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3. Benralizumab for eosinophilic granulomatosis with polyangiitis: a retrospective, multicentre, cohort study

Author

Bettiol, Alessandra, Urban, Maria Letizia, Padoan, Roberto, Groh, Matthieu, Lopalco, Giuseppe, Egan, Allyson, Cottin, Vincent, Fraticelli, Paolo, Crimi, Claudia, Del Giacco, Stefano, Losappio, Laura, Moi, Laura, Cinetto, Francesco, Caminati, Marco, Novikov, Pavel, Berti, Alvise, Cameli, Paolo, Cathébras, Pascal, Coppola, Angelo, Durel, Cécile-Audrey, Folci, Marco, Gullo, Alberto Lo, Lombardi, Carlo, Monti, Sara, Parronchi, Paola, Rivera, Carlos Martinez, Solans, Roser, Vacca, Angelo, Espígol-Frigolé, Georgina, Guarnieri, Gabriella, Bianchi, Fulvia Chieco, Marchi, Maria Rita, Tcherakian, Colas, Kahn, Jean-Emmanuel, Iannone, Florenzo, Venerito, Vincenzo, Desaintjean, Charlene, Moroncini, Gianluca, Nolasco, Santi, Costanzo, Giulia Anna Maria Luigia, Schroeder, Jan Walter, Ribi, Camillo, Tesi, Michelangelo, Gelain, Elena, Mattioli, Irene, Bello, Federica, Jayne, David, Prisco, Domenico, Vaglio, Augusto, and Emmi, Giacomo

Published
2023
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4. Cohort Enrichment Strategies for Progressive Interstitial Lung Disease in Systemic Sclerosis From European Scleroderma Trials and Research

Author

Vacca, Alessandra, Giollo, Alessandro, Balbir-Gurman, Alexandra, Gheorghiu, Ana Maria, Marcoccia, Antonella, Herrick, Ariane, Radic, Mislav, Stamenkovic, Bojana, Anic, Branimir, Granel, Brigitte, Ribi, Camillo, Selmi, Carlo Francesco, Carlos de la Puente, Milano, de Souza Müller, Carolina, Denton, Christopher, Kayser, Cristiane, Tanaseanu, Cristina-Mihaela, Majewski, Dominik, Rimar, Doron, Krasowska, Dorota, Veale, Douglas, Walker, Ulrich, Kerzberg, Eduardo, Rezus, Elena, Zanatta, Elisabetta, Siegert, Elise, De Langhe, Ellen, Oksel, Fahrettin, Ingegnoli, Francesca, Cantatore, Francesco Paolo, Szücs, Gabriela, Cuomo, Giovanna, Seskute, Goda, Litinsky, VilniusIra, Castellví, Ivan, Morovic-Vergles, Jadranka, Sibilia, Jean, Henes, Jörg, Solanki, Kamal, Perdan-Pirkmajer, Katja, Herrmann, Kristine, Saketkoo, Lesley Ann, Stamp, Lisa, Mouthon, Luc, Salvador, Maria João, Pozzi, Maria Rosa, Üprus, Maria, Vanthuyne, Marie, Engelhart, Merete, Köhm, Michaela, Iudici, Michele, Inanc, Murat, Fathi, Nihal, Pamuk, Nuri, García de la Peña Lefebv, Paloma, Carreira, Patricia E., Bancel, Dominique Farge, Moroncini, Luca, Montecucco, C., Ancuta, Codrina, Sunderkötter, Cord, Müller-Ladner, Ulf, Rosato, Edoardo, Kucharz, Eugene J., Iannone, Florenzo, Del Galdo, Francesco, Poormoghim, Hadi, Kötter, Ina, Distler, Jörg, Cutolo, Maurizio, Tikly, Mohammed, Damjanov, Nemanja, Hunzelmann, Nicolas, Vlachoyiannopoulos, P., Hasler, Paul, Sarzi Puttini, Piercarlo, Wiland, Piotr, Becvar, Radim, Yavuz, Sule, Zdrojewski, Zbigniew, Pellerito, Raffaele, Foti, Rosario, Ionescu, Ruxandra Maria, Adler, Sabine, Kahl, Sarah, Moiseev, Sergey, Stebbings, Simon, Rednic, Simona, Negrini, Simone, Heitmann, Stefan, Ullman, Susanne, Agachi, Svetlana, Martin, Thierry, Schmeiser, Tim, Riccieri, Valeria, Smith, Vanessa, Bernardino, Vera, Ortiz-Santamaria, Vera, Hsu, Vivien M., Abdel Atty Mohamed, Walid Ahmed, Hoffmann-Vold, Anna-Maria, Brunborg, Cathrine, Airò, Paolo, Ananyeva, Lidia P., Czirják, László, Guiducci, Serena, Hachulla, Eric, Li, Mengtao, Mihai, Carina, Riemekasten, Gabriela, Sfikakis, Petros P., Valentini, Gabriele, Kowal-Bielecka, Otylia, Allanore, Yannick, and Distler, Oliver

Published
2023
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5. Physician Global Assessment International Standardisation COnsensus in Systemic Lupus Erythematosus: the PISCOS study

Author

Appenzeller, Simone, Aranow, Cynthia, Askanase, Anca, Avcin, Tadej, Bae, Sang-Cheol, Bertsias, George, Bonfa, Eloisa, Cairoli, Ernesto, Cardiel, Mario H, Cervera, Ricard, Chasset, François, Chizzolini, Carlo, Clarke, Ann E, Conti, Fabrizio, Costedoat-Chalumeau, Nathalie, Czirják, László, Doria, Andrea, Dörner, Thomas, Espinosa, Gerard, Fischer-Betz, Rebecca, Garcìa, Mercedes, Gladman, Dafna D, González, Luis A, Gunnarsson, Iva, Hamijoyo, Laniyati, Hanly, John G, Hasni, Sarfaraz A, Houssiau, Frédéric A, Inanç, Murat, Inês, Luís S, Isenberg, David, Jacobsen, Soren, Jan Wu, Yeong-Jian, Kaneko, Yuko, Katsumata, Yasuhiro, Lau, Chak S, Legge, Alexandra C, Lerang, Karoline, Limper, Maarten, Louthrenoo, Worawit, Luo, Shue-Fen, Marinho, António, Massardo, Loreto, Mathian, Alexis, Mosca, Marta, Nikpour, Mandana, Pego-Reigosa, José M, Peschken, Christine A, Pons-Estel, Bernardo A, Pons-Estel, Guillermo J, Rahman, Anisur, Rednic, Simona, Ribi, Camillo, Ruiz-Irastorza, Guillermo, Sato, Emilia I, Saxena, Amit, Schneider, Matthias, Sebastiani, Gian Domenico, Strand, Vibeke, Svenungsson, Elisabet, Tanaka, Yoshiya, Tazi Mezalek, Zoubida, Tee, Michael L, Tincani, Angela, Touma, Zahi, Troldborg, Anne, Vasconcelos, Carlos, Vinet, Évelyne, Vital, Edward M, Voskuyl, Alexandre E, Voss, Anne, Wallace, Daniel, Ward, Michael, Zamora, Leonid D, Piga, Matteo, Chessa, Elisabetta, Morand, Eric F, Ugarte-Gil, Manuel F, Tektonidou, Maria, van Vollenhoven, Ronald, Petri, Michelle, and Arnaud, Laurent

Published
2022
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7. Factor H-related protein 1 in systemic lupus erythematosus.

Author

Kleer, Jessica S., Klehr, Juliane, Dubler, Denise, Infanti, Laura, Chizzolini, Carlo, Huynh-Do, Uyen, Ribi, Camillo, and Trendelenburg, Marten

Subjects
SYSTEMIC lupus erythematosus, COMPLEMENT inhibition, COMPLEMENT activation, AUTOANTIBODIES, WESTERN immunoblotting
Abstract

Background: Factor H (FH) is a major soluble inhibitor of the complement system and part of a family comprising five related proteins (FHRs 1-5). Deficiency of FHR1 was described to be linked to an elevated risk of systemic lupus erythematosus (SLE). As FHR1 can partially antagonize the functionality of FH, an altered FHR1/FH ratio could not only enhance SLE vulnerability but also affect the disease expression. This study focuses on the analysis of FH and FHR1 at a protein level, and the occurrence of anti-FH autoantibodies (anti-FH) in a large cohort of SLE patients to explore their association with disease activity and/or expression. Methods: We assessed FH and FHR1 levels in plasma from 378 SLE patients compared to 84 healthy controls (normal human plasma, NHP), and sera from another cohort of 84 healthy individuals (normal human serum, NHS), using RayBio® CFH and CFHR1 ELISA kits. Patients were recruited by the Swiss SLE Cohort Study (SSCS). Unmeasurable FHR1 levels were all confirmed by Western blot, and in a subgroup of patients by PCR. Anti-FH were measured in SLE patients with non-detectable FHR1 levels and matched control patients using Abnova's CFH IgG ELISA kit. Results: Overall, FH and FHR1 levels were significantly higher in healthy controls, but there was no significant difference in FHR1/FH ratios between SLE patients and NHPs. However, SLE patients showed a significantly higher prevalence of undetectable FHR1 compared to all healthy controls (35/378 SLE patients versus 6/168 healthy controls; p= 0.0214, OR=2.751, 95% CI = 1.115 - 8.164), with a consistent trend across all ethnic subgroups. Levels of FH and FHR1, FHR1/FH ratios and absence of FHR1 were not consistently associated with disease activity and/or specific disease manifestations, but absence of FHR1 (primarily equivalent to CFHR1 deficiency) was linked to the presence of anti-FH in SLE patients (p=0.039). Conclusions: Deficiency of FHR1 is associated with a markedly elevated risk of developing SLE. A small proportion of FHR1-deficient SLE patients was found to have autoantibodies against FH but did not show clinical signs of microangiopathy. [ABSTRACT FROM AUTHOR]

Published
2024
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8. Therapeutic management of fibrosis in systemic sclerosis patients – an analysis from the Swiss EUSTAR cohort

Author

Windirsch, Kevin, primary, Jordan, Suzana, additional, Becker, Mike Oliver, additional, Bruni, Cosimo, additional, Dobrota, Rucsandra, additional, Elhai, Muriel, additional, Garaiman, Ion-Alexandru, additional, Mihai, Carmen-Marina, additional, Iudici, Michele, additional, Hasler, Paul, additional, Ribi, Camillo, additional, Maurer, Britta, additional, Gabrielli, Armando, additional, Hoffmann-Vold, Anna-Maria, additional, and Distler, Oliver, additional

Published
2024
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9. Therapeutic management of fibrosis in systemic sclerosis patients - an analysis from the Swiss EUSTAR cohort

Author

Windirsch, Kevin, Jordan, Suzana; https://orcid.org/0000-0002-8114-8239, Becker, Mike Oliver; https://orcid.org/0000-0001-9102-3088, Bruni, Cosimo; https://orcid.org/0000-0003-2813-2083, Dobrota, Rucsandra; https://orcid.org/0000-0001-9819-7574, Elhai, Muriel; https://orcid.org/0000-0001-8627-5758, Garaiman, Ion-Alexandru, Mihai, Carmen-Marina; https://orcid.org/0000-0002-8627-8817, Iudici, Michele, Hasler, Paul; https://orcid.org/0000-0001-7179-3018, Ribi, Camillo, Maurer, Britta; https://orcid.org/0000-0001-9385-8097, Gabrielli, Armando, Hoffmann-Vold, Anna-Maria; https://orcid.org/0000-0001-6467-7422, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Windirsch, Kevin, Jordan, Suzana; https://orcid.org/0000-0002-8114-8239, Becker, Mike Oliver; https://orcid.org/0000-0001-9102-3088, Bruni, Cosimo; https://orcid.org/0000-0003-2813-2083, Dobrota, Rucsandra; https://orcid.org/0000-0001-9819-7574, Elhai, Muriel; https://orcid.org/0000-0001-8627-5758, Garaiman, Ion-Alexandru, Mihai, Carmen-Marina; https://orcid.org/0000-0002-8627-8817, Iudici, Michele, Hasler, Paul; https://orcid.org/0000-0001-7179-3018, Ribi, Camillo, Maurer, Britta; https://orcid.org/0000-0001-9385-8097, Gabrielli, Armando, Hoffmann-Vold, Anna-Maria; https://orcid.org/0000-0001-6467-7422, and Distler, Oliver; https://orcid.org/0000-0002-0546-8310

Abstract

OBJECTIVES Systemic sclerosis is a chronic autoimmune connective tissue disease leading to microvascular and fibrotic manifestations in multiple organs. Several treatment options and recommendations from different European countries are available. In this study, for which the ambit is Switzerland specifically, we aim to describe the treatment patterns of systemic sclerosis patients with fibrotic manifestations. METHODS Systemic sclerosis patients were selected from six Swiss tertiary centres recorded in the multicentre, prospective European Scleroderma Trials and Research (EUSTAR) registry. Patients fulfilling the 2013 ACR/EULAR systemic sclerosis classification criteria at baseline were included. To determine the differences in treatment of varying degrees of fibrosis, four groups were identified: (1) patients with a modified Rodnan skin score (mRSS) >0; (2) those with mRSS ≥7; (3) those with interstitial lung disease (SSc-ILD), diagnosed by either chest X-Ray or high-resolution computed tomography; and (4) patients fulfilling one of the additional criteria for extensive interstitial lung disease, defined as interstitial lung disease involvement of >20% in high-resolution computed tomography, dyspnea NYHA-stage 3/4, or a predicted forced vital capacity (FVC) of <70%. RESULTS A total of 590 patients with systemic sclerosis fulfilled the inclusion criteria. In this cohort, 421 (71.4%) had mRSS >0, of whom 195 (33.1%) had mRSS ≥7; interstitial lung disease was diagnosed in 198 of 456 (43.4%), of whom 106 (18.0 %) showed extensive interstitial lung disease. Regarding non-biologic disease-modifying medications (DMARDs), the most frequently prescribed was methotrexate, followed by hydroxychloroquine and mycophenolate mofetil. Rituximab and tocilizumab were most frequently used among the biologic DMARDs. Specifically, 148/372 (39.8%) of treated patients with skin fibrosis received methotrexate, mycophenolate mofetil or rituximab, and 80/177 (45.2%) with interstitial lung

Published
2024

10. Mitochondrial Dysfunction in Systemic Lupus Erythematosus with a Focus on Lupus Nephritis.

Author

Halfon, Matthieu, Tankeu, Aurel T., and Ribi, Camillo

Subjects
SYSTEMIC lupus erythematosus, LUPUS nephritis, MITOCHONDRIAL DNA, MITOCHONDRIA, MITOCHONDRIAL dynamics, HOMEOSTASIS
Abstract

Systemic lupus erythematosus (SLE) is an autoimmune disease affecting mostly women of child-bearing age. Immune dysfunction in SLE results from disrupted apoptosis which lead to an unregulated interferon (IFN) stimulation and the production of autoantibodies, leading to immune complex formation, complement activation, and organ damage. Lupus nephritis (LN) is a common and severe complication of SLE, impacting approximately 30% to 40% of SLE patients. Recent studies have demonstrated an alteration in mitochondrial homeostasis in SLE patients. Mitochondrial dysfunction contributes significantly to SLE pathogenesis by enhancing type 1 IFN production through various pathways involving neutrophils, platelets, and T cells. Defective mitophagy, the process of clearing damaged mitochondria, exacerbates this cycle, leading to increased immune dysregulation. In this review, we aim to detail the physiopathological link between mitochondrial dysfunction and disease activity in SLE. Additionally, we will explore the potential role of mitochondria as biomarkers and therapeutic targets in SLE, with a specific focus on LN. In LN, mitochondrial abnormalities are observed in renal cells, correlating with disease progression and renal fibrosis. Studies exploring cell-free mitochondrial DNA as a biomarker in SLE and LN have shown promising but preliminary results, necessitating further validation and standardization. Therapeutically targeting mitochondrial dysfunction in SLE, using drugs like metformin or mTOR inhibitors, shows potential in modulating immune responses and improving clinical outcomes. The interplay between mitochondria, immune dysregulation, and renal involvement in SLE and LN underscores the need for comprehensive research and innovative therapeutic strategies. Understanding mitochondrial dynamics and their impact on immune responses offers promising avenues for developing personalized treatments and non-invasive biomarkers, ultimately improving outcomes for LN patients. [ABSTRACT FROM AUTHOR]

Published
2024
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11. ANCA-associated vasculitides: Recommendations of the French Vasculitis Study Group on the use of immunosuppressants and biotherapies for remission induction and maintenance

Author

Terrier, Benjamin, Charles, Pierre, Aumaître, Olivier, Belot, Alexandre, Bonnotte, Bernard, Crabol, Yoann, Durel, Cécile-Audrey, Ebbo, Mikael, Jourde-Chiche, Noémie, Lega, Jean-Christophe, Puéchal, Xavier, Pugnet, Grégory, Quemeneur, Thomas, Ribi, Camillo, Samson, Maxime, Vandergheynst, Frédéric, and Guillevin, Loïc

Published
2020
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23. Case report: First-in-human combined low-dose whole-heart irradiation and high-dose stereotactic arrhythmia radioablation for immunosuppressive refractory cardiac sarcoidosis and ventricular tachycardia

Author

van der Ree, Martijn H., primary, Herrera Siklody, Claudia, additional, Le Bloa, Mathieu, additional, Pascale, Patrizio, additional, Porretta, Alessandra P., additional, Teres, Cheryl C., additional, Solana Munoz, Jorge, additional, Luca, Adrian, additional, Domenichini, Giulia, additional, Ozasahin, Mahmut, additional, Jumeau, Raphael, additional, Postema, Pieter G., additional, Ribi, Camillo, additional, Bourhis, Jean, additional, Schiappacasse, Luis, additional, and Pruvot, Etienne, additional

Published
2023
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25. Systemic Lupus Erythematosus

Author

Baglivo, Edoardo, Ribi, Camillo, Zierhut, Manfred, editor, Pavesio, Carlos, editor, Ohno, Shigeaki, editor, Orefice, Fernando, editor, and Rao, Narsing A., editor

Published
2016
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28. Cutaneous Manifestations, Clinical Characteristics, and Prognosis of Patients With Systemic Sclerosis Sine Scleroderma: Data From the International EUSTAR Database

Author

Lescoat, Alain, Huang, Suiyuan, Carreira, Patricia, Siegert, Elise, de Vries-Bouwstra, Jeska, Distler, Jörg, Smith-Castro, Vanessa, del Galdo, Francesco, Anic, Branimir, Damjanov, Nemanja, Rednic, Simona, Ribi, Camillo, Bancel, Pr Dominique Farge, Hoffmann-Vold, Anna-Maria, Gabrielli, Armando, Distler, Oliver, Khanna, Dinesh, Allanore, Yannick, 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 ), CHU Pontchaillou [Rennes], University of Michigan [Ann Arbor], University of Michigan System, Charité - UniversitätsMedizin = Charité - University Hospital [Berlin], Leiden University Medical Center (LUMC), Universiteit Leiden, Ghent University Hospital, University of Leeds, Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), McGill University = Université McGill [Montréal, Canada], University hospital of Zurich [Zurich], Institut Cochin (IC UM3 (UMR 8104 / U1016)), and Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPCité)

Subjects
[SDV]Life Sciences [q-bio]
Abstract

International audience; IMPORTANCE: Systemic sclerosis (SSc) sine scleroderma (ssSSc) is a subset of SSc defined by the absence of skin fibrosis. Little is known about the natural history and skin manifestations among patients with ssSSc. OBJECTIVE: To characterize the clinical phenotype of patients with ssSSc compared with patients with limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) within the EUSTAR database. DESIGN, SETTING, AND PARTICIPANTS: This longitudinal observational cohort study based on the international EUSTAR database included all patients fulfilling the classification criteria for SSc assessed by the modified Rodnan Skin score (mRSS) at inclusion and with at least 1 follow-up visit; ssSSc was defined by the absence of skin fibrosis (mRSS = 0 and no sclerodactyly) at all available visits. Data extraction was performed in November 2020, and data analysis was performed from April 2021 to April 2023. MAIN OUTCOMES AND MEASURES: Main outcomes were survival and skin manifestations (onset of skin fibrosis, digital ulcers, telangiectasias, puffy fingers). RESULTS: Among the 4263 patients fulfilling the inclusion criteria, 376 (8.8%) were classified as having ssSSc (mean [SD] age, 55.3 [13.9] years; 345 [91.8%] were female). At last available visit, in comparison with 708 patients with lcSSc and 708 patients with dcSSc with the same disease duration, patients with ssSSc had a lower prevalence of previous or current digital ulcers (28.2% vs 53.1% in lcSSc; P 40%) and SSc renal crisis (almost 3%). Patients with ssSSc had a higher survival than other subsets. Dermatologists should be aware that cutaneous findings in this subgroup may be associated with internal organ dysfunction. In particular, skin telangiectasias in ssSSc were associated with diastolic heart dysfunction.

Published
2023

33. Myositis manifesting as respiratory insufficiency with anti-RNA polymerase III antibodies: an unusual presentation of graft-versus-host disease.

Author

Wolff, Louis, Clottu, Aurélie, Mamez, Anne-Claire, Ribi, Camillo, and Grandoni, Francesco

Subjects
MYASTHENIA gravis, SYMPTOMS, GRAFT versus host disease, MYOSITIS, RESPIRATORY insufficiency, ETIOLOGY of diseases
Abstract

Myositis, a distinctive feature of chronic GVHD, is rare and estimated to occur in 23 per 100.000 persons-years among GVHD, compared to 1 per 100.000 person-years in the general population [[2]]. The final diagnosis was an acute respiratory failure caused by diaphragm paresis due to myositis as an unusual presentation of chronic GVHD. We report the management of a patient treated for acute leukemia who developed a rare manifestation of chronic graft-versus-host disease (GVHD). [Extracted from the article]

Published
2023
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34. Granulomatose avec polyangéite : quoi de neuf ?

Author

Ringwald, Maxime, primary, Chevalley, Dehlia, additional, Bongard, Cédric, additional, Kissling, Sébastien, additional, Rotman, Samuel, additional, Von Garnier, Christophe, additional, Ribi, Camillo, additional, and Comte, Denis, additional

Published
2023
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38. Phenotype of limited cutaneous systemic sclerosis patients with positive anti-topoisomerase I antibodies: data from EUSTAR cohort

Author

Zanatta, Elisabetta, Huscher, Dörte, Ortolan, Augusta, Avouac, Jérôme, Airó, Paolo, Balbir-Gurman, Alexandra, Siegert, Elise, Cerinic, Marco Matucci, Cozzi, Franco, Riemekasten, Gabriela, Hoffmann-Vold, Anna-Maria, Distler, Oliver, Gabrielli, Armando, Heitmann, Stefan, Hunzelmann, Nicolas, Montecucco, Carlomaurizio, Morovic-Vergles, Jadranka, Ribi, Camillo, Doria, Andrea, Allanore, Yannick, EUSTAR collaborators, and University of Zurich

Subjects
10051 Rheumatology Clinic and Institute of Physical Medicine, 610 Medicine & health
Published
2022

40. Des reins sous pression

Author

Cancela Costa, Alicia, primary, Kissling, Sbastien, additional, Ribi, Camillo, additional, Rotman, Samuel, additional, Vogel, Grard, additional, and Pongan, Damiano, additional

Published
2022
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41. Nieren unter Druck

Author

Cancela Costa, Alicia, primary, Kissling, Sbastien, additional, Ribi, Camillo, additional, Rotman, Samuel, additional, Vogel, Grard, additional, and Pongan, Damiano, additional

Published
2022
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43. List of Contributors

Author

Abrahamian, Fredrick M., primary, Aldape, Michael J., additional, Aldasoro, Edelweiss, additional, Allen, Upton D., additional, Al-Sum, Hythem, additional, Anadkat, Milan J., additional, Anders, Katherine, additional, Angelakis, Emmanouil, additional, Angus, Brian John, additional, Antoniadou, Anastasia, additional, Arena, Fabio, additional, Arends, Joop E., additional, Arribas, Jose R., additional, Artenstein, Andrew W., additional, Atherton, John C., additional, Aucott, John N., additional, Aw, Tar-Ching, additional, Babcock, Hilary M., additional, Bailey, Robin, additional, Bailey, Thomas C., additional, Banks, Adam Z., additional, Barillo, David J., additional, Barrette, Ernie-Paul, additional, Bauer, Martijn P., additional, Bayston, Roger, additional, Beard, C. Ben, additional, Beardsley, Justin, additional, Beeching, Nick J., additional, Bégué, Rodolfo E., additional, Beldi, Guido, additional, Benson, Constance A., additional, Berbari, Elie F., additional, Berenger, Jean-Michel, additional, Berger, Christoph, additional, Bernardino, Jose I., additional, Bille, Jacques, additional, Billioux, Alexander C., additional, Bitnun, Ari, additional, Blair, Iain, additional, Blanche, Stéphane, additional, Bleck, Thomas P., additional, Bleeker-Rovers, Chantal P., additional, Bleijenberg, Gijs, additional, Bloch, Karen C., additional, Blum, Johannes, additional, Blumberg, Emily A., additional, Bonomo, Robert A., additional, Bonten, Marc J.M., additional, Bourayou, Rafik, additional, Bouza, Emilio, additional, Brandt, K. Ashley, additional, Bretelle, Florence, additional, Brisse, Sylvain, additional, Britton, Warwick J., additional, Brook, Itzhak, additional, Brouwer, Matthijs C., additional, Browne, Sarah K., additional, Bryant, Amy E., additional, Bühler, Silja, additional, Bulger, Eileen M., additional, Buller, R. Mark L., additional, Burke, Leah A., additional, Burri, Christian, additional, Butler, Marcus W., additional, Calandra, Thierry, additional, Calfee, David P., additional, Calvo-Cano, Antonia, additional, Cameron, D. William, additional, Carcillo, Joseph A., additional, Carson, Gail, additional, Chambers, Stephen T., additional, Charrel, Remi N., additional, Nguyen, Vinh Chau Van, additional, Chevaliez, Stéphane, additional, Chiller, Tom M., additional, Christaki, Eirini, additional, Chung, Kevin K., additional, Clifford, David B., additional, Clumeck, Nathan, additional, Cohen, Jonathan, additional, Collinge, John, additional, Conlon, Christopher P., additional, Conrad, Curdin, additional, Cooke, Fiona J., additional, Cope, Jennifer Rittenhouse, additional, Corey, G. Ralph, additional, Cross, John H., additional, Cunha, Burke A., additional, Cunha, Cheston B., additional, D'Journo, Benoit, additional, Daikos, George L., additional, Daniels, Johannes M.A., additional, Davidson, Robert N., additional, Day, Nicholas P.J., additional, De Cock, Kevin M., additional, de Silva, Thushan I., additional, de Vries, Henry J.C., additional, de Wit, Stéphane, additional, Delaloye, Julie, additional, Denning, David W., additional, Dennis, David T., additional, Dhanireddy, Shireesha, additional, Dielubanza, Elodi J., additional, Diemert, David J., additional, Doganay, Mehmet, additional, Doherty, Tom, additional, Dolecek, Christiane, additional, Dondorp, Arjen M., additional, Douglas, Abby, additional, Drancourt, Michel, additional, Dubourg, Grégory, additional, Dudley, Michael N., additional, Durand, Guillaume, additional, Eckhardt, Benjamin J., additional, Efstratiou, Androulla, additional, Ekkelenkamp, Miquel B., additional, Eranki, Ambika, additional, Erdem, Hakan, additional, Escota, Gerome V., additional, Evans, Heather L., additional, Eziefula, Alice Chijioke, additional, Fenollar, Florence, additional, Fenwick, Alan, additional, Fierer, Joshua, additional, Finch, Roger G., additional, Fleckenstein, James M., additional, Forstner, Christina, additional, Foschi, Federico, additional, Fournier, Pierre-Edouard, additional, French, Martyn A., additional, Gage, Kenneth L., additional, Garcia, Lynne S., additional, Gascon, Joaquim, additional, Gastañaduy, Arturo S., additional, Gautret, Philippe, additional, Geisler, William M., additional, Ghanem, Khalil G., additional, Giani, Tommaso, additional, Giannella, Maddalena, additional, Gilliam, Bruce L., additional, Gilliet, Michel, additional, Glaser, Carol A., additional, Glupczynski, Youri, additional, Gnann, John W., additional, Goldstein, Ellie J.C., additional, Gottstein, Bruno, additional, Gouriet, Frederique, additional, Gravitt, Patti E., additional, Green, Michael D., additional, Green, Stephen T., additional, Groll, Andreas H., additional, Gulick, Roy M., additional, Gupta, Arjun, additional, Habib, Gilbert, additional, Harbarth, Stephan, additional, Harris, Marianne, additional, Hayden, Frederick G., additional, Hetem, David J., additional, Hill, Philip C., additional, Hirschel, Bernard, additional, Hodowanec, Aimee C., additional, Hoffart, Louis, additional, Hoffmann, Christian, additional, Holland, Steven M., additional, Horby, Peter W., additional, Horne, David J., additional, Hraiech, Sami, additional, Hull, Mark W., additional, Huttner, Angela, additional, Ingram, Richard J.M., additional, Islam, Jasmin, additional, Ison, Michael G., additional, James, Scott H., additional, Jenkins, Claire, additional, Jenkins, Stephen G., additional, Jensen, Jørgen Skov, additional, Johnston, Christine, additional, Jones, Theodore B., additional, Jordan, Stephen J., additional, Julian, Kathleen G., additional, Kato, Yasuyuki, additional, Kauffman, Carol A., additional, Kaye, Keith S., additional, Keane, Michael P., additional, Keeney, James, additional, Kelly, Paul, additional, Kent, Stephen J., additional, Kern, Winfried V., additional, Keynan, Yoav, additional, Kim, Andrea A., additional, Koné-Paut, Isabelle, additional, Kosmidis, Chris, additional, Kroes, Aloys C.M., additional, Kroon, Frank P., additional, Ksiazek, Thomas G., additional, Kuhlmann, F. Matthew, additional, Kuijper, Ed J., additional, Kwon, Jennie H., additional, Kyei, George B., additional, Lacombe, Karine, additional, Lagacé-Wiens, Philippe, additional, Lagier, Jean-Christophe, additional, Lamagni, Theresa, additional, Landraud, Luce, additional, Lanternier, Fanny, additional, LaPlante, Kerry L., additional, Lawn, Stephen D., additional, Lawrence, Steven J., additional, Leblebicioglu, Hakan, additional, Lee, Nelson, additional, Leggett, James E., additional, Lehours, Philippe, additional, Levy, Pierre-Yves, additional, Leyh, Rainer G., additional, Lillis, Rebecca A., additional, Limmathurotsakul, Direk, additional, Lin, Jennifer, additional, Lindquist, H.D. Alan, additional, Lipsky, Benjamin A., additional, Liscynesky, Christina, additional, Looney, David, additional, Lortholary, Olivier, additional, Lowy, Franklin D., additional, Luft, Benjamin J., additional, Mackowiak, Philip A., additional, MacPherson, Paul A., additional, Maghraoui-Slim, Valérie, additional, Mallon, Patrick W., additional, Mangino, Julie E., additional, Manuel, Oriol, additional, Marchetti, Oscar, additional, Marks, Kristen M., additional, Marr, Kieren A., additional, Marrazzo, Jeanne, additional, Marschall, Jonas, additional, Martin, David H., additional, Matonti, Frédéric, additional, Matulewicz, Richard S., additional, Mayer, Kenneth H., additional, McCulloh, Russell J., additional, McGready, Rose, additional, Mdodo, Rennatus, additional, Mead, Simon, additional, Mégraud, Francis, additional, Meintjes, Graeme, additional, Metcalf, Sarah C., additional, Michaels, Marian G., additional, Migliori, Giovanni Battista, additional, Miles, Michael A., additional, Miller, Alastair, additional, Mimiaga, Matthew J., additional, Mingeot-Leclercq, Marie-Paule, additional, Misch, Elizabeth Ann, additional, Mitreva, Makedonka, additional, Montaner, Julio S.G., additional, Moore, Caroline B., additional, Muñoz, Patricia, additional, Muñoz, Jose, additional, Murray, Clinton K., additional, Musso, Didier, additional, Mutengo, Mable, additional, Mutizwa, Misha M., additional, Naber, Kurt G., additional, Natarajan, Pavithra, additional, Neme, Santiago, additional, Newton, Paul N., additional, Nichols, Ronald A., additional, Nicolle, Lindsay E., additional, Nosten, François, additional, Notarangelo, Luigi D., additional, Nutman, Thomas B., additional, Nyirjesy, Paul, additional, O'Connell, P. Ronan, additional, Opal, Steven M., additional, Ormerod, L. Peter, additional, Osmon, Douglas R., additional, Pankert, Marie Boulze, additional, Pantaleo, Giuseppe, additional, Papazian, Laurent, additional, Parente, Diane M., additional, Parola, Philippe, additional, Parsaei, Shadi, additional, Pascual, Manuel A., additional, Patel, Rupa, additional, Patrozou, Eleni, additional, Pawlotsky, Jean-Michel, additional, Peacock, Sharon J., additional, Pechère, Jean-Claude, additional, Pelegrin, Ivan, additional, Peters, Barry S., additional, Peters, Edgar J.G., additional, Petersen, Jeannine M., additional, Petersen, Lyle R., additional, Petraitis, Vidmantas, additional, Pham, Luu-Ly, additional, Picado, Albert, additional, Pilatz, Adrian, additional, Pilmis, Benoit, additional, Pinazo, María-Jesús, additional, Pletz, Mathias W., additional, Pogue, Jason M., additional, Polgreen, Evelyn L., additional, Polgreen, Philip M., additional, Posfay-Barbe, Klara M., additional, Powderly, William G., additional, Presti, Rachel, additional, Prod'hom, Guy, additional, Puolakkainen, Mirja, additional, Quinn, Thomas C., additional, Raoult, Didier, additional, Razonable, Raymund R., additional, Read, Robert C., additional, Redfield, Robert R., additional, Rentenaar, Rob J., additional, Reynolds, Steven J., additional, Ribi, Camillo, additional, Richardson, Malcolm D., additional, Ritter, Michele L., additional, Roch, Antoine, additional, Rockstroh, Jürgen Kurt, additional, Rojek, Amanda, additional, Romero, José R., additional, Rooijakkers, Suzan H.M., additional, Rosenbluth, Daniel, additional, Rosenzweig, Sergio D., additional, Rossolini, Gian Maria, additional, Rubinstein, Ethan, additional, Ryan, Greg, additional, Safren, Steven A., additional, Sahasrabuddhe, Vikrant V., additional, Saikku, Pekka A.I., additional, Sajadi, Mohammad M., additional, Salvaggio, Michelle R., additional, Santos, Carlos A.Q., additional, Satlin, Michael J., additional, Schaeffer, Anthony J., additional, Schimmer, Christoph, additional, Schooley, Robert T., additional, Schumacher, Richard F., additional, Sha, Beverly E., additional, Shapiro, Daniel S., additional, Sheehan, Gerard, additional, Shlaes, David M., additional, Shoham, Shmuel, additional, Simmons, Cameron P., additional, Simon, Dennis W., additional, Simon, Matthew S., additional, Simonsen, Kari A., additional, Slack, Mary P.E., additional, Smith, Tyrel T., additional, Sobel, Jack D., additional, Souli, Maria, additional, Sridhar, Shruti, additional, Steckelberg, James M., additional, Stevens, Dennis L., additional, Strah, Heather, additional, Sturm, A. Willem, additional, Sungkanuparph, Somnuek, additional, Tabrizi, Sarah J., additional, Tacconelli, Evelina, additional, Tan, Chen Sabrina, additional, Taplitz, Randy A., additional, Thomas, Guillemette, additional, Thomas, Lora D., additional, Thuny, Franck, additional, Thwaites, Guy, additional, Tissot, Frederic, additional, Tønjum, Tone, additional, Torriani, Francesca J., additional, Toso, Christian, additional, Tulkens, Paul M., additional, Tunkel, Allan R., additional, Turner, Claire E., additional, Ustianowski, Andrew P., additional, van Bambeke, Françoise, additional, van Crevel, Reinout, additional, van de Beek, Diederik, additional, van Delden, Christian, additional, van der Eerden, Menno M., additional, van der Meer, Jos W.M., additional, van der Poll, Tom, additional, van Ingen, Jakko, additional, van Putten, Jos, additional, Vaudaux, Bernard P., additional, Vermund, Sten H., additional, Viscidi, Raphael P., additional, Visvanathan, Kumar, additional, Visvesvara, Govinda S., additional, von Seidlein, Lorenz, additional, Wagenlehner, Florian M.E., additional, Wald, Anna, additional, Walsh, Thomas J., additional, Warhurst, David C., additional, Warnock, David W., additional, Warrell, David A., additional, Warrell, Mary J., additional, Warris, Adilia, additional, Watkins, Richard R., additional, Weatherall, David J., additional, Weber, Rainer, additional, Weidner, Wolfgang, additional, White, Jonathan R., additional, White, Peter J., additional, Whitehorn, James, additional, Whitley, Richard J., additional, Whitty, Christopher J.M., additional, Wiersinga, Willem Joost, additional, Wilcox, Mark H., additional, Williams, Thomas N., additional, Wilson, Cara C., additional, Wilson, Mary Elizabeth, additional, Wisplinghoff, Hilmar, additional, Wood, Robin, additional, Wunderink, Richard G., additional, Wyles, David, additional, Yang, Zhi-Tao, additional, Yoder, Jonathan S., additional, Zaidi, Najam A., additional, Zimmer, Andrea J., additional, Zuckerman, Jane N., additional, and Zumla, Alimuddin, additional

Published
2017
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44. Physician Global Assessment International Standardisation COnsensus in Systemic Lupus Erythematosus: the PISCOS study

Author

Piga, Matteo, primary, Chessa, Elisabetta, additional, Morand, Eric F, additional, Ugarte-Gil, Manuel F, additional, Tektonidou, Maria, additional, van Vollenhoven, Ronald, additional, Petri, Michelle, additional, Arnaud, Laurent, additional, Appenzeller, Simone, additional, Aranow, Cynthia, additional, Askanase, Anca, additional, Avcin, Tadej, additional, Bae, Sang-Cheol, additional, Bertsias, George, additional, Bonfa, Eloisa, additional, Cairoli, Ernesto, additional, Cardiel, Mario H, additional, Cervera, Ricard, additional, Chasset, François, additional, Chizzolini, Carlo, additional, Clarke, Ann E, additional, Conti, Fabrizio, additional, Costedoat-Chalumeau, Nathalie, additional, Czirják, László, additional, Doria, Andrea, additional, Dörner, Thomas, additional, Espinosa, Gerard, additional, Fischer-Betz, Rebecca, additional, Garcìa, Mercedes, additional, Gladman, Dafna D, additional, González, Luis A, additional, Gunnarsson, Iva, additional, Hamijoyo, Laniyati, additional, Hanly, John G, additional, Hasni, Sarfaraz A, additional, Houssiau, Frédéric A, additional, Inanç, Murat, additional, Inês, Luís S, additional, Isenberg, David, additional, Jacobsen, Soren, additional, Jan Wu, Yeong-Jian, additional, Kaneko, Yuko, additional, Katsumata, Yasuhiro, additional, Lau, Chak S, additional, Legge, Alexandra C, additional, Lerang, Karoline, additional, Limper, Maarten, additional, Louthrenoo, Worawit, additional, Luo, Shue-Fen, additional, Marinho, António, additional, Massardo, Loreto, additional, Mathian, Alexis, additional, Mosca, Marta, additional, Nikpour, Mandana, additional, Pego-Reigosa, José M, additional, Peschken, Christine A, additional, Pons-Estel, Bernardo A, additional, Pons-Estel, Guillermo J, additional, Rahman, Anisur, additional, Rednic, Simona, additional, Ribi, Camillo, additional, Ruiz-Irastorza, Guillermo, additional, Sato, Emilia I, additional, Saxena, Amit, additional, Schneider, Matthias, additional, Sebastiani, Gian Domenico, additional, Strand, Vibeke, additional, Svenungsson, Elisabet, additional, Tanaka, Yoshiya, additional, Tazi Mezalek, Zoubida, additional, Tee, Michael L, additional, Tincani, Angela, additional, Touma, Zahi, additional, Troldborg, Anne, additional, Vasconcelos, Carlos, additional, Vinet, Évelyne, additional, Vital, Edward M, additional, Voskuyl, Alexandre E, additional, Voss, Anne, additional, Wallace, Daniel, additional, Ward, Michael, additional, and Zamora, Leonid D, additional

Published
2022
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45. Intradermal Testing With COVID-19 mRNA Vaccines Predicts Tolerance

Author

Stehlin, Florian, primary, Mahdi-Aljedani, Rima, additional, Canton, Loris, additional, Monzambani-Banderet, Véronique, additional, Miauton, Alix, additional, Girard, Cedric, additional, Kammermann, Kevin, additional, Meylan, Sylvain, additional, Ribi, Camillo, additional, Harr, Thomas, additional, Yerly, Daniel, additional, and Muller, Yannick D., additional

Published
2022
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46. Humoral Responses Against Variants of Concern by COVID-19 mRNA Vaccines in Immunocompromised Patients

Author

Obeid, Michel, primary, Suffiotti, Madeleine, additional, Pellaton, Celine, additional, Bouchaab, Hasna, additional, Cairoli, Anne, additional, Salvadé, Vanja, additional, Stevenel, Caroline, additional, Hottinger, Rosemary, additional, Pythoud, Catherine, additional, Coutechier, Lucie, additional, Molinari, Laura, additional, Trono, Didier, additional, Ribi, Camillo, additional, Gottardo, Raphael, additional, Fenwick, Craig, additional, Pascual, Manuel, additional, Duchosal, Michel A., additional, Peters, Solange, additional, and Pantaleo, Giuseppe, additional

Published
2022
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48. Prediction of improvement in skin fibrosis in diffuse cutaneous systemic sclerosis: a EUSTAR analysis

Author

Dobrota, Rucsandra, Maurer, Britta, Graf, Nicole, Jordan, Suzana, Mihai, Carina, Kowal-Bielecka, Otylia, Allanore, Yannick, Distler, Oliver, Cerinic, Marco Matucci, Guiducci, Serena, Walker, Ulrich, Lapadula, Giovanni, Iannone, Florenzo, Becvar, Radim, Sierakowsky, Stanislaw, Cutolo, Maurizio, Sulli, Alberto, Valentini, Gabriele, Cuomo, Giovanna, Vettori, Serena, Riemekasten, Gabriela, Siegert, Elise, Rednic, Simona, Nicoara, Ileana, Kahan, André, Vlachoyiannopoulos, P., Montecucco, C., Caporali, Roberto, Carreira, Patricia E., Novak, Srdan, Czirják, László, Varju, Cecilia, Chizzolini, Carlo, Kucharz, Eugene J., Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Wu, Chen, Marjanovic, Zora, Faivre, Helene, Hij, Darin, Dhamadi, Roza, Airò, Paolo, Hesselstrand, Roger, Wollheim, Frank, Wuttge, Dirk M, Andréasson, Kristofer, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Trotta, F., Lo Monaco, Andrea, Hunzelmann, Nicolas, Pellerito, Raffaele, Mauriziano, Ospedale, Bambara, Lisa Maria, Caramaschi, Paola, Black, Carol, Denton, Christopher, Damjanov, Nemanja, Henes, Jörg, Ortiz Santamaria, Vera, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthias, Burkhardt, Harald, Himsel, Andrea, Salvador, Maria J., Silva, José Antonio Pereira Da, Stamenkovic, Bojana, Stankovic, Aleksandra, Banja, Niska, Tikly, Mohammed, Ananieva, Lidia P., Denisov, Lev N., Müller-Ladner, Ulf, Frerix, Marc, Tarner, Ingo, Scorza, Raffaella, Clinica, U.O. Immunologia, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Mendoza, Antonio Zea, de la Puente, Carlos, Giraldo, Walter A. Sifuentes, Midtvedt, Øyvind, Reiseter, Silje, Hachulla, Eric, Launay, David, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Soare, Alina, Gorga, Marilena, Bojinca, Mihai, Schett, Georg, Distler, Jörg HW, Beyer, Christian, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco P., Corrado, Ada, Pozzi, Maria R., Eyerich, Kilian, Hein, Rüdiger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Krummel-Lorenz, Brigitte, Aringer, Martin, Günther, Claudia, Westhovens, Rene, de Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Radominski, Sebastião C., de Souza Müller, Carolina, Azevedo, Valderílio F., Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Popa, Sergei, Zenone, Thierry, Highton, John, Stamp, Lisa, Chapman, Peter, O’Donnell, John, Solanki, Kamal, Veale, Douglas, OʼRourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Amoroso, Antonio, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Chirieac, Rodica, Ancuta, Codrina, Villiger, Peter, Adler, Sabine, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Hélène, Litinsky, Ira, 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, Spertini, François, Ribi, Camillo, Buss, Guillaume, Pasquali, Jean Louis, Martin, Thierry, and Gorse, Audrey

Published
2016
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49. Phenotype of limited cutaneous systemic sclerosis patients with positive anti-topoisomerase I antibodies: data from the EUSTAR cohort

Author

Zanatta, Elisabetta, Huscher, Doerte, Ortolan, Augusta, Avouac, Jerome, Airo, Paolo, Balbir-Gurman, Alexandra, Siegert, Elise, Cerinic, Marco Matucci, Cozzi, Franco, Riemekasten, Gabriela, Hoffmann-Vold, Anna-Maria, Distler, Oliver, Gabrielli, Armando, Heitmann, Stefan, Hunzelmann, Nicolas, Montecucco, Carlomaurizio, Morovic-Vergles, Jadranka, Ribi, Camillo, Doria, Andrea, Allanore, Yannick, Zanatta, Elisabetta, Huscher, Doerte, Ortolan, Augusta, Avouac, Jerome, Airo, Paolo, Balbir-Gurman, Alexandra, Siegert, Elise, Cerinic, Marco Matucci, Cozzi, Franco, Riemekasten, Gabriela, Hoffmann-Vold, Anna-Maria, Distler, Oliver, Gabrielli, Armando, Heitmann, Stefan, Hunzelmann, Nicolas, Montecucco, Carlomaurizio, Morovic-Vergles, Jadranka, Ribi, Camillo, Doria, Andrea, and Allanore, Yannick

Abstract

Objectives To characterize patients with positive anti-topoisomerase I (ATA) in lcSSc. Methods SSc patients enrolled in the EUSTAR cohort with a disease duration of <= 3 years at database entry were considered. We assessed the risk of major organ involvement in the following groups: ATA-lcSSc vs ACA-lcSSc and vs ANA without specificity (ANA)-lcSSc, and ATA-lcSSc vs ATA-dcSSc. Cox regression models with time-dependent covariates were performed with the following outcomes: new-onset interstitial lung disease (ILD), ILD progression [forced vital capacity (FVC) decline >= 10% and >= 5% vs values at ILD diagnosis), primary myocardial involvement (PMI), pulmonary hypertension (PH), any organ involvement and all-cause mortality. Results We included 1252 patients [194 ATA-lcSSc (15.5%)], with 7.7 years (s.d. 3.5) of follow-up. ILD risk was higher in ATA-lcSSc vs ACA- and ANA-lcSSc and similar to ATA-dcSSc, although with less frequent restrictive lung disease. The risk of FVC decline >= 10% (35% of ATA-lcSSc) was lower in ATA-lcSSc than in ATA-dcSSc, whereas FVC decline >= 5% occurs similarly between ATA-lcSSc (58% of patients) and other SSc subsets, including ATA-dcSSc. The risk of PMI was similar in ATA-lcSSc and ANA-lcSSc but lower than in ACA-lcSSc; no difference in PH and mortality risk was observed among lcSSc subsets. The risk of any organ involvement, PMI and PH was lower and the mortality tended to be lower in ATA-lcSSc vs ATA-dcSSc. Conclusion ATA-lcSSc patients have a high risk of ILD, albeit with a lower risk of progression compared with ATA-dcSSc, supporting careful screening for ILD in this subgroup.

Published
2022

50. Mepolizumab for Eosinophilic Granulomatosis with Polyangiitis (EGPA): a European multicenter observational study

Author

Bettiol, A, Urban, M, Dagna, L, Cottin, V, Franceschini, F, Del Giacco, S, Schiavon, F, Neumann, T, Lopalco, G, Novikov, P, Baldini, C, Lombardi, C, Berti, A, Alberici, F, Folci, M, Negrini, S, Sinico, R, Quartuccio, L, Lunardi, C, Parronchi, P, Moosig, F, Espígol-Frigolé, G, Schroeder, J, Kernder, A, Monti, S, Silvagni, E, Crimi, C, Cinetto, F, Fraticelli, P, Roccatello, D, Vacca, A, Mohammad, A, Hellmich, B, Samson, M, Bargagli, E, Cohen Tervaert, J, Ribi, C, Fiori, D, Bello, F, Fagni, F, Moroni, L, Ramirez, G, Nasser, M, Marvisi, C, Toniati, P, Firinu, D, Padoan, R, Egan, A, Seeliger, B, Iannone, F, Salvarani, C, Jayne, D, Prisco, D, Vaglio, A, Emmi, G, Bettiol, Alessandra, Urban, Maria Letizia, Dagna, Lorenzo, Cottin, Vincent, Franceschini, Franco, Del Giacco, Stefano, Schiavon, Franco, Neumann, Thomas, Lopalco, Giuseppe, Novikov, Pavel, Baldini, Chiara, Lombardi, Carlo, Berti, Alvise, Alberici, Federico, Folci, Marco, Negrini, Simone, Sinico, Renato Alberto, Quartuccio, Luca, Lunardi, Claudio, Parronchi, Paola, Moosig, Frank, Espígol-Frigolé, Georgina, Schroeder, Jan, Kernder, Anna Luise, Monti, Sara, Silvagni, Ettore, Crimi, Claudia, Cinetto, Francesco, Fraticelli, Paolo, Roccatello, Dario, Vacca, Angelo, Mohammad, Aladdin J, Hellmich, Bernhard, Samson, Maxime, Bargagli, Elena, Cohen Tervaert, Jan Willem, Ribi, Camillo, Fiori, Davide, Bello, Federica, Fagni, Filippo, Moroni, Luca, Ramirez, Giuseppe Alvise, Nasser, Mouhamad, Marvisi, Chiara, Toniati, Paola, Firinu, Davide, Padoan, Roberto, Egan, Allyson, Seeliger, Benjamin, Iannone, Florenzo, Salvarani, Carlo, Jayne, David, Prisco, Domenico, Vaglio, Augusto, Emmi, Giacomo, Bettiol, A, Urban, M, Dagna, L, Cottin, V, Franceschini, F, Del Giacco, S, Schiavon, F, Neumann, T, Lopalco, G, Novikov, P, Baldini, C, Lombardi, C, Berti, A, Alberici, F, Folci, M, Negrini, S, Sinico, R, Quartuccio, L, Lunardi, C, Parronchi, P, Moosig, F, Espígol-Frigolé, G, Schroeder, J, Kernder, A, Monti, S, Silvagni, E, Crimi, C, Cinetto, F, Fraticelli, P, Roccatello, D, Vacca, A, Mohammad, A, Hellmich, B, Samson, M, Bargagli, E, Cohen Tervaert, J, Ribi, C, Fiori, D, Bello, F, Fagni, F, Moroni, L, Ramirez, G, Nasser, M, Marvisi, C, Toniati, P, Firinu, D, Padoan, R, Egan, A, Seeliger, B, Iannone, F, Salvarani, C, Jayne, D, Prisco, D, Vaglio, A, Emmi, G, Bettiol, Alessandra, Urban, Maria Letizia, Dagna, Lorenzo, Cottin, Vincent, Franceschini, Franco, Del Giacco, Stefano, Schiavon, Franco, Neumann, Thomas, Lopalco, Giuseppe, Novikov, Pavel, Baldini, Chiara, Lombardi, Carlo, Berti, Alvise, Alberici, Federico, Folci, Marco, Negrini, Simone, Sinico, Renato Alberto, Quartuccio, Luca, Lunardi, Claudio, Parronchi, Paola, Moosig, Frank, Espígol-Frigolé, Georgina, Schroeder, Jan, Kernder, Anna Luise, Monti, Sara, Silvagni, Ettore, Crimi, Claudia, Cinetto, Francesco, Fraticelli, Paolo, Roccatello, Dario, Vacca, Angelo, Mohammad, Aladdin J, Hellmich, Bernhard, Samson, Maxime, Bargagli, Elena, Cohen Tervaert, Jan Willem, Ribi, Camillo, Fiori, Davide, Bello, Federica, Fagni, Filippo, Moroni, Luca, Ramirez, Giuseppe Alvise, Nasser, Mouhamad, Marvisi, Chiara, Toniati, Paola, Firinu, Davide, Padoan, Roberto, Egan, Allyson, Seeliger, Benjamin, Iannone, Florenzo, Salvarani, Carlo, Jayne, David, Prisco, Domenico, Vaglio, Augusto, and Emmi, Giacomo

Abstract

Objective: Mepolizumab proved efficacious for eosinophilic granulomatosis with polyangiitis (EGPA, former Churg-Strauss) at the dosage of 300mg/4 weeks in the randomized controlled MIRRA trial. Few successful real-life experiences with the dosage approved for severe eosinophilic asthma (100mg/4 weeks) were recently reported. We retrospectively assessed the effectiveness and safety of mepolizumab 100 and 300mg/4 weeks in a large European EGPA cohort. Methods: We included all EGPA patients treated with mepolizumab at the recruiting centres in 2015-2020. Treatment response was evaluated from month 3 through 24 (T3-T24) after mepolizumab starting. Complete response (CR) was defined as no disease activity (Birmingham Vasculitis Activity Score, BVAS=0) and a prednisone dose ≤4mg/day. Respiratory outcomes included asthma and ear-nose-throat (ENT) exacerbations. Results: We included 203 patients, of whom 191 at stable dosage (158 mepolizumab 100mg/4 weeks, 33 300mg/4 weeks). At T3, 25 patients (12.3%) had a CR. CR rates increased to 30.4% and 35.7% at T12 and T24 and were comparable between mepolizumab 100 and 300mg/4 weeks. Mepolizumab led to a significant reduction in BVAS, prednisone dose, eosinophil counts from T3 through T24, with no significant differences between 100 and 300 mg/4weeks. Eighty-two patients (40.4%) experienced asthma exacerbations [57/158 (36%) on 100mg/4 weeks; 17/33 (52%) on 300mg/4 weeks]. Thirty-one (15.3%) experienced ENT exacerbations. Forty-four patients (21.7%) experienced adverse events, most being non-serious (38/44). Conclusion: Mepolizumab both at 100 and 300mg/4 weeks is effective for EGPA. The two dosages should be compared in the setting of a controlled trial.

Published
2022

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