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2. POS0211 PAH TREATMENT AT TIME OF DIAGNOSIS IS ASSOCIATED WITH IMPROVED SURVIVAL REGARDLESS OF HEMODYNAMIC THRESHOLDS AND RISK STRATIFICATION - A EUSTAR ANALYSIS

Author

Bjørkekjær, H. J., primary, Bruni, C., additional, Brunborg, C., additional, Carreira, P., additional, Airò, P., additional, Simeón-Aznar, C. P., additional, Truchetet, M. E., additional, Giollo, A., additional, Balbir-Gurman, A., additional, Martin, M., additional, Denton, C. P., additional, Gabrielli, A., additional, Fretheim, H., additional, Bitter, H., additional, Midtvedt, Ø., additional, Broch, K., additional, Andreassen, A., additional, Høie, S., additional, Tanaka, Y., additional, Riemekasten, G., additional, Müller-Ladner, U., additional, Matucci-Cerinic, M., additional, Castellví, I., additional, Siegert, E., additional, Hachulla, E., additional, Molberg, Ø., additional, Distler, O., additional, and Hoffmann-Vold, A. M., additional

Published
2024
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3. AB1196 BASELINE DEMOGRAPHICS AND DISEASE CHARACTERISTICS IN SUBJECTS WITH ILD IN A PHASE 2 STUDY TO EVALUATE EFFICACY, SAFETY, AND TOLERABILITY OF MT-7117 IN DIFFUSE CUTANEOUS SYSTEMIC SCLEROSIS

Author

Khanna, D., primary, Carreira, P., additional, Chung, L., additional, Denton, C. P., additional, Matucci-Cerinic, M., additional, Riemekasten, G., additional, Yanai, Y., additional, Cai, S., additional, Inagaki, M., additional, Sasson, N., additional, and Bogoslovsky, T., additional

Published
2024
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4. POS0832 IMMUNOSUPPRESSION VERSUS COMBINATION OF IMMUNOSUPPRESSION AND ORAL GLUCOCORTICOIDS FOR SKIN FIBROSIS IN EARLY DIFFUSE SYSTEMIC SCLEROSIS PATIENTS. A TARGET TRIAL EMULATION STUDY FROM THE EUSTAR DATABASE

Author

Mongin, D., primary, Matucci-Cerinic, M., additional, Walker, U., additional, Distler, O., additional, Bečvář, R., additional, Siegert, E., additional, Ananyeva, L. P., additional, Smith, V., additional, Alegre Sancho, J. J., additional, Yavuz, S., additional, Limonta, M., additional, Riemekasten, G., additional, Rezus, E., additional, Vonk, M., additional, Truchetet, M. E., additional, Del Galdo, F., additional, Courvoisier, D. S., additional, and Iudici, M., additional

Published
2024
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5. OP0013 ANTI-Ro/SSA ANTIBODIES ARE PREDICTIVE OF A MORE SEVERE LUNG INVOLVEMENT IN PATIENTS WITH SYSTEMIC SCLEROSIS: A STUDY FROM THE EUSTAR HDATABASE

Author

Burja, B., primary, Boubaya, M., additional, Bruni, C., additional, Carreira, P., additional, Bergmann, C., additional, Ananyeva, L. P., additional, Riemekasten, G., additional, Masado, O., additional, De Vries-Bouwstra, J. K., additional, Rosato, E., additional, Truchetet, M. E., additional, Del Papa, N., additional, Marcoccia, A., additional, Atzeni, F., additional, Schmeiser, T., additional, Vonk, M., additional, Del Galdo, F., additional, Distler, O., additional, and Elhai, M., additional

Published
2024
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6. Versorgungsrealität der stationären vasoaktiven Therapie mit Prostazyklinderivaten bei Patienten mit akralen Durchblutungsstörungen bei systemischer Sklerose in Deutschland

Author

Juche, A., Siegert, E., Mueller-Ladner, U., Riemekasten, G., Günther, C., Kötter, I., Henes, J., Blank, N., Voll, R. E., Ehrchen, J., Schmalzing, M., Susok, L., Schmeiser, T., Sunderkoetter, C., Distler, J., Worm, M., Kreuter, A., Horváth, O. N., Schön, M. P., Korsten, P., Zeidler, G., Pfeiffer, C., Krieg, T., Hunzelmann, N., and Moinzadeh, P.

Published
2020
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7. Serum Levels of Autoantibodies Against Extracellular Antigens and Neutrophil Granule Proteins Increase in Patients with COPD Compared to Non-COPD Smokers

Author

Ma A, Wen L, Yin J, Hu Y, Yue X, Li J, Dong X, Gupta Y, Ludwig RJ, Krauss-Etschmann S, Riemekasten G, Petersen F, and Yu X

Subjects
chronic obstructive pulmonary disease, autoimmunity, autoantibody profile, neutrophil granule proteins, lactorferrin., Diseases of the respiratory system, RC705-779
Abstract

Aiping Ma,1,* Lifang Wen,2,* Junping Yin,3 Yi Hu,4 Xiaoyang Yue,3 Jiurong Li,1 Xiaoru Dong,2 Yask Gupta,5 Ralf J Ludwig,5 Susanne Krauss-Etschmann,3,6 Gabriela Riemekasten,7 Frank Petersen,3 Xinhua Yu2,3 1Department of Respiratory Medicine, The First Affiliated Hospital, School of Medicine, Xiamen University, Xiamen, People’s Republic of China; 2Xiamen-Borstel Joint Laboratory of Autoimmunity, The Medical College of Xiamen University; 3Priority Area Asthma and Allergy, Research Center Borstel, Airway Research Center North (ARCN), Members of the German Center for Lung Research (DZL), Borstel, Germany; 4Department of Clinical Laboratory, The First Affiliated Hospital, School of Medicine, Xiamen University, Xiamen, People’s Republic of China; 5Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany; 6Institute for Experimental Medicine, Christian-Albrechts-Universitaetzu Kiel, Kiel, Germany; 7Department of Rheumatology, University of Lübeck, Lübeck 23538, Germany*These authors contributed equally to this workCorrespondence: Xinhua YuPriority Area Asthma and Allergy, Research Center Borstel, Borstel 23845, GermanyTel +49 453 7188 2520Email xinhuayu@fz-borstel.deBackground: Chronic obstructive pulmonary disease (COPD) is a highly prevalent disease leading to irreversible airflow limitation and is characterized by chronic pulmonary inflammation, obstructive bronchiolitis and emphysema. Etiologically, COPD is mediated by toxic gases and particles, eg, cigarette smoke, while the pathogenesis of the disease is largely unknown. Several lines of evidence indicate a link between COPD and autoimmunity but comprehensive studies are lacking.Methods: By using a protein microarray assaying more than 19,000 human proteins we determined in this study the autoantibody profiles of COPD and non-COPD smokers. The discovery cohort included 5 COPD patients under acute exacerbation (AECOPD) and 5 age- and gender-matched non-COPD smokers. One putative candidate autoantibody, anti-lactoferrin IgG, was further investigated by using immunoblotting with a large validation cohort containing 124 healthy controls, 92 patients with AECOPD and 52 patients with stable COPD.Results: We show that i) autoantigens targeted by autoantibodies with higher titers in COPD patients were enriched in extracellular regions, while those with lower titers in COPD patients were enriched in intracellular compartments. ii) levels of IgG autoantibodies against many neutrophil granule proteins were significantly higher in COPD patients than in non-COPD smokers. Furthermore, increased levels of anti-lactoferrin antibodies in COPD patients were confirmed in a cohort with a large number of samples.Conclusion: The comprehensive autoantibody profiles from COPD patients established in this study demonstrated for the first time a shift in the cellular localization of antigens targeted by autoantibodies in COPD.Keywords: chronic obstructive pulmonary disease, autoimmunity, autoantibody profile, neutrophil granule proteins, lactoferrin

Published
2020

8. 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

12. OP0238 IMMUNOSUPPRESSION WITH TARGETED DMARDS REDUCES MORBIDITY AND MORTALITY IN PRE-CAPILLARY PULMONARY HYPERTENSION ASSOCIATED WITH SYSTEMIC SCLEROSIS: A EUSTAR ANALYSIS

Author

Bruni, C., primary, Tofani, L., additional, Fretheim, H., additional, Weber, Y., additional, Hachulla, E., additional, Carreira, P., additional, Giuggioli, D., additional, Airò, P., additional, Siegert, E., additional, Müller-Ladner, U., additional, Matucci-Cerinic, M., additional, Riemekasten, G., additional, Simeon Aznar, C. P., additional, De Vries-Bouwstra, J., additional, Saketkoo, L. A., additional, Distler, J., additional, Balbir-Gurman, A., additional, Castellví, I., additional, Zanatta, E., additional, Smith, V., additional, Denton, C. P., additional, Maurer, B., additional, Giollo, A., additional, Iannone, F., additional, Dagna, L., additional, Truchetet, M. E., additional, Kuwana, M., additional, Allanore, Y., additional, Tanaka, Y., additional, Martin, M., additional, Rosato, E., additional, Gheorghiu, A. M., additional, Del Galdo, F., additional, Solanki, K., additional, Vacca, A., additional, Resende, C., additional, Vieira, S., additional, Czirják, L., additional, Baresic, M., additional, Cantatore, F. P., additional, Riccieri, V., additional, Andréasson, K., additional, Chung, L., additional, Souza Muller, C., additional, Opris-Belinski, D., additional, Rednic, S., additional, Sfikakis, P., additional, Levy, Y., additional, Hsu, V., additional, Heitmann, S., additional, Henes, J., additional, Moroncini, G., additional, Iudici, M., additional, De Langhe, E., additional, Herrick, A., additional, Montecucco, C., additional, Hoffmann-Vold, A. M., additional, and Distler, O., additional

Published
2023
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13. POS0150 AUTOANTIBODIES AGAINST FIBROBLAST GROWTH FACTOR (FGF-2), PLACENTAL GROWTH FACTOR (PLGF) AND BETA-ADRENERGIC RECEPTOR 1 (ADRB1) IN AN ALTERED NETWORK OF AUTOANTIBODIES IN SYSTEMIC SCLEROSIS

Author

Schinke, S., primary, Sterner, K., additional, Heidecke, H., additional, Busch, H., additional, Kuenstner, A., additional, König, I., additional, Fouodo, C. J. K., additional, Müller, A., additional, Comduehr, S., additional, Lange, T., additional, Lübber, F., additional, Paulus, F., additional, Grasshoff, H., additional, and Riemekasten, G., additional

Published
2023
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15. POS0121 COMPARISON OF FOUR RISK STRATIFICATION MODELS FOR PREDICTION OF MORTALITY IN SSc-PAH IN THE EUSTAR COHORT

Author

Bjørkekjær, H. J., primary, Bruni, C., additional, Carreira, P., additional, Airò, P., additional, Simeon Aznar, C. P., additional, Truchetet, M. E., additional, Giollo, A., additional, Balbir-Gurman, A., additional, Martin, M., additional, Denton, C. P., additional, Gabrielli, A., additional, Fretheim, H., additional, Barua, I., additional, Bitter, H., additional, Midtvedt, Ø., additional, Garen, T., additional, Broch, K., additional, Andreassen, A., additional, Tanaka, Y., additional, Riemekasten, G., additional, Müller-Ladner, U., additional, Matucci-Cerinic, M., additional, Castellví, I., additional, Siegert, E., additional, Hachulla, E., additional, Distler, O., additional, and Hoffmann-Vold, A. M., additional

Published
2023
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19. Classification, categorization and essential items for digital ulcer evaluation in systemic sclerosis: a DeSScipher/European Scleroderma Trials and Research group (EUSTAR) survey

Author

Blagojevic, J., Bellando-Randone, S., Abignano, G., Avouac, J., Cometi, L., Czirják, L., Denton, C. P., Distler, O., Frerix, M., Guiducci, S., Huscher, D., Jaeger, V. K., Lóránd, V., Maurer, B., Nihtyanova, S., Riemekasten, G., Siegert, E., Tarner, I. H., Vettori, S., Walker, U. A., Allanore, Y., Müller-Ladner, U., Del Galdo, F., Matucci-Cerinic, M., and EUSTAR co-workers

Published
2019
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20. Fighting Post-COVID and ME/CFS – development of curative therapies

Author

Scheibenbogen, C., Bellmann-Strobl, J.T., Heindrich, C., Wittke, K., Stein, E., Franke, C., Prüss, H., Preßler, H., Machule, M.L., Audebert, H., Finke, C., Zimmermann, H.G., Sawitzki, B., Meisel, C., Toelle, M., Krueger, A., Aschenbrenner, A.C., Schultze, J.L., Beyer, M.D., Ralser, M., Mülleder, M., Sander, L.E., Konietschke, F., Paul, F., Stojanov, S., Bruckert, L., Hedderich, D.M., Knolle, F., Riemekasten, G., Vehreschild, M.J.G.T., Cornely, O.A., Behrends, U., and Burock, S.

Subjects
clinical trials, post-COVID, autoantibodies, inflammation, COVID-19, ddc:610, General Medicine, Function and Dysfunction of the Nervous System, ME/CFS, endothelial dysfunction
Abstract

The sequela of COVID-19 include a broad spectrum of symptoms that fall under the umbrella term post-COVID-19 condition or syndrome (PCS). Immune dysregulation, autoimmunity, endothelial dysfunction, viral persistence, and viral reactivation have been identified as potential mechanisms. However, there is heterogeneity in expression of biomarkers, and it is unknown yet whether these distinguish different clinical subgroups of PCS. There is an overlap of symptoms and pathomechanisms of PCS with postinfectious myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS). No curative therapies are available for ME/CFS or PCS. The mechanisms identified so far provide targets for therapeutic interventions. To accelerate the development of therapies, we propose evaluating drugs targeting different mechanisms in clinical trial networks using harmonized diagnostic and outcome criteria and subgrouping patients based on a thorough clinical profiling including a comprehensive diagnostic and biomarker phenotyping.

Published
2023
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26. Impact of systemic sclerosis-associated interstitial lung disease with and without pulmonary hypertension on survival in over 5,000 patients

Author

Moinzadeh, P, Sunderkötter, C, Ehrchen, J, Kötter, I, Korsten, P, Lorenz, HM, Müller-Ladner, U, Oberste, M, Kreuter, A, Susok, L, Hunzelmann, N, Siegert, E, Worm, M, Pfeiffer, C, Riemekasten, G, Jandova, I, Schmalzing, M, Kreuter, M, Ramming, A, Bonella, F, Henes, J, Schmeiser, T, Zeidler, G, Görl, N, Juche, A, Günther, C, Keyßer, G, Blank, N, Moinzadeh, P, Sunderkötter, C, Ehrchen, J, Kötter, I, Korsten, P, Lorenz, HM, Müller-Ladner, U, Oberste, M, Kreuter, A, Susok, L, Hunzelmann, N, Siegert, E, Worm, M, Pfeiffer, C, Riemekasten, G, Jandova, I, Schmalzing, M, Kreuter, M, Ramming, A, Bonella, F, Henes, J, Schmeiser, T, Zeidler, G, Görl, N, Juche, A, Günther, C, Keyßer, G, and Blank, N

Published
2023

31. Stratification in systemic sclerosis according to autoantibody status versus skin involvement: a study of the prospective EUSTAR cohort

Author

Elhai, M., Sritharan, N., Boubaya, M., Balbir-Gurman, A., Siegert, E., Hachulla, E., Vries-Bouwstra, J. de, Riemekasten, G., Distler, J.H.W., Rosato, E., Galdo, F. del, Mendoza, F.A., Furst, D.E., Puente, C. de la, Hoffmann-Vold, A.M., Gabrielli, A., Distler, O., Bloch-Queyrat, C., Allanore, Y., and USTAR Collaborators

Abstract

Background The current subclassification of systemic sclerosis into cutaneous subtypes does not fully capture the heterogeneity of the disease. We aimed to compare the performances of stratification into LeRoy's cutaneous subtypes versus stratification by autoantibody status in systemic sclerosis. Methods For this cohort study, we assessed people with systemic sclerosis in the multicentre international European Scleroderma Trials and Research (EUSTAR) database. Individuals positive for systemic-sclerosis autoantibodies of two specificities were excluded, and remaining individuals were classified by cutaneous subtype, according to their systemic sclerosis-specific autoantibodies, or both. We assessed the performance of each model to predict overall survival, progression-free survival, disease progression, and different organ involvement. The three models were compared by use of the area under the curve (AUC) of the receiver operating characteristic and the net reclassification improvement (NRI). Missing data were imputed. Findings We assessed the database on July 26, 2019. Of 16 939 patients assessed for eligibility, 10 711 patients were included: 1647 (15middot4%) of 10 709 were male, 9062 (84middot6%) were female, mean age was 54middot4 (SD 13middot8) years, and mean disease duration was 7middot9 (SD 8middot2) years. Information regarding cutaneous subtype was available for 10 176 participants and antibody data were available for 9643 participants. In the prognostic analysis, there was no difference in AUC for overall survival (0middot82, 95% CI 0middot81-0middot84 for cutaneous only vs 0middot84, 0middot82-0middot85 for antibody only vs 0middot84, 0middot83-0middot86 for combined) or for progression-free survival (0middot70, 0middot69-0middot71 vs 0middot71, 0middot70-0middot72 vs 0middot71, 0middot70-0middot72). However, at 4 years the NRI showed substantial improvement for the antibody-only model compared with the cutaneous-only model in prediction of overall survival (0middot57, 0middot46-0middot71 for antibody only vs 0middot29, 0middot19-0middot39 for cutaneous only) and disease progression (0middot36, 0middot29-0middot46 vs 0middot21, 0middot14-0middot28). The antibody -only model did better than the cutaneous-only model in predicting renal crisis (AUC 0middot72, 0middot70-0middot74 for antibody only vs 0middot66, 0middot64-0middot69 for cutaneous only) and lung fibrosis leading to restrictive lung function (AUC 0middot76, 0middot75-0middot77 vs 0middot71, 0middot70-0middot72). The combined model improved the prediction of digital ulcers and elevated systolic pulmonary artery pressure, but did poorly for cardiac involvement. Interpretation The autoantibody-only model outperforms cutaneous-only subsetting for risk stratifying people with systemic sclerosis in the EUSTAR cohort. Physicians should be aware of these findings at the time of decision making for patient management. Copyright (C) 2022 Elsevier Ltd. All rights reserved.

Published
2022

32. S11.3 Low-dose interleukin-2 therapy in active systemic lupus erythematosus (lupil-2): a multi-center, double-blind, randomized and placebo-controlled phase 2 trial

Author

Humrich, J, primary, Cacoub, P, additional, Rosenzwajg, M, additional, Pitoiset, F, additional, Pham, H, additional, Guidoux, J, additional, Leroux, D, additional, Vazquez, T, additional, Riemekasten, G, additional, Smolen, J, additional, Tsokos, G, additional, and Klatzmann, D, additional

Published
2022
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34. Cohort enrichment strategies for progressive interstitial lung disease in systemic sclerosis from EUSTAR

Author

Hoffmann-Vold, A M, primary, Brunborg, C, additional, Airò, P, additional, Ananyeva, L P, additional, Czirják, L, additional, Guiducci, S, additional, Hachulla, E, additional, Li, M, additional, Mihai, C, additional, Riemekasten, G, additional, Sfikakis, P P, additional, Valentini, G, additional, Kowal-Bielecka, O, additional, Allanore, Y, additional, and Distler, O, additional

Published
2022
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35. Progressive interstitial lung disease is frequent also in late disease stages in systemic sclerosis patients from EUSTAR

Author

Hoffmann-Vold, A M, primary, Brunborg, C, additional, Airò, P, additional, Ananyeva, L P, additional, Czirják, L, additional, Guiducci, S, additional, Hachulla, E, additional, Li, M, additional, Mihai, C, additional, Riemekasten, G, additional, Sfikakis, P P, additional, Valentini, G, additional, Kowal-Bielecka, O, additional, Allanore, Y, additional, and Distler, O, additional

Published
2022
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36. Risk stratification approaches perform differently in SSc-associated PAH in EUSTAR

Author

Bjørkekjær, H J, primary, Bruni, C, additional, Carreira, P E, additional, Airò, P, additional, Simeón-Aznar, C, additional, Truchetet, M, additional, Giollo, A, additional, Balbir-Gurman, A, additional, Martin, M, additional, Denton, C, additional, Gabrielli, A, additional, Fretheim, H, additional, Barua, I, additional, Bitter, H, additional, Midtvedt, Ø, additional, Broch, K, additional, Andreassen, A K, additional, Tanaka, Y, additional, Riemekasten, G, additional, Müller-Ladner, U, additional, Matucci Cerinic, M, additional, Castellví, I, additional, Siegert, E, additional, Hachulla, E, additional, Distler, O, additional, and Hoffmann-Vold, A, additional

Published
2022
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37. ACE inhibitors in SSc patients display a risk factor for scleroderma renal crisis—a EUSTAR analysis

Author

Bütikofer L, Varisco PA, Distler O, Kowal-Bielecka O, Allanore Y, Riemekasten G, Villiger PM, Adler S, Avouac J, Walker UA, Guiducci S, Airò P, Hachulla E, Valentini G, Carreira PE, Cozzi F, Gurman AB, Braun-Moscovici Y, Damjanov N, Ananieva LP, Scorza R, Jimenez S, Busquets J, Li M, Müller-Ladner U, Maurer B, Tyndall A, Lapadula G, Iannone F, Becvar R, Sierakowsky S, Bielecka OK, Cutolo M, Sulli A, Cuomo G, Vettori S, Rednic S, Nicoara I, Vlachoyiannopoulos P, Montecucco C, Caporali R, Novak S, Czirják L, Varju C, Chizzolini C, Kucharz EJ, Kotulska A, Kopec-Medrek M, Widuchowska M, Rozman B, Mallia C, Coleiro B, Gabrielli A, Farge D, Hij A, Hesselstrand R, Scheja A, Wollheim F, Martinovic D, Govoni M, Monaco AL, Hunzelmann N, Pellerito R, Bambara LM, Caramaschi P, Black C, Denton C, Henes J, Santamaria VO, Heitmann S, Krasowska D, Seidel M, Oleszowsky M, Burkhardt H, Himsel A, Salvador MJ, Stamenkovic B, Stankovic A, Tikly M, Starovoytova MN, Engelhart M, Strauss G, Nielsen H, Damgaard K, Szücs G, Mendoza AZ, de la Puente Buijdos C, Sifuentes Giraldo WA, Midtvedt Ø, Garen T, Launay D, Valesini G, Riccieri V, Ionescu RM, Opris D, Groseanu L, Wigley FM, Mihai CM, Cornateanu RS, Ionitescu R, Gherghe AM, Gorga M, Dobrota R, Bojinca M, Schett G, Distler JHW, Meroni P, Zeni S, Mouthon L, De Keyser F, Smith V, Cantatore FP, Corrado A, Ullman S, Iversen L, Pozzi MR, Eyerich K, Hein R, Knott E, Szechinski J, Wiland P, Szmyrka-Kaczmarek M, Sokolik R, Morgiel E, Krummel-Lorenz B, Saar P, Aringer M, Günther C, Anic B, Baresic M, Mayer M, Radominski SC, de Souza Müller C, Azevedo VF, Agachi S, Groppa L, Chiaburu L, Russu E, Zenone T, Stebbings S, Highton J, Stamp L, Chapman P, Baron M, O'Donnell J, Solanki K, Doube A, Veale D, O'Rourke M, Loyo E, Rosato E, Pisarri S, Tanaseanu CM, Popescu M, Dumitrascu A, Tiglea I, Chirieac R, Ancuta C, Furst DE, Kafaja S, de la Peña Lefebvre PG, Rubio SR, Exposito MV, Sibilia J, Chatelus E, Gottenberg JE, Chifflot H, Litinsky I, Venalis A, Butrimiene I, Venalis P, Rugiene R, Karpec D, Kerzberg E, Montoya F, Cosentino V, Castellvi I., Publica, Bütikofer, L, Varisco, Pa, Distler, O, Kowal-Bielecka, O, Allanore, Y, Riemekasten, G, Villiger, Pm, Adler, S, Avouac, J, Walker, Ua, Guiducci, S, Airò, P, Hachulla, E, Valentini, G, Carreira, Pe, Cozzi, F, Gurman, Ab, Braun-Moscovici, Y, Damjanov, N, Ananieva, Lp, Scorza, R, Jimenez, S, Busquets, J, Li, M, Müller-Ladner, U, Maurer, B, Tyndall, A, Lapadula, G, Iannone, F, Becvar, R, Sierakowsky, S, Bielecka, Ok, Cutolo, M, Sulli, A, Cuomo, G, Vettori, S, Rednic, S, Nicoara, I, Vlachoyiannopoulos, P, Montecucco, C, Caporali, R, Novak, S, Czirják, L, Varju, C, Chizzolini, C, Kucharz, Ej, Kotulska, A, Kopec-Medrek, M, Widuchowska, M, Rozman, B, Mallia, C, Coleiro, B, Gabrielli, A, Farge, D, Hij, A, Hesselstrand, R, Scheja, A, Wollheim, F, Martinovic, D, Govoni, M, Monaco, Al, Hunzelmann, N, Pellerito, R, Bambara, Lm, Caramaschi, P, Black, C, Denton, C, Henes, J, Santamaria, Vo, Heitmann, S, Krasowska, D, Seidel, M, Oleszowsky, M, Burkhardt, H, Himsel, A, Salvador, Mj, Stamenkovic, B, Stankovic, A, Tikly, M, Starovoytova, Mn, Engelhart, M, Strauss, G, Nielsen, H, Damgaard, K, Szücs, G, Mendoza, Az, de la Puente Buijdos, C, Sifuentes Giraldo, Wa, Midtvedt, Ø, Garen, T, Launay, D, Valesini, G, Riccieri, V, Ionescu, Rm, Opris, D, Groseanu, L, Wigley, Fm, Mihai, Cm, Cornateanu, R, Ionitescu, R, Gherghe, Am, Gorga, M, Dobrota, R, Bojinca, M, Schett, G, Distler, Jhw, Meroni, P, Zeni, S, Mouthon, L, De Keyser, F, Smith, V, Cantatore, Fp, Corrado, A, Ullman, S, Iversen, L, Pozzi, Mr, Eyerich, K, Hein, R, Knott, E, Szechinski, J, Wiland, P, Szmyrka-Kaczmarek, M, Sokolik, R, Morgiel, E, Krummel-Lorenz, B, Saar, P, Aringer, M, Günther, C, Anic, B, Baresic, M, Mayer, M, Radominski, Sc, de Souza Müller, C, Azevedo, Vf, Agachi, S, Groppa, L, Chiaburu, L, Russu, E, Zenone, T, Stebbings, S, Highton, J, Stamp, L, Chapman, P, Baron, M, O'Donnell, J, Solanki, K, Doube, A, Veale, D, O'Rourke, M, Loyo, E, Rosato, E, Pisarri, S, Tanaseanu, Cm, Popescu, M, Dumitrascu, A, Tiglea, I, Chirieac, R, Ancuta, C, Furst, De, Kafaja, S, de la Peña Lefebvre, Pg, Rubio, Sr, Exposito, Mv, Sibilia, J, Chatelus, E, Gottenberg, Je, Chifflot, H, Litinsky, I, Venalis, A, Butrimiene, I, Venalis, P, Rugiene, R, Karpec, D, Kerzberg, E, Montoya, F, Cosentino, V, and Castellvi, I.

Subjects
INVOLVEMENT, Male, Hypertension, Renal, ACE inhibitors, lcsh:Diseases of the musculoskeletal system, Scleroderma Renal Crisis, MULTICENTER, Angiotensin-Converting Enzyme Inhibitors, Scleroderma, Scleroderma renal crisis, 0302 clinical medicine, Risk Factors, Medicine and Health Sciences, Medicine, 030212 general & internal medicine, Prospective Studies, 610 Medicine & health, Renal, Antihypertensive drugs, Outcome, antihypertensive drugs, arterial hypertension, outcome, scleroderma renal crisis, Incidence (epidemiology), Incidence, Hazard ratio, Acute Kidney Injury, Middle Aged, Europe, Treatment Outcome, Population Surveillance, Cohort, Hypertension, Female, 360 Social problems & social services, Proto-oncogene tyrosine-protein kinase Src, Research Article, Arterial hypertension, medicine.medical_specialty, 03 medical and health sciences, ENDOTHELIN-1, Internal medicine, Humans, Risk factor, Aged, 030203 arthritis & rheumatology, Scleroderma, Systemic, business.industry, SYSTEMIC-SCLEROSIS, Systemic, medicine.disease, Concomitant, lcsh:RC925-935, business
Abstract

Objectives To investigate the effect of ACE inhibitors (ACEi) on the incidence of scleroderma renal crisis (SRC) when given prior to SRC in the prospectively collected cohort from the European Scleroderma Trial and Research Group (EUSTAR). Methods SSc patients without prior SRC and at least one follow-up visit were included and analyzed regarding SRC, arterial hypertension, and medication focusing on antihypertensive medication and glucocorticoids (GC). Results Out of 14,524 patients in the database, we identified 7648 patients with at least one follow-up. In 27,450 person-years (py), 102 patients developed SRC representing an incidence of 3.72 (3.06–4.51) per 1000 py. In a multivariable time-to-event analysis adjusted for age, sex, disease severity, and onset, 88 of 6521 patients developed SRC. The use of ACEi displayed an increased risk for the development of SRC with a hazard ratio (HR) of 2.55 (95% confidence interval (CI) 1.65–3.95). Adjusting for arterial hypertension resulted in a HR of 2.04 (95%CI 1.29–3.24). There was no evidence for an interaction of ACEi and arterial hypertension (HR 0.83, 95%CI 0.32–2.13, p = 0.69). Calcium channel blockers (CCB), angiotensin receptor blockers (ARB), endothelin receptor antagonists, and GC—mostly in daily dosages below 15 mg of prednisolone—did not influence the hazard for SRC. Conclusions ACEi in SSc patients with concomitant arterial hypertension display an independent risk factor for the development of SRC but are still first choice in SRC treatment. ARBs might be a safe alternative, yet the overall safety of alternative antihypertensive drugs in SSc patients needs to be further studied.

Published
2020

38. Therapy satisfaction and health literacy are key factors to improve medication adherence in systemic sclerosis.

Author

Matrisch, L, Graßhoff, H, Müller, A, Schinke, S, and Riemekasten, G

Subjects
PATIENT compliance, HEALTH literacy, SYSTEMIC scleroderma, SATISFACTION, EMOTIONAL state, WEIGHT loss
Abstract

Although medication adherence (MA) contributes to therapeutic outcome in systemic sclerosis (SSc), research data are scarce. Factors influencing MA in SSc are hardly known. We conducted a monocentric, cross-sectional study on 85 patients with SSc at the University of Lübeck, Germany, using the Compliance Questionnaire of Rheumatology as the main measurement tool of MA. We also used the Scleroderma Health Assessment Questionnaire, Illness Perception Questionnaire – Revised, Health Literacy Questionnaire, Lübeck Medication Satisfaction Questionnaire (a novel instrument created for this study), and patients' demographic and clinical data, to find factors contributing to MA. Good MA was seen in 51.8% of patients. MA was positively associated with therapy satisfaction (p < 0.001), modified Rodnan Skin Score (p = 0.032), age (p = 0.025), intake of micronutrients (p = 0.033), number of prescribed drugs (p = 0.014), and some dimensions of health literacy. Negative associations were found for patients with weight loss attributed to SSc (p = 0.009) and the perception that the disease is caused by the patient's personality (p = 0.011) or emotional state (p = 0.037). Although most SSc patients display good MA, non-adherence remains a major problem. Patients should be assessed for non-adherence. The factors affecting MA identified herein could help to improve therapeutic outcomes. [ABSTRACT FROM AUTHOR]

Published
2023
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40. Europäische Versorgungsstandards für Menschen mit rheumatoider Arthritis: Übersetzung und Kommentierung der von der EULAR unterstützten Vorschläge des eumusc.net durch eine Task Force von DGRh und VRA mit Unterstützung der Deutschen Rheuma-Liga

Author

Braun, J., Krause, A., Aringer, M., Burmester, G., Bessler, F., Engel, J.-M., Faubel, U., Fischer-Betz, R., Genth, E., Gromnica-Ihle, E., Hellmich, B., Kötter, I., Krüger, K., Lakomek, J., Lorenz, H.-M., Manger, B., Märker-Hermann, E., Minden, K., Müller-Ladner, U., Rautenstrauch, J., Rehart, S., Riemekasten, G., Rudwaleit, M., Rüther, W., Schett, G., Schuch, F., Schulze-Koops, H., Specker, C., Wassenberg, S., Wiek, D., Zink, A., and Schneider, M.

Published
2016
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42. POS0140 PREDICTING OUTCOMES IN SYSTEMIC SCLEROSIS: STRATIFICATION BY AUTO-ANTIBODIES OUTPERFORMS CUTANEOUS SUBSETTING IN THE EUSTAR COHORT

Author

Elhai, M., primary, Boubaya, M., additional, Sritharan, N., additional, Balbir-Gurman, A., additional, Siegert, E., additional, Hachulla, E., additional, De Vries-Bouwstra, J., additional, Riemekasten, G., additional, Distler, J. H. W., additional, Veale, D., additional, Rosato, E., additional, Del Galdo, F., additional, Mendoza, F. A., additional, Furst, D., additional, De la Puente Bujidos, C., additional, Hoffmann-Vold, A. M., additional, Gabrielli, A., additional, Distler, O., additional, Bloch-Queyrat, C., additional, and Allanore, Y., additional

Published
2022
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43. POS0914 LATE SKIN FIBROSIS IN SYSTEMIC SCLEROSIS: A STUDY FROM THE EUSTAR COHORT

Author

Hughes, M., primary, Huang, S., additional, Alegre Sancho, J. J., additional, Carreira, P., additional, Engelhart, M., additional, Hachulla, E., additional, Henes, J., additional, Kerzberg, E., additional, Pozzi, M. R., additional, Riemekasten, G., additional, Smith, V., additional, Szucs, G., additional, Vanthuyne, M., additional, Zanatta, E., additional, Distler, O., additional, Gabrielli, A., additional, Hoffmann-Vold, A. M., additional, Steen, V., additional, and Khanna, D., additional

Published
2022
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47. POS0387 RISK STRATIFICATION APPROACHES PERFORM DIFFERENTLY IN SSc-ASSOCIATED PAH IN EUSTAR

Author

Bjørkekjær, H. J., primary, Bruni, C., additional, Carreira, P., additional, Airò, P., additional, Simeón-Aznar, C. P., additional, Truchetet, M. E., additional, Giollo, A., additional, Balbir-Gurman, A., additional, Martin, M., additional, Denton, C. P., additional, Gabrielli, A., additional, Fretheim, H., additional, Barua, I., additional, Bitter, H., additional, Midtvedt, Ø., additional, Broch, K., additional, Andreassen, A., additional, Tanaka, Y., additional, Riemekasten, G., additional, Müller-Ladner, U., additional, Matucci-Cerinic, M., additional, Castellví, I., additional, Siegert, E., additional, Hachulla, E., additional, Distler, O., additional, and Hoffmann-Vold, A. M., additional

Published
2022
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