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

Author

Hoffmann-Vold A. -M., Brunborg C., Airo P., Ananyeva L. P., Czirjak L., Guiducci S., Hachulla E., Li M., Mihai C., Riemekasten G., Sfikakis P. P., Valentini G., Kowal-Bielecka O., Allanore Y., Distler O., Vacca A., Giollo A., Balbir-Gurman A., Gheorghiu A. M., Marcoccia A., Herrick A., Radic M., Stamenkovic B., Anic B., Granel B., Ribi C., Selmi C. F., Carlos de la Puente M., de Souza Muller C., Denton C., Kayser C., Tanaseanu C. -M., Majewski D., Rimar D., Krasowska D., Veale D., Walker U., Kerzberg E., Rezus E., Zanatta E., Siegert E., De Langhe E., Oksel F., Ingegnoli F., Cantatore F. P., Szucs G., Cuomo G., Seskute G., Litinsky V., Castellvi I., Morovic-Vergles J., Sibilia J., Henes J., Solanki K., Perdan-Pirkmajer K., Herrmann K., Saketkoo L. A., Stamp L., Mouthon L., Salvador M. J., Pozzi M. R., Uprus M., Vanthuyne M., Engelhart M., Kohm M., Iudici M., Inanc M., Fathi N., Pamuk N., Garcia de la Pena Lefebv P., Carreira P. E., Bancel D. F., Moroncini L., Montecucco C., Ancuta C., Sunderkotter C., Muller-Ladner U., Rosato E., Kucharz E. J., Iannone F., Del Galdo F., Poormoghim H., Kotter I., Distler J., Cutolo M., Tikly M., Damjanov N., Hunzelmann N., Vlachoyiannopoulos P., Hasler P., Sarzi Puttini P., Wiland P., Becvar R., Yavuz S., Zdrojewski Z., Pellerito R., Foti R., Ionescu R. M., Adler S., Kahl S., Moiseev S., Stebbings S., Rednic S., Negrini S., Heitmann S., Ullman S., Agachi S., Martin T., Schmeiser T., Riccieri V., Smith V., Bernardino V., Ortiz-Santamaria V., Hsu V. M., Abdel Atty Mohamed W. A., Hoffmann-Vold, A. -M., Brunborg, C., Airo, P., Ananyeva, L. P., Czirjak, L., Guiducci, S., Hachulla, E., Li, M., Mihai, C., Riemekasten, G., Sfikakis, P. P., Valentini, G., Kowal-Bielecka, O., Allanore, Y., Distler, O., Vacca, A., Giollo, A., Balbir-Gurman, A., Gheorghiu, A. M., Marcoccia, A., Herrick, A., Radic, M., Stamenkovic, B., Anic, B., Granel, B., Ribi, C., Selmi, C. F., Carlos de la Puente, M., de Souza Muller, C., Denton, C., Kayser, C., Tanaseanu, C. -M., Majewski, D., Rimar, D., Krasowska, D., Veale, D., Walker, U., Kerzberg, E., Rezus, E., Zanatta, E., Siegert, E., De Langhe, E., Oksel, F., Ingegnoli, F., Cantatore, F. P., Szucs, G., Cuomo, G., Seskute, G., Litinsky, V., Castellvi, I., Morovic-Vergles, J., Sibilia, J., Henes, J., Solanki, K., Perdan-Pirkmajer, K., Herrmann, K., Saketkoo, L. A., Stamp, L., Mouthon, L., Salvador, M. J., Pozzi, M. R., Uprus, M., Vanthuyne, M., Engelhart, M., Kohm, M., Iudici, M., Inanc, M., Fathi, N., Pamuk, N., Garcia de la Pena Lefebv, P., Carreira, P. E., Bancel, D. F., Moroncini, L., Montecucco, C., Ancuta, C., Sunderkotter, C., Muller-Ladner, U., Rosato, E., Kucharz, E. J., Iannone, F., Del Galdo, F., Poormoghim, H., Kotter, I., Distler, J., Cutolo, M., Tikly, M., Damjanov, N., Hunzelmann, N., Vlachoyiannopoulos, P., Hasler, P., Sarzi Puttini, P., Wiland, P., Becvar, R., Yavuz, S., Zdrojewski, Z., Pellerito, R., Foti, R., Ionescu, R. M., Adler, S., Kahl, S., Moiseev, S., Stebbings, S., Rednic, S., Negrini, S., Heitmann, S., Ullman, S., Agachi, S., Martin, T., Schmeiser, T., Riccieri, V., Smith, V., Bernardino, V., Ortiz-Santamaria, V., Hsu, V. M., and Abdel Atty Mohamed, W. A.

Subjects
interstitial lung disease, Pulmonary and Respiratory Medicine, enrichment, systemic sclerosis, clinical trial, Cardiology and Cardiovascular Medicine, Critical Care and Intensive Care Medicine
Abstract

BACKGROUND: Enrichment strategies from clinical trials for progressive systemic sclerosis-associated interstitial lung disease (SSc-ILD) have not been tested in a real-life cohort.RESEARCH QUESTION: Do enrichment strategies for progressive ILD impact efficacy, repre-sentativeness, and feasibility in patients with SSc-ILD from the European Scleroderma Trials and Research (EUSTAR) database?STUDY DESIGN AND METHODS: We applied the inclusion criteria of major recent SSc-ILD trials (Study of the Efficacy and Safety of Tocilizumab in Participants With Systemic Sclerosis [focuSSced], Scleroderma Lung Study II [SLS II], and Safety and Efficacy of Nintedanib in Systemic Sclerosis [SENSCIS]) and assessed progressive ILD, which was defined as absolute change in FVC and as significant progression (FVC decline $10%). Data were compared with all patients and with patients who did not fulfill any inclusion criteria. RESULTS: In total, 2,258 patients with SSc-ILD were included: 31.2% of the patients met SENSCIS criteria; 5.8% of the patients met SLS II criteria; 1.6% of the patients met focuSSced criteria, and 67.7% (1,529) of the patients did not meet any criteria. In the first 12 + 3 months, the absolute FVC decline in all patients and in patients who fulfilled criteria from SENSCIS was -0.1%, in patients who fulfilled criteria from focuSSced was -3.7%, and in patients who fulfilled criteria from SLS II was 2.3%, with accompanying more progressors in focuSSced. The patient populations that fulfilled the different study inclusion criteria significantly differed in various clinical parameters. In the second 12-month period, SENSCIS-enriched patients had a further absolute FVC% decline as described for the total cohort. In contrast, patients who fulfilled the focuSSced and SLS II criteria showed numeric improvement of lung function. There were no significant associations of enrichment criteria and ILD progression.INTERPRETATION: The application of enrichment criteria from previous clinical trials showed enrichment for progression with variable success, which led to selected patient populations reducing feasibility of recruitment. These findings are important for future clinical trial design and interpretation of the results of published trials.CHEST 2023; 163(3):586-598

Published
2023
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7. Granulomatose avec polyangéite : quoi de neuf ? [Granulomatosis with polyangiitis: what's new?]

Author

Ringwald, M., Chevalley, D., Bongard, C., Kissling, S., Rotman, S., Von Garnier, C., Ribi, C., and Comte, D.

Subjects
Humans, Granulomatosis with Polyangiitis/diagnosis, Granulomatosis with Polyangiitis/therapy, Granulomatosis with Polyangiitis/complications, Antibodies, Antineutrophil Cytoplasmic/therapeutic use, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/therapy, Immunosuppressive Agents/therapeutic use, Adrenal Cortex Hormones/therapeutic use
Abstract

Within the group of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides, granulomatosis with polyangiitis (GPA) is the most frequent. The incidence is around 10 to 20 cases/million/year. Clinical manifestations are varied, with ENT, lungs and kidneys most frequently involved. ANCA are pathogenic by triggering neutrophil activation, which leads to vascular damage. Detection of ANCA is most helpful in establishing the diagnosis, but serology may be negative in GPA limited to the airways. Diagnostic work-up and therapy require a multidisciplinary approach. Treatment includes an induction and maintenance phase, combining corticosteroids and immunosuppressive drugs. It aims at limiting the risk of relapses, which is important in GPA, and at reducing corticosteroids toxicity.

Published
2023

8. Maladie des anticorps anti-membrane basale glomérulaire [Anti-glomerular basement membrane disease]

Author

Stevanin, M., Kissling, S., Daccord, C., Rotman, S., Comte, D., and Ribi, C.

Subjects
Humans, Anti-Glomerular Basement Membrane Disease/diagnosis, Anti-Glomerular Basement Membrane Disease/therapy, Autoantibodies, Hemorrhage/etiology, Immunosuppressive Agents/therapeutic use
Abstract

Anti-glomerular basement membrane disease is a rare disease. In its classical presentation it associates rapidly progressive glomerulonephritis and diffuse alveolar hemorrhage, linked to the presence of antibodies targeting type IV collagen in the glomerular and alveolar basal membrane. Anti-GBM disease warrants prompt medical management to limit permanent kidney damage and mortality. Treatment includes plasma exchanges to quickly remove pathogenic antibodies and immunosuppressants to stop their production. This article reviews the pathogenesis and current treatments.

Published
2023

9. Inhibiteurs des Janus kinases : nouvelles perspectives pour la médecine de précision ? [Janus kinase inhibitors : new perspectives for precision medicine ?]

Author

Tachet, J., Dumusc, A., Conrad, C., Grandoni, F., Chalandon, Y., Ribi, C., Buclin, T., and Girardin, F.

Subjects
Humans, Janus Kinase Inhibitors/therapeutic use, Precision Medicine, Arthritis, Rheumatoid/drug therapy, COVID-19/drug therapy
Abstract

Janus kinase inhibitors (JAKi), such as tofacitinib, baricitinib, upadacitinib or ruxolitinib, are small molecules active on specific intracellular targets and used orally for the treatment of autoimmune or myeloproliferative diseases. Their remarkable therapeutic efficacy is offset by a significant risk of toxicities, essentially dose-dependent and a variable pharmacokinetic profile. The JAKi represent a new therapeutic armamentarium for treating autoimmune, myeloproliferative and inflammatory diseases (incl. COVID-19), but require thorough treatment individualization and close monitoring. Therapeutic Drug Monitoring (TDM) of JAKi could allow a personalized prescription and improve the efficacy-toxicity profile.

Published
2022

10. Mepolizumab for Eosinophilic Granulomatosis With Polyangiitis: A European Multicenter Observational Study

Author

Bettiol, A., Urban, M. L., 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. A., Quartuccio, L., Lunardi, C., Parronchi, P., Moosig, F., Espigol-Frigole, G., Schroeder, J., Kernder, A. L., Monti, S., Silvagni, E., Crimi, C., Cinetto, F., Fraticelli, P., Roccatello, D., Vacca, A., Mohammad, A. J., Hellmich, B., Samson, M., Bargagli, E., Cohen Tervaert, J. W., Ribi, C., Fiori, D., Bello, F., Fagni, F., Moroni, L., Ramirez, G. A., 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., Ahmad, K., Beccalli, M., Bonnotte, B., Bortolotti, R., Cariddi, A., Caminati, M., Cid, M. C., Deidda, M., Delvino, P., Scala, G. D., Felicetti, M., Ferro, F., Furini, F., Gelain, E., Ghirelli, G., Holle, J., Losappio, L. M., Mahr, A., Malandrino, D., Marhhold, J., Mattioli, I., Moi, L., Moiseev, S., Muratore, F., Nolasco, S., Olivieri, B., Palermo, A., Regola, F., Sander, O., Scarpa, R., Sciascia, S., Silvestri, E., Susca, N., Terrier, B., Treppo, E., Trezzi, B., Uzzo, M., Vitiello, G., Yacyshyn, E., RS: MHeNs - R3 - Neuroscience, Faculteit FHML Centraal, 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, Jayne, David [0000-0002-1712-0637], and Apollo - University of Cambridge Repository

Subjects
Male, Epidemiology, Birmingham Vasculitis Activity Score, law.invention, Glucocorticoid, Randomized controlled trial, Prednisone, law, Eosinophilic, Monoclonal, Immunology and Allergy, Humanized, PLACEBO, Middle Aged, egpa mepolizumab, Treatment Outcome, SAFETY, Female, ANCA-associated Vasculitis, Biologicals, Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss), Glucocorticoids, Granulomatosis with polyangiitis, ANCA-associated Vasculiti, medicine.drug, Keywords: ANCA-associated Vasculitis, Adult, medicine.medical_specialty, Immunology, Antibodies, Monoclonal, Humanized, Antibodies, Drug Administration Schedule, Eosinophilia, Granulomatosis with Polyangiitis, Humans, Retrospective Studies, Rheumatology, Internal medicine, medicine, Adverse effect, Asthma, business.industry, medicine.disease, Biological, EGPA, European EGPA Study Group, business, FOLLOW-UP, Mepolizumab
Abstract

OBJECTIVE: Mepolizumab proved to be an efficacious treatment for eosinophilic granulomatosis with polyangiitis (EGPA) at a dose of 300 mg every 4 weeks in the randomized, controlled MIRRA trial. In a few recently reported studies, successful real-life experiences with the approved dose for treating severe eosinophilic asthma (100 mg every 4 weeks) were observed. We undertook this study to assess the effectiveness and safety of mepolizumab 100 mg every 4 weeks and 300 mg every 4 weeks in a large European EGPA cohort. METHODS: We included all patients with EGPA treated with mepolizumab at the recruiting centers in 2015-2020. Treatment response was evaluated from 3 months to 24 months after initiation of mepolizumab. Complete response to treatment was defined as no disease activity (Birmingham Vasculitis Activity Score [BVAS] = 0) and a prednisolone or prednisone dose (or equivalent) of ���4 mg/day. Respiratory outcomes included asthma and ear, nose, and throat (ENT) exacerbations. RESULTS: Two hundred three patients, of whom 191 received a stable dose of mepolizumab (158 received 100 mg every 4 weeks and 33 received 300 mg every 4 weeks) were included. Twenty-five patients (12.3%) had a complete response to treatment at 3 months. Complete response rates increased to 30.4% and 35.7% at 12 months and 24 months, respectively, and rates were comparable between mepolizumab 100 mg every 4 weeks and 300 mg every 4 weeks. Mepolizumab led to a significant reduction in BVAS score, prednisone dose, and eosinophil counts from 3 months to 24 months, with no significant differences observed between 100 mg every 4 weeks and 300 mg every 4 weeks. Eighty-two patients (40.4%) experienced asthma exacerbations (57 of 158 [36%] who received 100 mg every 4 weeks; 17 of 33 [52%] who received 300 mg every 4 weeks), and 31 patients (15.3%) experienced ENT exacerbations. Forty-four patients (21.7%) experienced adverse events (AEs), most of which were nonserious AEs (38 of 44). CONCLUSION: Mepolizumab at both 100 mg every 4 weeks and 300 mg every 4 weeks is effective for the treatment of EGPA. The 2 doses should be compared in the setting of a controlled trial.

Published
2022
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11. Measurement of circulating CD21-CD27- B lymphocytes in SLE patients is associated with disease activity independently of conventional serological biomarkers

Author

Horisberger, A., Humbel, M., Fluder, N., Bellanger, F., Fenwick, C., Ribi, C., and Comte, D.

Subjects
Autoimmune Diseases, B-Lymphocytes, Biomarkers, Complement System Proteins, Humans, Lupus Erythematosus, Systemic, Lymphocyte Count
Abstract

Determining disease activity in systemic lupus erythematosus (SLE) patients is challenging and limited by the lack of reliable biomarkers. Abnormally activated B cells play a key role in the pathogenesis of SLE, but their measure in clinical practice is currently not recommended. Here, we studied peripheral B cells to identify a valid biomarker. We analyzed peripheral B cells in a discovery cohort of 30 SLE patients compared to 30 healthy controls (HC) using mass cytometry and unsupervised clustering analysis. The relevant B cell populations were subsequently studied by flow cytometry in a validation cohort of 63 SLE patients, 28 autoimmune diseases controls and 39 HC. Our data show an increased frequency of B cell populations with activated phenotype in SLE compared to healthy and autoimmune diseases controls. These cells uniformly lacked the expression of CD21 and CD27. Measurement of CD21 - CD27 - B cells in the blood identified patients with active disease and their frequency correlated with disease severity. Interestingly, we did not observe an increase in the frequency of CD21 - CD27 - B cells in patients with clinically inactive disease but with elevated conventional biomarkers (anti-dsDNA and complement levels). Accordingly, measurement of CD21 - CD27 - B cells represents a robust and easily accessible biomarker to assess the activity of the disease in SLE patients.

Published
2022

12. Maladies causées par Anisakis simplex chez l’humain [Human diseases caused by Anisakis simplex]

Author

Ringwald, M., Muller, Y.D., and Ribi, C.

Subjects
Animals, Anisakiasis/diagnosis, Anisakiasis/epidemiology, Anisakiasis/parasitology, Anisakis, Fishes/parasitology, Humans, Hypersensitivity, Immunoglobulin E, Larva, Mammals, Seafood/adverse effects, Seafood/parasitology
Abstract

Anisakis simplex is a parasitic worm. It infects marine mammals that feed on fish and cephalopods, its intermediary hosts. Human disease is caused by accidental ingestion of Anisakis larvae. Upon consumption of contaminated fish, cuttlefish or squid, human may develop two distinct clinical pictures: Anisakiasis is provoked by living larvae penetrating the digestive mucosa. Allergy is caused by IgE-mediate hypersensitivity to living or dead larvae in a previously sensitized individual. Anisakiasis may manifests with violent epi gastric pain, acute abdomen or eosinophilic gastroenteritis. The larvae may be visualized by endoscopy or histology. The main Anisakis allergens are not denaturated by heat or cold and resist to digestion. Allergy diagnosis relies on careful history and detection of specific IgE.

Published
2022

13. Réactions d’hypersensibilité au fer intraveineux : le point de vue de l’allergologue [Hypersensitivity reactions to intravenous iron: an allergist' perspective]

Author

Moi, L., Stehlin, F., Desseauve, D., Ribi, C., and Muller, Y.D.

Subjects
Administration, Intravenous, Allergists, Anaphylaxis/chemically induced, Anemia, Iron-Deficiency/drug therapy, Drug Hypersensitivity/diagnosis, Drug Hypersensitivity/etiology, Drug Hypersensitivity/therapy, Humans, Infusions, Intravenous, Iron/adverse effects
Abstract

Intravenous iron infusions rarely result in severe hypersensitivity reactions. The primary suspected hypersensitivity mechanism is an abnormal complement activation by non-IgE antibodies to the carbohydrate moieties stabilizing iron formulations. A major risk factor for hypersensitivity reactions is related to the infusion speed. Fishbane-like reactions usually resolve after pausing the infusion, which can be resumed under medical surveillance and at a lower infusion rate. Yet, anaphylactic reactions require emergency first aid and subsequent strict avoidance of intravenous iron. Desensitization protocols can be implemented in selected cases and under strict medical surveillance to reduce the risks of severe reactions upon re-exposure.

Published
2022

14. 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

15. POS0893 FACTORS TO CONSIDER FOR MEASURING THE EFFECT OF LUNG FUNCTION ON PATIENT REPORTED OUTCOMES IN SYSTEMIC SCLEROSIS PATIENTS: ANALYSIS OF THE EUSTAR DATABASE.

Author

Lazzaroni, M. G., primary, Wilson, M., additional, Hensor, E., additional, Distler, J. H. W., additional, Cuomo, G., additional, Siegert, E., additional, Müller-Ladner, U., additional, Allanore, Y., additional, Salvador, M. J., additional, Anic, B., additional, Walker, U., additional, Czirják, L., additional, Ribi, C., additional, Tanaseanu, C. M., additional, Gabrielli, A., additional, Hoffmann-Vold, A. M., additional, Distler, O., additional, and Del Galdo, F., additional

Published
2022
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16. POS0383 CLINICAL CHARACTERISTICS AND PROGNOSIS OF PATIENTS WITH SYSTEMIC SCLEROSIS SINE SCLERODERMA: DATA FROM THE INTERNATIONAL EUSTAR DATABASE.

Author

Lescoat, A., primary, Huang, S., additional, Carreira, P., additional, Siegert, E., additional, De Vries-Bouwstra, J., additional, Distler, J. H. W., additional, Smith, V., additional, Del Galdo, F., additional, Anic, B., additional, Damjanov, N., additional, Rednic, S., additional, Ribi, C., additional, Farge, D., additional, Hoffmann-Vold, A. M., additional, Gabrielli, A., additional, Distler, O., additional, Khanna, D., additional, and Allanore, Y., additional

Published
2022
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18. CLINICAL CHARACTERISTICS AND PROGNOSIS OF PATIENTS WITH SYSTEMIC SCLEROSIS SINE SCLERODERMA: DATA FROM THE INTERNATIONAL EUSTAR DATABASE

Author

Lescoat, A, Huang, S., Carreira, P, Siegert, E., de Vries-Bouwstra, J., Distler, Jörg, Smith, V., del Galdo, F., Anic, B., Damjanov, N., Rednic, S., Ribi, C., Farge, D., Hoffmann-Vold, A. M., Gabrielli, A., Distler, O., Khanna, D., Allanore, Y, 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 ), École des Hautes Études en Santé Publique [EHESP] (EHESP), University of Michigan [Ann Arbor], University of Michigan System, Hospital Universitario 12 de Octubre [Madrid], Charité - UniversitätsMedizin = Charité - University Hospital [Berlin], Universiteit Leiden, Friedrich-Alexander Universität Erlangen-Nürnberg (FAU), Ghent University Hospital, University of Leeds, University Hospital Centre Zagreb, Partenaires INRAE, University of Belgrade [Belgrade], Babes-Bolyai University [Cluj-Napoca] (UBB), Lausanne University Hospital, Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Oslo University Hospital [Oslo], Università Politecnica delle Marche [Ancona] (UNIVPM), University hospital of Zurich [Zurich], Hôpital Cochin [AP-HP], Universiteit Leiden [Leiden], and Chard-Hutchinson, Xavier

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

International audience

Published
2022
Full Text
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19. Approche diagnostique et thérapeutique d’un asthme difficile [Diagnostic and therapeutic approach to difficult asthma]

Author

Grandbastien, M., Kolb, M., Ribi, C., and Von Garnier, C.

Subjects
Asthma/diagnosis, Asthma/drug therapy, Chronic Disease, Humans
Abstract

Asthma is a chronic disease and asthma control can be affected by many factors. In case of difficult asthma, intensifying drug therapy is not the key. This type of asthma needs an overall management in order to diagnose and treat each factor known to be associated with poor asthma control. The aim of this article is to describe the structured and systematic approach for these patients.

Published
2021

20. Une allergie insolite : le syndrome cannabis-fruits et legumes [An unusual allergy : cannabis-fruit and vegetable syndrome]

Author

Ringwald, M., Moi, L., Muller, Y.D., and Ribi, C.

Subjects
Allergens, Antigens, Plant, Cannabis, Cross Reactions, Food Hypersensitivity/diagnosis, Fruit, Humans, Immunoglobulin E, Plant Proteins, Vegetables
Abstract

Cannabis-fruit and vegetable syndrome is of recent discovery and linked to lipid transfer protein (LTP) sensitization. It is thought that the primary sensitization originates from the cannabis LTP (Can s 3). Sensitized patients can cross-react to others LTP homologs such as peach LTP (Pru p 3). Diagnosis may be challenging, as consumption of cannabis is often omitted by the patient and needs to be specifically addressed during the interview. Thus, meticulous history taking is mandatory. Laboratory workup includes LTP-specific IgE and skin testing. Management relies on allergen eviction.

Published
2021

21. Syndrome de Sjögren : du diagnostic au traitement [Sjögren's syndrome: from diagnosis to treatment]

Author

Dumusc, A. and Ribi, C.

Subjects
Humans, Rheumatology, Sjogren's Syndrome/diagnosis, Sjogren's Syndrome/drug therapy
Abstract

Sjögren's syndrome (SS) is an auto-immune condition involving salivary and lacrymal glands leading to dry mouth and dry eyes symptoms. Some patients also present with systemic manifestations. Diagnosis of SS is made after clinical, serological, and histological assessment according to the American College of Rheumatology and European League Against Rheumatism (EULAR) classification criteria. Recent clinical trials showed a significant decrease of systemic activity of SS in patients treated with iscalimab (anti-CD40) and ianalumab (anti-BAFF-R). These results need to be confirmed in larger studies. However, two phase 3 randomized trials did not show efficacy treating SS with abatacept. We also describe in this article the first EULAR recommendations on SS management.

Published
2021

22. CLINICAL PHENOTYPE IN SCLERODERMA PATIENTS WITH ANTI-TOPOISOMERASE I POSITIVITY AND LIMITED CUTANEOUS FORM: DATA FROM THE EUSTAR DATABASE

Author

Zanatta, E., Huscher, D., Airo, P., Balbir-Gurman, A., Siegert, E., Ortolan, A., Matucci-Cerinic, M., Cozzi, F., Riemekasten, G., Hoffmann-Vold, A. M., Distler, O., Gabrielli, A., Heitmann, S., Hunzelmann, N., Montecucco, C., Morovic-Vergles, J., Ribi, C., Doria, A., Allanore, Y., Zanatta, E., Huscher, D., Airo, P., Balbir-Gurman, A., Siegert, E., Ortolan, A., Matucci-Cerinic, M., Cozzi, F., Riemekasten, G., Hoffmann-Vold, A. M., Distler, O., Gabrielli, A., Heitmann, S., Hunzelmann, N., Montecucco, C., Morovic-Vergles, J., Ribi, C., Doria, A., and Allanore, Y.

Published
2021

23. POS0318 CLINICAL PHENOTYPE IN SCLERODERMA PATIENTS WITH ANTI-TOPOISOMERASE I POSITIVITY AND LIMITED CUTANEOUS FORM: DATA FROM THE EUSTAR DATABASE

Author

Zanatta, E., primary, Huscher, D., additional, Airò, P., additional, Balbir-Gurman, A., additional, Siegert, E., additional, Ortolan, A., additional, Matucci-Cerinic, M., additional, Cozzi, F., additional, Riemekasten, G., additional, Hoffmann-Vold, A. M., additional, Distler, O., additional, Gabrielli, A., additional, Heitmann, S., additional, Hunzelmann, N., additional, Montecucco, C., additional, Morovic-Vergles, J., additional, Ribi, C., additional, Doria, A., additional, and Allanore, Y., additional

Published
2021
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25. Cardiotoxicité des inhibiteurs de points de contrôle immunitaire utilisés dans le traitement des cancers [Cardiotoxicity of immune checkpoint inhibitors used in cancer treatment]

Author

Arangalage, D., Pavon, A.G., Hugelshofer, S., Desgraz, B., Tzimas, G., Delyon, J., Muller, O., Obeid, M., Ribi, C., Michielin, O., Özdemir, B.C., and Monney, P.

Subjects
Cardiotoxicity/etiology, Humans, Immunotherapy/adverse effects, Myocarditis/chemically induced, Neoplasms/drug therapy, Neoplasms/immunology, Neoplasms/pathology
Abstract

Immune checkpoint inhibitors (ICI) have revolutionized the field of oncology, by reshaping the prognosis of many cancers and are progressively becoming the standard of care. One of the costs of these advances is the emergence of a new spectrum of immune-related adverse events (irAEs), of which cardiovascular irAEs are particularly feared. ICI-induced myocarditis is often a diagnostic challenge because of the vast heterogeneity of clinical presentations, and it is associated with a high mortality rate of around 50%. The present article summarizes the cardiac manifestations, the diagnostic strategy and the therapeutic management of patients with ICI-induced myocarditis used in the treatment of cancer.

Published
2020

26. Maladies autoimmunes dans le contexte de la pandémie COVID-19 [Autoimmune diseases in the context of pandemic COVID-19]

Author

Horisberger, A., Moi, L., Ribi, C., and Comte, D.

Subjects
Autoimmune Diseases/complications, Autoimmune Diseases/therapy, Betacoronavirus, Coronavirus Infections/complications, Coronavirus Infections/epidemiology, Humans, Pandemics, Pneumonia, Viral/complications, Pneumonia, Viral/epidemiology
Abstract

Patient suffering from autoimmune diseases (AID) typically have an increased risk of infection, which is attributed to the disease itself, but also to immunosuppressive drugs (IS) and comorbidities. During the current COVID-19 outbreak, the way to manage these diseases remains elusive. Limited data is currently available on AID and IS in the context of this new coronavirus infection. To date, there is no evidence to support an increase in complications of COVID-19 in these patients. In addition, certain drugs that are commonly used to treat AID could be part of the therapeutic arsenal used in COVID-19. The purpose of this article is to review the unique aspects of patients with AID during the COVID-19 outbreak.

Published
2020

27. Prise en charge du syndrome des ­anticorps antiphospholipides chez l’adulte [Management of the antiphospholipid syndrome in adults]

Author

Doms, J., Horisberger, A., and Ribi, C.

Subjects
Adult, Antibodies, Antiphospholipid/blood, Anticoagulants/therapeutic use, Antiphospholipid Syndrome/blood, Antiphospholipid Syndrome/complications, Antiphospholipid Syndrome/drug therapy, Female, Humans, Hydroxychloroquine/therapeutic use, Knowledge, Pregnancy, Pregnancy Complications/blood, Pregnancy Complications/drug therapy, Risk Factors, Thrombosis/blood, Thrombosis/drug therapy, Thrombosis/etiology
Abstract

The antiphospholipid syndrome (APS) is a complex autoimmune -disease characterized by the expression of antiphospholipid -antibodies (APL) and a variety of clinical presentation. The latest classification defines APS by the occurrence of vascular thrombosis and/or typical obstetrical morbidity together with persistently -detectable APL at least 12 weeks apart. The latest recommendation proposes a risk profile based on the type and titer of APL detected, in order to guide the intensity of prophylactic measures. Based on current knowledge, novel oral anticoagulants should not be used in APS, particularly in patients with a high-risk APL profile or arterial thrombosis. Beyond the mere aspect of anticoagulant treatment, immunomodulatory approaches to the APS such as hydroxychloroquine are under investigation.

Published
2020

28. Angiœdèmes héréditaires sur ­déficit en C1-inhibiteur : nouveautés ­thérapeutiques [Hereditary angioedema due to C1-esterase inhibitor deficiency : novel approaches]

Author

Stehlin, F. and Ribi, C.

Subjects
Angioedema, Angioedemas, Hereditary/drug therapy, Angioedemas, Hereditary/enzymology, Antibodies, Monoclonal, Bradykinin/analogs & derivatives, Bradykinin/therapeutic use, Complement C1 Inhibitor Protein/therapeutic use, Complement Inactivating Agents/therapeutic use, Esterases/antagonists & inhibitors, Humans
Abstract

Hereditary angioedema type 1 and 2 are due to a deficiency in C1--esterase inhibitor. This molecule inhibits the generation of bradykinin, a potent inflammatory mediator that increases vascular permeability. Upon accumulation of bradykinin, patients affected develop painful subcutaneous or submucosal edemas that last for several days. In case the upper airways are affected, there is risk of suffocation. This type of angioedema does not respond to antihistamines, cortico-steroids or epinephrine. Management of angioedema attacks consists in injecting C1-esterase inhibitor concentrate or icatibant, a bradykinin receptor B2 antagonist. Preventive measures aim at reducing the frequency and the severity of angioedema attacks. Inhibition of -plasma kallikrein by lanadelumab, a monoclonal antibody adminis-tered subcutaneously, is effective and well tolerated.

Published
2020

29. 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

31. Anti-C1q Antibodies as Occurring in Systemic Lupus Erythematosus Could Be Induced by an Epstein-Barr Virus-Derived Antigenic Site

Author

Csorba, K., Schirmbeck, L.A., Tuncer, E., Ribi, C., Roux-Lombard, P., Chizzolini, C., Huynh-Do, U., Vanhecke, D., and Trendelenburg, M.

Subjects
Adult, Male, lcsh:Immunologic diseases. Allergy, Herpesvirus 4, Human, Adolescent, Immunology, anti-C1q antibody, chemical and pharmacologic phenomena, 610 Medicine & health, urologic and male genital diseases, Mice, Young Adult, A08 epitope, fluids and secretions, systemic lupus erythematosus, immune system diseases, Animals, Humans, Lupus Erythematosus, Systemic, Epstein-Barr virus, molecular mimicry, skin and connective tissue diseases, Original Research, Aged, Autoantibodies, ddc:616, Aged, 80 and over, C1q deficient mice, EBNA-1, autoimmunity, Complement C1q, Middle Aged, Mice, Inbred C57BL, Epstein-Barr Virus Nuclear Antigens, Female, lcsh:RC581-607
Abstract

Previous infection with Epstein-Barr virus (EBV) is believed to trigger autoimmunity and to drive autoantibody generation as occurring in patients with systemic lupus erythematosus (SLE). Complement C1q and autoantibodies targeting it (anti-C1q) are also considered to be involved in the pathogenesis of SLE, independently of the impact of environmental insults. Still, the circumstances under which these autoantibodies arise remain elusive. By studying a major antigenic site of C1q targeted by anti-C1q (A08), we aimed to determine environmental factors and possible mechanisms leading to the development of anti-C1q. First, we determined antigenic residues of A08 that were critical for the binding of anti-C1q; importantly, we found the binding to depend on amino-acid-identity. Anti-C1q of SLE patients targeting these critical antigenic residues specifically cross-reacted with the EBV-related EBNA-1 (Epstein-Barr virus nuclear antigen 1)-derived peptide EBNA348. In a cohort of 180 SLE patients we confirmed that patients that were seropositive for EBV and recognized the EBNA348 peptide had increased levels of anti-A08 and anti-C1q, respectively. The correlation of anti-EBNA348 with anti-A08 levels was stronger in SLE patients than in matched healthy controls. Finally, EBNA348 peptide-immunization of C1q -/- mice induced the generation of cross-reactive antibodies which recognized both the A08 epitope of C1q and intact C1q. These findings suggest that anti-C1q in SLE patients could be induced by an EBV-derived epitope through molecular mimicry, thereby further supporting the pathogenic role of EBV in the development of SLE. Considering the role of C1q and anti-C1q, modifying the anti-EBV response might be a promising strategy to improve the course of the disease.

Published
2019
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32. 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

33. New therapeutic perspectives to manage refractory immune checkpoint-related toxicities

Author

Martins, F., Sykiotis, G.P., Maillard, M., Fraga, M., Ribi, C., Kuntzer, T., Michielin, O., Peters, S., Coukos, G., Spertini, F., Thompson, J.A., and Obeid, M.

Subjects
Algorithms, Antibodies, Monoclonal/adverse effects, Antineoplastic Agents/adverse effects, Drug-Related Side Effects and Adverse Reactions/drug therapy, Drug-Related Side Effects and Adverse Reactions/immunology, Drug-Related Side Effects and Adverse Reactions/pathology, Drug-Related Side Effects and Adverse Reactions/physiopathology, Humans, Immunosuppressive Agents/adverse effects, Immunosuppressive Agents/therapeutic use, Immunotherapy/adverse effects, Neoplasms/therapy, Precision Medicine
Abstract

Immune checkpoint inhibitors are reshaping the prognosis of many cancer and are progressively becoming the standard of care in the treatment of many tumour types. Immunotherapy is bringing new hope to patients, but also a whole new spectrum of toxicities for healthcare practitioners to manage. Oncologists and specialists involved in the pluridisciplinary management of patients with cancer are increasingly confronted with the therapeutic challenge of treating patients with severe and refractory immune-related adverse events. In this Personal View, we summarise the therapeutic strategies that have been used to manage such toxicities resulting from immune checkpoint inhibitor treatment. On the basis of current knowledge about their pathogenesis, we discuss the use of new biological and non-biological immunosuppressive drugs to treat severe and steroid refractory immune-related adverse events. Depending on the immune infiltrate type that is predominant, we propose a treatment algorithm for personalised management that goes beyond typical corticosteroid use. We propose a so-called shut-off strategy that aims at inhibiting key inflammatory components involved in the pathophysiological processes of immune-related adverse events, and limits potential adverse effects of drug immunosuppression on tumour response. This approach develops on current guidelines and challenges the step-by-step increase approach to drug immunosuppression.

Published
2019

34. 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

35. Predictors of disease worsening defined by progression of organ damage in diffuse systemic sclerosis: a European Scleroderma Trials and Research (EUSTAR) analysis

Author

Becker M, Graf N, Sauter R, Curram J, Denton C, Khanna D, Pena J, Pope J, Distler O, Matucci-Cerinic M, Guiducci S, Walker U, Jaeger V, Bannert B, Lapadula G, Becvarare R, Cutolo M, Valentini G, Siegert E, Rednic S, Allanore Y, Montecucco C, Carreira P, Novak S, Czirjak L, Varju C, Chizzolini C, Allai D, Kucharz E, Cozzi F, Rozman B, Mallia C, Gabrielli A, Bancel D, Airo P, Hesselstrand R, Martinovic D, Balbir-Gurman A, Braun-Moscovici Y, Hunzelmann N, Pellerito R, Caramaschi P, Black C, Damjanov N, Henes J, Santamaria V, Heitmann S, Seidel M, Da Silva J, Stamenkovic B, Selmi C, Tikly M, Denisov L, Muller-Ladner U, Engelhart M, Hachulla E, Riccieri V, Ionescu R, Mihai C, Sunderkotter C, Kuhn A, Schett G, Distler J, Meroni P, Ingegnoli F, Mouthon L, De Keyser F, Smith V, Cantatore F, Corrado A, Ullman S, Iversen L, Pozzi M, Eyerich K, Hein R, Knott E, Wiland P, Szmyrka-Kaczmarek M, Sokolik R, Morgiel E, Madej M, Alegre-Sancho J, Krummel-Lorenz B, Saar P, Aringer M, Gunther C, Anne E, Westhovens R, De Langhe E, Lenaerts J, Anic B, Baresic M, Mayer M, Uprus M, Otsa K, Yavuz S, Radominski S, Muller C, Azevedo V, Popa S, Zenone T, Stebbings S, Highton J, Mathieu A, Vacca A, Stamp L, Chapman P, O'Donnell J, Solanki K, Doube A, Veale D, O'Rourke M, Loyo E, Li M, Rosato E, Amoroso A, Gigante A, Oksel F, Yargucu F, Tanaseanu C, Popescu M, Dumitrascu A, Tiglea I, Foti R, Visalli E, Benenati A, Amato G, Ancuta C, Chirieac R, Villiger P, Adler S, Dan D, Lefebvre P, Rubio S, Exposito M, Sibilia J, Chatelus E, Gottenberg J, Chifflot H, Litinsky I, Del Galdo F, Venalis A, Saketkoo L, Lasky J, Kerzberg E, Montoya F, Cosentino V, Limonta M, Brucato A, Lupi E, Spertini F, Ribi C, Buss G, Martin T, Guffroy A, Poindron V, Chung L, Schmeiser T, Zebryk P, Riso N, Riemekasten G, Rezus E, Puttini P, and EUSTAR Collaborators

Abstract

Objectives Mortality and worsening of organ function are desirable endpoints for clinical trials in systemic sclerosis (SSc). The aim of this study was to identify factors that allow enrichment of patients with these endpoints, in a population of patients from the European Scleroderma Trials and Research group database. Methods Inclusion criteria were diagnosis of diffuse SSc and follow-up over 12 +/- 3 months. Disease worsening/organ progression was fulfilled if any of the following events occurred: new renal crisis; decrease of lung or heart function; new echocardiography-suspected pulmonary hypertension or death. In total, 42 clinical parameters were chosen as predictors for the analysis by using (1) imputation of missing data on the basis of multivariate imputation and (2) least absolute shrinkage and selection operator regression. Results Of 1451 patients meeting the inclusion criteria, 706 had complete data on outcome parameters and were included in the analysis. Of the 42 outcome predictors, eight remained in the final regression model. There was substantial evidence for a strong association between disease progression and age, active digital ulcer (DU), lung fibrosis, muscle weakness and elevated C-reactive protein (CRP) level. Active DU, CRP elevation, lung fibrosis and muscle weakness were also associated with a significantly shorter time to disease progression. A bootstrap validation step with 10 000 repetitions successfully validated the model. Conclusions The use of the predictive factors presented here could enable cohort enrichment with patients at risk for overall disease worsening in SSc clinical trials.

Published
2019

41. S6D:4 Antibodies to myelin oligodendrocyte glycoprotein in patients with sle are associated with central nervous system involvement: an unbiased pilot study of the swiss sle cohort study (sscs)

Author

Pröbstel, A, primary, Thanei, M, additional, Erni, B, additional, Lecourt, A-C, additional, Branco, L, additional, André, R, additional, Koenig, KF, additional, Huynh-Do, U, additional, Ribi, C, additional, Chizzolini, C, additional, Kappos, L, additional, Trendelenburg, M, additional, and Derfuss, T, additional

Published
2018
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44. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database

Author

Walker U. A., Tyndall A., Czirják L, Denton C. ., Farge Bancel D., Kowal Bielecka O., Müller Ladner U., Bocelli Tyndall C., Matucci Cerinic M., Riemekasten G. ., Brückner C. ., Airó P. ., Scarsi M. ., Scorza R. ., Beretta L. ., Cozzi F. ., Tiso F., Vonk Mc M. C., Hoogen Van Den, Fhj F. H. J., Wigley Fm F. M., Hummers L. ., Nevskaya T. ., Ananieva L. ., Miniati I. ., Tartaglia N. ., Lomater C. ., Balbir Gurman A. ., Braun Moscovici Y. ., Bambara Lm L. M., Caramaschi P. ., Ruocco L. ., Krieg T. ., Hunzelmann N. ., Varjú C. ., Carriera Pe P. E., Joven B. ., Iannone F. ., Lapadula G. ., Kahan A. ., Allanore Y. ., Gabrielli A. ., Imperatore M. ., Scheja A. ., Wollheim F. ., Damjanov N. ., Ostojic P. ., Saar P. ., Tarner Ih I. H., Kötter I. ., Bombardieri S. ., Bazzichi L. ., Papa Del N. ., Comina Dp D. P., Monaco Lo A. ., Corte La R. ., Hachulla E. ., Launay D. ., Distler O. ., Ciurea A. ., Sierakowski S. ., Mitchell H. ., Silver Rm R. M., Krasowska D. ., Michalska Jakubus M. ., Tikly M. ., Aboo N. ., Worm M. ., Klaus P. ., Rovenský J. ., Lukáčová O. ., Rozman B. ., Sipek A. ., Clemente Coelho P. ., Shoenfeld Y. ., Langewitch P. ., José Da Silva Ap A. P., Salvador Mj M. J., Kuhn A. ., Erdmann G. ., Bečvář R. ., Friedl E. ., Graninger W. ., Riccieri V. ., Caporali R. ., Montecucco C. ., Vlachoyiannopoulos P. ., Distler M. ., Reich K. ., Majdan M. ., Wielosz E. ., Rednic S. ., Laar Van Jm J. M., Heitmann S. ., Bruckner A. ., Himsel A. ., Riemann J. ., Meyringer R. ., Müller A. ., Martinovic D. ., Radic M. ., Sticherling M. ., Szekanecz Z. ., Szücs G. ., Giacomelli R. ., Marrelli A. ., Stamenkovic B. ., Stankovic A. ., Aringer M. ., Smolen Js J. S., Kucharz Ej E. J., Kotulska At A. T., Jablonska S. ., Blasczik M. ., Jun J. B. J. B., Mallia C. ., Coleiro B. ., Santamaria Vo V. O., Hinrichs R. ., Nielsen H. ., Cossutta R. ., Ionescu R. ., Opris D. ., Steinbrink K. ., Grundt B. ., Bajocchi G. ., Jiří Š. ., Lefebvre Pgdlp P. G. D. L. P., Mendoza ZeaAc A. C., Ribi C. ., Chizzolini C. ., Wisłowska M. ., Novak S. ., Indiveri F. ., Jacobsen S. ., Frandsen Pb P. B., Gorska Iz I. Z., Gran Tore J. ., Midtvedt Ø. ., Ramos Fo F. O., Rajcevska Ld L. D., Bozinovski G. ., Schöffel D. ., Sunderkötter C. ., Böhm M. ., Morović Vergles J. ., Čulo M. I. M. I., Cutolo M. ., Sulli A. ., Derk Ct C. T., Jimenez Sa S. A., Siakka P. ., Søndergaard K. ., Stengaard Pedersen K. ., Cabane J. ., Tiev Kp K. P., Mihai C. ., Sfrent Cornateanu R. ., Jendro M. ., Tuvik P. ., Antivalle M. ., Randisi G. ., Seidel M. ., Clarenbach R. ., Simsek I. ., Dinc A. ., Inanc M. ., Capraru Ms M. S., Capraru D. ., Bañegil I. ., Richter J. ., Alhasani S. ., Földvari I. ., Pinto S. ., Brandão F. ., VALENTINI, Gabriele, Walker, U. A., Tyndall, A., Czirják, L, Denton, C. ., Farge Bancel, D., Kowal Bielecka, O., Müller Ladner, U., Bocelli Tyndall, C., Matucci Cerinic, M., Riemekasten, G. ., Brückner, C. ., Airó, P. ., Scarsi, M. ., Scorza, R. ., Beretta, L. ., Cozzi, F. ., Tiso, F., Vonk Mc, M. C., Hoogen Van, Den, Fhj, F. H. J., Wigley Fm, F. M., Hummers, L. ., Nevskaya, T. ., Ananieva, L. ., Miniati, I. ., Tartaglia, N. ., Lomater, C. ., Balbir Gurman, A. ., Braun Moscovici, Y. ., Bambara Lm, L. M., Caramaschi, P. ., Valentini, Gabriele, Ruocco, L. ., Krieg, T. ., Hunzelmann, N. ., Varjú, C. ., Carriera Pe, P. E., Joven, B. ., Iannone, F. ., Lapadula, G. ., Kahan, A. ., Allanore, Y. ., Gabrielli, A. ., Imperatore, M. ., Scheja, A. ., Wollheim, F. ., Damjanov, N. ., Ostojic, P. ., Saar, P. ., Tarner Ih, I. H., Kötter, I. ., Bombardieri, S. ., Bazzichi, L. ., Papa Del, N. ., Comina Dp, D. P., Monaco Lo, A. ., Corte La, R. ., Hachulla, E. ., Launay, D. ., Distler, O. ., Ciurea, A. ., Sierakowski, S. ., Mitchell, H. ., Silver Rm, R. M., Krasowska, D. ., Michalska Jakubus, M. ., Tikly, M. ., Aboo, N. ., Worm, M. ., Klaus, P. ., Rovenský, J. ., Lukáčová, O. ., Rozman, B. ., Sipek, A. ., Clemente Coelho, P. ., Shoenfeld, Y. ., Langewitch, P. ., José Da Silva Ap, A. P., Salvador Mj, M. J., Kuhn, A. ., Erdmann, G. ., Bečvář, R. ., Friedl, E. ., Graninger, W. ., Riccieri, V. ., Caporali, R. ., Montecucco, C. ., Vlachoyiannopoulos, P. ., Distler, M. ., Reich, K. ., Majdan, M. ., Wielosz, E. ., Rednic, S. ., Laar Van Jm, J. M., Heitmann, S. ., Bruckner, A. ., Himsel, A. ., Riemann, J. ., Meyringer, R. ., Müller, A. ., Martinovic, D. ., Radic, M. ., Sticherling, M. ., Szekanecz, Z. ., Szücs, G. ., Giacomelli, R. ., Marrelli, A. ., Stamenkovic, B. ., Stankovic, A. ., Aringer, M. ., Smolen Js, J. S., Kucharz Ej, E. J., Kotulska At, A. T., Jablonska, S. ., Blasczik, M. ., Jun, J. B. J. B., Mallia, C. ., Coleiro, B. ., Santamaria Vo, V. O., Hinrichs, R. ., Nielsen, H. ., Cossutta, R. ., Ionescu, R. ., Opris, D. ., Steinbrink, K. ., Grundt, B. ., Bajocchi, G. ., Jiří, Š. ., Lefebvre Pgdlp, P. G. D. L. P., Mendoza ZeaAc, A. C., Ribi, C. ., Chizzolini, C. ., Wisłowska, M. ., Novak, S. ., Indiveri, F. ., Jacobsen, S. ., Frandsen Pb, P. B., Gorska Iz, I. Z., Gran Tore, J. ., Midtvedt, Ø. ., Ramos Fo, F. O., Rajcevska Ld, L. D., Bozinovski, G. ., Schöffel, D. ., Sunderkötter, C. ., Böhm, M. ., Morović Vergles, J. ., Čulo, M. I. M. I., Cutolo, M. ., Sulli, A. ., Derk Ct, C. T., Jimenez Sa, S. A., Siakka, P. ., Søndergaard, K. ., Stengaard Pedersen, K. ., Cabane, J. ., Tiev Kp, K. P., Mihai, C. ., Sfrent Cornateanu, R. ., Jendro, M. ., Tuvik, P. ., Antivalle, M. ., Randisi, G. ., Seidel, M. ., Clarenbach, R. ., Simsek, I. ., Dinc, A. ., Inanc, M. ., Capraru Ms, M. S., Capraru, D. ., Bañegil, I. ., Richter, J. ., Alhasani, S. ., Földvari, I. ., Pinto, S. ., Brandão, F. ., Ribi, Camillo, and Chizzolini, Carlo

Subjects
Male, Systemic disease, Databases, Factual, Cross-sectional study, Scleroderma, Immunopathology, Immunology and Allergy, Age of Onset, skin and connective tissue diseases, ddc:616, integumentary system, Nuclear Proteins, Orvostudományok, Middle Aged, Connective tissue disease, Extended Report, Raynaud Disease/etiology/immunology, DNA Topoisomerases, Type I, Nuclear Proteins/immunology, Female, Adult, medicine.medical_specialty, Scleroderma, Diffuse/complications/immunology, Immunology, Klinikai orvostudományok, Scleroderma, Systemic/complications/immunology, Risk Assessment, General Biochemistry, Genetics and Molecular Biology, systemic sclerosis, EULAR, risk assessment, Age Distribution, Sex Factors, Rheumatology, Scleroderma, Limited, medicine, Humans, Risk factor, Aged, Autoantibodies, Scleroderma, Systemic, business.industry, Autoantibody, Raynaud Disease, medicine.disease, Dermatology, Cross-Sectional Studies, Scleroderma, Diffuse, Scleroderma, Limited/complications/immunology, Age of onset, business, Autoantibodies/blood
Abstract

Background: Systemic sclerosis (SSc) is a multisystem autoimmune disease which is classified into a diffuse cutaneous (dcSSc) and a limited cutaneous (lcSSc) subset according to the skin involvement. In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004. Aims and Methods: EUSTAR collects prospectively the Minimal Essential Data Set (MEDS) on all sequential patients fulfilling the ACR diagnostic criteria in participating centres. We aimed to characterize demographic, clinical and laboratory characteristics of disease presentation in SSc and analysed EUSTAR baseline visits. Results: In April 2006, a total of 3656 patients (1349 with dcSSc and 2101 with lcSSc) were enrolled in 102 centres and 30 countries. 1330 individuals had autoantibodies against Scl70 and 1106 against anticentromere antibodies. 87% of patients were female. On multivariate analysis, scleroderma subsets (dcSSc vs. lcSSc), antibody status and age at onset of Raynaud’s phenomenon, but not gender were independently associated with the prevalence of organ manifestations. Autoantibody status in this analysis appeared more closely associated with clinical manifestations than were SSc subsets. Conclusion: dcSSc and lcSSc subsets are associated with particular organ manifestations, but in this analysis the clinical distinction appeared superseded by an antibody based classification in predicting some scleroderma complications. The EUSTAR MEDS data base facilitates the analysis of clinical patterns in SSc and contributes to the standardised assessment and monitoring of SSc internationally.

Published
2007

50. Clinical risk assessment of organ manifestations in systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group database

Author

Walker, U.A. Tyndall, A. Czirják, L. Denton, C. Farge-Bancel, D. Kowal-Bielecka, O. Müller-Ladner, U. Bocelli-Tyndall, C. Matucci-Cerinic, M. Riemekasten, G. Brückner, C. Airó, P. Scarsi, M. Scorza, R. Beretta, L. Cozzi, F. Tiso, F. Vonk, M.C. Van Den Hoogen, F.H.J. Wigley, F.M. Hummers, L. Nevskaya, T. Ananieva, L. Miniati, I. Tartaglia, N. Lomater, C. Balbir-Gurman, A. Braun-Moscovici, Y. Bambara, L.M. Caramaschi, P. Valentini, G. Ruocco, L. Krieg, T. Hunzelmann, N. Varjú, C. Carriera, P.E. Joven, B. Iannone, F. Lapadula, G. Kahan, A. Allanore, Y. Gabrielli, A. Imperatore, M. Scheja, A. Wollheim, F. Damjanov, N. Ostojic, P. Saar, P. Tarner, I.H. Kötter, I. Bombardieri, S. Bazzichi, L. Del Papa, N. Comina, D.P. Lo Monaco, A. La Corte, R. Hachulla, E. Launay, D. Distler, O. Ciurea, A. Sierakowski, S. Mitchell, H. Silver, R.M. Krasowska, D. Michalska-Jakubus, M. Tikly, M. Aboo, N. Worm, M. Klaus, P. Rovenský, J. Lukáčová, O. Rozman, B. Sipek, A. Clemente-Coelho, P. Shoenfeld, Y. Langewitch, P. Da Silva José, A.P. Salvador, M.J. Kuhn, A. Erdmann, G. Bečvář, R. Friedl, E. Graninger, W. Riccieri, V. Caporali, R. Montecucco, C. Vlachoyiannopoulos, P. Distler, M. Reich, K. Majdan, M. Wielosz, E. Rednic, S. Van Laar, J.M. Heitmann, S. Bruckner, A. Himsel, A. Riemann, J. Meyringer, R. Müller, A. Martinovic, D. Radic, M. Sticherling, M. Szekanecz, Z. Szücs, G. Giacomelli, R. Marrelli, A. Stamenkovic, B. Stankovic, A. Aringer, M. Smolen, J.S. Kucharz, E.J. Kotulska, A.T. Jablonska, S. Blasczik, M. Jun, J.-B. Mallia, C. Coleiro, B. Santamaria, V.O. Hinrichs, R. Nielsen, H. Cossutta, R. Ionescu, R. Opris, D. Steinbrink, K. Grundt, B. Bajocchi, G. Jiří, Š. Lefebvre, P.G.D.L.P. Zea Mendoza, A.C. Ribi, C. Chizzolini, C. Wisłowska, M. Novak, S. Indiveri, F. Jacobsen, S. Frandsen, P.B. Gorska, I.Z. Tore Gran, J. Midtvedt, Ø. Ramos, F.O. Rajcevska, L.D. Bozinovski, G. Schöffel, D. Sunderkötter, C. Böhm, M. Morović-Vergles, J. Čulo, M.-I. Cutolo, M. Sulli, A. Derk, C.T. Jimenez, S.A. Siakka, P. Søndergaard, K. Stengaard-Pedersen, K. Cabane, J. Tiev, K.P. Mihai, C. Sfrent-Cornateanu, R. Jendro, M. Tuvik, P. Antivalle, M. Randisi, G. Seidel, M. Clarenbach, R. Simsek, I. Dinc, A. Inanc, M. Capraru, M.S. Capraru, D. Bañegil, I. Richter, J. Alhasani, S. Földvari, I. Pinto, S. Brandão, F. Mas, A.J.

Subjects
integumentary system, skin and connective tissue diseases
Abstract

Background: Systemic sclerosis (SSc) is a multisystem autoimmune disease, which is classified into a diffuse cutaneous (dcSSc) and a limited cutaneous (IcSSc) subset according to the skin involvement. In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment, the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004. Aims and methods: EUSTAR collects prospectively the Minimal Essential Data Set (MEDS) on all sequential patients fulfilling the American College of Rheumatology diagnostic criteria in participating centres. We aimed to characterise demographic, clinical and laboratory characteristics of disease presentation in SSc and analysed EUSTAR baseline visits. Results: In April 2006, a total of 3656 patients (1349 with dcSSc and 2101 with IcSSc) were enrolled in 102 centres and 30 countries. 1330 individuals had autoantibodies against Scl70 and 1106 against anticentromere antibodies. 87% of patients were women. On multivariate analysis, scleroderma subsets (dcSSc vs IcSSc), antibody status and age at onset of Raynaud's phenomenon, but not gender, were found to be independently associated with the prevalence of organ manifestations. Autoantibody status in this analysis was more closely associated with clinical manifestations than were SSc subsets. Conclusion: dcSSc and IcSSc subsets are associated with particular organ manifestations, but in this analysis the clinical distinction seemed to be superseded by an antibody-based classification in predicting some scleroderma complications. The EUSTAR MEDS database facilitates the analysis of clinical patterns in SSc, and contributes to the standardised assessment and monitoring of SSc internationally.

Published
2007

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