., Kayser, C., Eduardo C, A.L., Fathi, N., Hassanien, M., de la Peña Lefebvre, P.G., Rodriguez Rubio, S., Valero Exposito, M., Sibilia, J., Chatelus, E., Gottenberg, J.E., Chifflot, H., Litinsky, I., Emery, P., Buch, M., Del Galdo, F., Venalis, A., Butrimiene, I., Venalis, P., Rugiene, R., Karpec, D., Ann Saketkoo, L., Lasky, J.A., Kerzberg, E., Montoya, F., Cosentino, V., Limonta, M., Luca Brucato, A., Lupi, E., Rosner, I., Rozenbaum, M., Slobodin, G., Boulman, N., Rimar, D., Couto, M., Spertini, F., Ribi, C., Buss, G., Kahl, S., Hsu, V.M., Chen, F., McCloskey, D., Malveaux, H., Louis Pasquali, J., Martin, T., Gorse, A., Guffroy, A., Poindron, V., and Chizzolini, Carlo

Subjects
0301 basic medicine, Male, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences, Databases, Factual, systemic sclerosis, SUBSETS, Disease, Severity of Illness Index, Scleroderma, DISEASE, 0302 clinical medicine, Medicine and Health Sciences, Immunology and Allergy, Cluster Analysis, CRITERIA, Prospective Studies, Prospective cohort study, skin and connective tissue diseases, integumentary system, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti, Adult, Aged, Autoantibodies/blood, Europe/epidemiology, Female, Humans, Middle Aged, Phenotype, Prognosis, Scleroderma, Diffuse/blood, Scleroderma, Diffuse/epidemiology, Scleroderma, Diffuse/pathology, Scleroderma, Limited/blood, Scleroderma, Limited/epidemiology, Scleroderma, Limited/pathology, Scleroderma, Systemic/blood, Scleroderma, Systemic/epidemiology, Scleroderma, Systemic/pathology, Connective tissue disease, ddc, Europe, MANIFESTATIONS, Cohort, Life Sciences & Biomedicine, medicine.medical_specialty, Immunology, PROFILE, CLASSIFICATION, 03 medical and health sciences, Rheumatology, Scleroderma, Limited, Internal medicine, Severity of illness, medicine, Autoantibodies, 030203 arthritis & rheumatology, Science & Technology, Scleroderma, Systemic, business.industry, Autoantibody, Systemic sclerosis (SSc), medicine.disease, 030104 developmental biology, Scleroderma, Diffuse, business
Abstract

OBJECTIVE: Systemic sclerosis (SSc) is a heterogeneous connective tissue disease that is typically subdivided into limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) depending on the extent of skin involvement. This subclassification may not capture the entire variability of clinical phenotypes. The European Scleroderma Trials and Research (EUSTAR) database includes data on a prospective cohort of SSc patients from 122 European referral centers. This study was undertaken to perform a cluster analysis of EUSTAR data to distinguish and characterize homogeneous phenotypes without any a priori assumptions, and to examine survival among the clusters obtained. METHODS: A total of 11,318 patients were registered in the EUSTAR database, and 6,927 were included in the study. Twenty-four clinical and serologic variables were used for clustering. RESULTS: Clustering analyses provided a first delineation of 2 clusters showing moderate stability. In an exploratory attempt, we further characterized 6 homogeneous groups that differed with regard to their clinical features, autoantibody profile, and mortality. Some groups resembled usual dcSSc or lcSSc prototypes, but others exhibited unique features, such as a majority of lcSSc patients with a high rate of visceral damage and antitopoisomerase antibodies. Prognosis varied among groups and the presence of organ damage markedly impacted survival regardless of cutaneous involvement. CONCLUSION: Our findings suggest that restricting subsets of SSc patients to only those based on cutaneous involvement may not capture the complete heterogeneity of the disease. Organ damage and antibody profile should be taken into consideration when individuating homogeneous groups of patients with a distinct prognosis. ispartof: ARTHRITIS & RHEUMATOLOGY vol:71 issue:9 pages:1553-1570 ispartof: location:United States status: published

Published
2019

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