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1. 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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2. 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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3. 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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4. 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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6. 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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8. 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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10. 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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11. POS1243 THE ROLE OF TAPSE/SPAP RATIO IN PREDICTING PULMONARY HYPERTENSION AND MORTALITY IN THE SYSTEMIC SCLEROSIS EUSTAR COHORT

Author

Colalillo, A., primary, Hoffmann-Vold, A. M., additional, Pellicano, C., additional, Romaniello, A., additional, Gabrielli, A., additional, Hachulla, E., additional, Smith, V., additional, Simeon Aznar, C. P., additional, Castellví, I., additional, Airò, P., additional, Truchetet, M. E., additional, Siegert, E., additional, Distler, O., additional, and Rosato, E., additional

Published
2023
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12. 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

14. 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

15. Sex-specific risk of anti-topoisomerase antibodies on mortality and disease severity in systemic sclerosis: 10-year analysis of the Leiden CCISS and EUSTAR cohorts

Author

Liem, S.I.E., Boonstra, M., Cessie, S. le, Riccardi, A., Airo, P., Distler, O., Matucci-Cerinic, M., Caimmi, C., Siegert, E., Allanore, Y., Huizinga, T.W.J., Toes, R.E.M., Scherer, H.U., Vries-Bouwstra, J.K. de, and EUSTAR Collaborators

Abstract

Background We aimed to evaluate sex-specific risk of anti-topoisomerase I antibodies (ATA) on mortality, diffuse cutaneous systemic sclerosis, interstitial lung disease, and pulmonary hypertension in two cohorts of people with systemic sclerosis.Methods This study was a 10-year analysis of the prospective Leiden Combined Care in Systemic Sderosis (CCISS) cohort in the Netherlands and the international European Scleroderma Trials and Research (EUSTAR) cohort. We included participants with systemic sderosis according to the 2013 American College of Rheumatology-European League Against Rheumatism (ACR-EULAR) classification criteria; available autoantibody status; available skin subtyping; at least one available radiographic assessment of interstitial lung disease; and with a known date of disease onset. People with systemic sclerosis were categorised in six risk groups by sex and autoantibody status (anti-centromere antibody [ACA]-positive female, ACA-positive male, ACA and ATA-negative female, ACA and ATA-negative male, ATA-positive female, and ATA-positive male). We constructed Kaplan-Meier curves and Cox proportional hazard models, accounting for left-truncated survival to prevent bias because the date of disease onset (first non-Raynaud's symptom) preceded the date of cohort entry for all patients. The primary outcome was all-cause mortality and the secondary outcomes were diffuse cutaneous systemic sclerosis, interstitial lung disease, and pulmonary hypertension.Findings 445 (63%) of 708 participants between April 1,2009, and Jan 1,2022, in CCISS (101 [23%] male and 344[77%] female) and 4263 (50%) of 8590 between June 1, 2004, and March 28,2018, in EUSTAR (783[18%] male and 3480 [82%] female) were eligible for this study. In both cohorts, ATA expression occurred significantly more often in males than in females (39 [39%] of 101 males vs 67 [19%] of 344 females in CCISS; p

Published
2022

16. 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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17. Late Skin Fibrosis in Systemic Sclerosis: A Study from the EUSTAR Cohort

Author

Michael Hughes 1 2, Suiyuan Huang 3 4, Juan Jose Alegre-Sancho 5, Patricia E Carreira 6, Merete Engelhart 7, Eric Hachulla 8, Joerg Henes 9, Eduardo Kerzberg 10, Maria Rosa Pozzi 11, Gabriela Riemekasten 12, Vanessa Smith 13, Gabriella Szücs 14, Marie Vanthuyne 15, Elisabetta Zanatta 16, Oliver Distler 17, Armando G Gabrielli 18, Anna-Maria Hoffmann-Vold 19, Virginia D Steen 20, Dinesh Khanna 3 21, EUSTAR Airò P, Allanore A, Ananieva Lp, Anic B, Balbir-Gurman A, Becvar R, Benvenuti F, Cantatore F P, Chung L S, Cuomo G, Cutolo M, Czirják L, Damjanov N, de Vries-Bouwstra J, Del Galdo F, Distler J, Eyerich K, Farge D, Foti R, Gheorghiu A M, Giollo A, Heitmann S, Herrick A, Hesselstrand R, Hsu I M, Hunzelmann N, Iannone F, Iudici M, Ionescuc M R, Ingegnoli F, Jose J, Joven B E, Kerzberg E, Kucharz E J, Kuwana M, Langhe E D, Launay D, Lefebvre P, Litinsky I, García de la Peña Lefebvre P, González-Martín J J, Li M, Loyo E, Martin T, Matucci-Cerinic M, Maurer B, Moroncini G, Mouthon L, Müller Cs, Müller-Ladner U, Novak S, Pastor P, Pecher A-C, Pellerito R, Pozzi M R, Oksel F, Rednic S, Rezus E, Riccieri V, Rosato E, Saketkoo L A, Salvador M J, Schmeiser T, Selmi C F, Sibilia J, Siegert E, Solanki K, Sommerlatte S, Spertini F, Stamenkovic B, Stamp L, Tanaseanu C-M, Tikly M, Tineo C, Ullman S, Üprus M, Vanthuyne M, Veale D, Walker U, Wiland P, Yargucu F, Yavuz S, University of Zurich, Michael Hughes, 1 2, Suiyuan Huang, 3 4, Juan Jose Alegre-Sancho, 5, Patricia, E Carreira 6, Merete Engelhart, 7, Eric Hachulla, 8, Joerg Henes, 9, Eduardo Kerzberg, 10, Maria Rosa Pozzi, 11, Gabriela Riemekasten, 12, Vanessa Smith, 13, Gabriella Szücs, 14, Marie Vanthuyne, 15, Elisabetta Zanatta, 16, Oliver Distler, 17, Armando, G Gabrielli 18, Anna-Maria Hoffmann-Vold, 19, Virginia, D Steen 20, Dinesh Khanna, 3 21, EUSTAR Airò, P, Allanore, A, Ananieva, Lp, Anic, B, Balbir-Gurman, A, Becvar, R, Benvenuti, F, Cantatore, F P, Chung, L S, Cuomo, G, Cutolo, M, Czirják, L, Damjanov, N, de Vries-Bouwstra, J, Del Galdo, F, Distler, J, Eyerich, K, Farge, D, Foti, R, Gheorghiu, A M, Giollo, A, Heitmann, S, Herrick, A, Hesselstrand, R, Hsu, I M, Hunzelmann, N, Iannone, F, Iudici, M, Ionescuc, M R, Ingegnoli, F, Jose, J, Joven, B E, Kerzberg, E, Kucharz, E J, Kuwana, M, Langhe, E D, Launay, D, Lefebvre, P, Litinsky, I, García de la Peña Lefebvre, P, González-Martín, J J, Li, M, Loyo, E, Martin, T, Matucci-Cerinic, M, Maurer, B, Moroncini, G, Mouthon, L, Müller, C, Müller-Ladner, U, Novak, S, Pastor, P, Pecher, A-C, Pellerito, R, Pozzi, M R, Oksel, F, Rednic, S, Rezus, E, Riccieri, V, Rosato, E, Saketkoo, L A, Salvador, M J, Schmeiser, T, Selmi, C F, Sibilia, J, Siegert, E, Solanki, K, Sommerlatte, S, Spertini, F, Stamenkovic, B, Stamp, L, Tanaseanu, C-M, Tikly, M, Tineo, C, Ullman, S, Üprus, M, Vanthuyne, M, Veale, D, Walker, U, Wiland, P, Yargucu, F, and Yavuz, S

Subjects
Rheumatology, Cohort enrichment, 10051 Rheumatology Clinic and Institute of Physical Medicine, Late disease, Systemic sclerosis, Pharmacology (medical), 610 Medicine & health, Fibrosis, Clinical trial design, Scleroderma, Skin
Abstract

Objectives The early trajectory of skin fibrosis provides insights into the disease course of systemic sclerosis (SSc) including mortality; however, little is known about late skin fibrosis. The aims of our study were to ascertain the prevalence and characteristics of late skin fibrosis in SSc. Methods We developed and tested three conceptual scenarios of late (>5 years after first non-RP feature) skin fibrosis including new worsening of skin disease, and failure to improve after worsening within 5-year window. We defined skin worsening as change in modified Rodnan skin score (mRSS) ≥5 units or ≥25%. Using strict inclusion criteria including complete mRSS, we identified 1,043 (out of 19 115) patients within the EUSTAR database for our analysis. We further restricted analysis within 887 (out of 1043) patients who had lcSSc or dcSSc at baseline. Results One-fifth of patients among the whole cohort (n = 208/1043, 19.9%) experienced mRSS worsening, including in patients with lcSSc or dcSSc at baseline (n = 193/887, 21.8%). This was largely due to new skin worsening after the 5-year window or failure to improve with worsening within the 5-year window. Patients with lower baseline mRSS and lcSSc were more likely to develop late skin fibrosis. Anti-Scl-70 was associated with progression from baseline lcSSc to dcSSc, and anticentromere was protective. Conclusions Late skin fibrosis is not uncommon in SSc. We have identified different patterns relevant to clinical practice and trial design. Late skin fibrosis is a neglected manifestation of SSc and warrants further investigation including to determine clinical outcomes and optimal therapeutic strategy.

Published
2022

19. 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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20. 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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21. POS0874 GLUCOCORTICOIDS PRESCRIBING PRACTICES IN SYSTEMIC SCLEROSIS: AN ANALYSIS OF THE EUSTAR DATABASE.

Author

Iudici, M., primary, Mongin, D., additional, Siegert, E., additional, Carreira, P., additional, Distler, J. H. W., additional, Henes, J., additional, Zanatta, E., additional, Hachulla, E., additional, De Luca, G., additional, Souza Muller, C., additional, Salvador, M. J., additional, Tandaipan, J. L., additional, Valdetaro Bianchi, B., additional, De Santis, M., additional, Hoffmann-Vold, A. M., additional, Gabrielli, A., additional, Distler, O., additional, and Courvoisier, D., additional

Published
2022
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22. 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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23. 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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24. 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
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26. Therapy of Acute Myelogenous Leukemia in Childhood — Results of the Multicenter Trial AML II/87 in East Germany

Author

Hermanni, J., Fuchs, D., Häfer, R., Müller, A., Palme, H., Prager, J., Dörffel, W., Domula, M., Eggers, G., Exadaktylos, P., Hilgenfeld, E., Krause, I., Kunert, W., Mittler, U., Möbius, D., Reddemann, H., Scharfe, V., Siegert, E., Weinmann, G., Zintl, F., Büchner, T., editor, Hiddemann, W., editor, Wörmann, B., editor, Schellong, G., editor, and Ritter, J., editor

Published
1994
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28. Outcomes of limited cutaneous systemic sclerosis patients: Results on more than 12,000 patients from the EUSTAR database

Author

Frantz C., Huscher D., Avouac J., Hachulla E., Balbir-Gurman A., Riemekasten G., Siegert E., Lazzaroni M. -G., Carreira P. E., Vettori S., Zanatta E., Ullman S., Czirjak L., Kowal-Bielecka O., Distler O., Matucci-Cerinic M., Allanore Y, Cuomo Giovanna, University of Zurich, Allanore, Yannick, Frantz, C., Huscher, D., Avouac, J., Hachulla, E., Balbir-Gurman, A., Riemekasten, G., Siegert, E., Lazzaroni, M. -G., Carreira, P. E., Vettori, S., Zanatta, E., Ullman, S., Czirjak, L., Kowal-Bielecka, O., Distler, O., Matucci-Cerinic, M., Allanore, Y, and Cuomo, Giovanna

Subjects
0301 basic medicine, Skin score, Limited cutaneous systemic sclerosis, Male, Databases, Factual, Fibrosi, Limited cutaneous systemic sclerosi, Immunology, Digital ulcer, 610 Medicine & health, Interstitial lung disease, Disease, Outcomes, computer.software_genre, Scleroderma, 03 medical and health sciences, FEV1/FVC ratio, Databases, 0302 clinical medicine, Scleroderma, Limited, medicine, Immunology and Allergy, Humans, In patient, Limited, Lung, Factual, Skin, 030203 arthritis & rheumatology, Peripheral Vascular Diseases, 2403 Immunology, integumentary system, Database, business.industry, 10051 Rheumatology Clinic and Institute of Physical Medicine, Digital ulcers, Middle Aged, medicine.disease, Fibrosis, Female, Clinical trial, 030104 developmental biology, 2723 Immunology and Allergy, business, computer
Abstract

Objectives: Limited cutaneous systemic sclerosis (LcSSc) is the most common subset of SSc but it has been overlooked in the past years. At a time at which clinical trials focus on diffuse cutaneous SSc (DcSSc) we aimed at clarifying the outcomes of LcSSc and at evaluating whether potential drug positioned in DcSSc may also be used in LcSSc. Methods: The EUSTAR database was used to investigate skin, lung and peripheral vasculopathy outcomes in LcSSc. Worsening of skin fibrosis, ILD and peripheral vasculopathy were defined by an increase in modified Rodnan skin score (mRSS) > 3.5 points, a decrease of FVC > 10% in patients with ILD at baseline, and by the development of new digital ulcers (DU) in patients without DU at baseline. Results: 8013 LcSSc and 4786 DcSSc patients were included. In contrast to DcSSc, skin disease was remarkably stable in the majority of LcSSc patients with >80% having a change lower than ±4 units of mRSS at 12, 24 and 36 months follow-up. Conversely, FVC changes over time were very similar between LcSSc and DcSSc. Regarding DU, numbers of patients with new DU over time seemed to be almost similar between the two subsets. Conclusions: LcSSc patients have a low mRSS at baseline with marginal changes with time. Conversely, SSc-ILD can be as progressive as in DcSSc supporting the inclusion of LcSSc patients in SSc-ILD trials and suggesting potential benefit of any anti-ILD drugs. Similarly, although slightly less common, DU should receive the same attention in the two subsets.

Published
2020

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

Author

Bruni, C., Tofani, L., Fretheim, H., Weber, Y., Hachulla, E., Carreira, P., Giuggioli, D., Airò, P., Siegert, E., Müller-Ladner, U., Matucci-Cerinic, M., Riemekasten, G., Aznar, C. P. Simeon, De Vries-Bouwstra, J., Saketkoo, L. A., Distler, J., Balbir-Gurman, A., Castellví, I., Zanatta, E., and Smith, V.

Published
2023
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31. EFFECTIVENESS AND SAFETY OF TOCILIZUMAB IN PATIENTS WITH SYSTEMIC SCLEROSIS: A PROPENSITY SCORE CONTROL MATCHED OBSERVATIONAL STUDY OF THE EUSTAR COHORT

Author

Kuster S, Jordan S, Elhai M, Held U, Steigmiller K, Bruni C, Iannone F, Vettori S, Siegert E, Rednic S, Codullo V, Airo P, Braun-Moscovici Y, Hunzelmann N, Salvador M, Riccieri V, Gheorghiu A, Sancho J, Romanowska-Prochnicka K, Castellvi I, Koetter I, Truchetet M, Lopez-Longo F, Novikov P, Giollo A, Shirai Y, Belloli L, Zanatta E, Hachulla E, Smith V, Denton C, Ionescu R, Schmeiser T, Distler J, Gabrielli A, Hoffmann-Vold A, Kuwana M, Allanore Y, Distler O, and EUSTAR

Published
2021

32. EFFECTIVENESS AND SAFETY OF TOCILIZUMAB IN PATIENTS WITH SYSTEMIC SCLEROSIS: A PROPENSITY SCORE CONTROL MATCHED OBSERVATIONAL STUDY OF THE EUSTAR COHORT

Author

Kuster, S., Jordan, S., Elhai, M. D., Held, U., Steigmiller, K., Bruni, C., Iannone, F., Vettori, S., Siegert, E., Rednic, S., Codullo, V., Airo, P., Braun-Moscovici, Y., Hunzelmann, N., Salvador, M. J., Riccieri, V., Gheorghiu, A. M., Alegre Sancho, J. J., Romanowska-Prochnicka, K., Castellvi, I., Koetter, I., Truchetet, M. E., Lopez-Longo, F. J., Novikov, P., Giollo, A., Shirai, Y., Belloli, L., Zanatta, E., Hachulla, E., Smith, V., Denton, C., Ionescu, R., Schmeiser, T., Distler, J. H. W., Gabrielli, A., Hoffmann-Vold, A. M., Kuwana, M., Allanore, Y., Distler, O., Kuster, S., Jordan, S., Elhai, M. D., Held, U., Steigmiller, K., Bruni, C., Iannone, F., Vettori, S., Siegert, E., Rednic, S., Codullo, V., Airo, P., Braun-Moscovici, Y., Hunzelmann, N., Salvador, M. J., Riccieri, V., Gheorghiu, A. M., Alegre Sancho, J. J., Romanowska-Prochnicka, K., Castellvi, I., Koetter, I., Truchetet, M. E., Lopez-Longo, F. J., Novikov, P., Giollo, A., Shirai, Y., Belloli, L., Zanatta, E., Hachulla, E., Smith, V., Denton, C., Ionescu, R., Schmeiser, T., Distler, J. H. W., Gabrielli, A., Hoffmann-Vold, A. M., Kuwana, M., Allanore, Y., and Distler, O.

Published
2021

33. 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

34. LONG-TERM OUTCOME OF SSC ASSOCIATED ILD: IMPROVED SURVIVAL IN PPI TREATED PATIENTS

Author

Kreuter, M., Bonella, F., Kathrin, K., Henes, J., Siegert, E., Riemekasten, G., Blank, N., Pfeiffer, C., Mueller-Ladner, U., Kreuter, A., Korsten, P., Juche, A., Schmalzing, M., Worm, M., Jandova, I., Susok, L., Schmeiser, T., Guenther, C., Keyszer, G., Ehrchen, J., Ramming, A., Koetter, I., Lorenz, H. M., Moinzadeh, P., Hunzelmann, N., Kreuter, M., Bonella, F., Kathrin, K., Henes, J., Siegert, E., Riemekasten, G., Blank, N., Pfeiffer, C., Mueller-Ladner, U., Kreuter, A., Korsten, P., Juche, A., Schmalzing, M., Worm, M., Jandova, I., Susok, L., Schmeiser, T., Guenther, C., Keyszer, G., Ehrchen, J., Ramming, A., Koetter, I., Lorenz, H. M., Moinzadeh, P., and Hunzelmann, N.

Published
2021

35. POS0861 EFFECTIVENESS AND SAFETY OF TOCILIZUMAB IN PATIENTS WITH SYSTEMIC SCLEROSIS: A PROPENSITY SCORE CONTROL MATCHED OBSERVATIONAL STUDY OF THE EUSTAR COHORT

Author

Kuster, S., primary, Jordan, S., additional, Elhai, M. D., additional, Held, U., additional, Steigmiller, K., additional, Bruni, C., additional, Iannone, F., additional, Vettori, S., additional, Siegert, E., additional, Rednic, S., additional, Codullo, V., additional, Airò, P., additional, Braun-Moscovici, Y., additional, Hunzelmann, N., additional, Salvador, M. J., additional, Riccieri, V., additional, Gheorghiu, A. M., additional, Alegre Sancho, J. J., additional, Romanowska-Prochnicka, K., additional, Castellví, I., additional, Koetter, I., additional, Truchetet, M. E., additional, López-Longo, F. J., additional, Novikov, P., additional, Giollo, A., additional, Shirai, Y., additional, Belloli, L., additional, Zanatta, E., additional, Hachulla, E., additional, Smith, V., additional, Denton, C., additional, Ionescu, R., additional, Schmeiser, T., additional, Distler, J. H. W., additional, Gabrielli, A., additional, Hoffmann-Vold, A. M., additional, Kuwana, M., additional, Allanore, Y., additional, and Distler, O., additional

Published
2021
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36. POS0834 LONG-TERM OUTCOME OF SSC ASSOCIATED ILD: IMPROVED SURVIVAL IN PPI TREATED PATIENTS

Author

Kreuter, M., primary, Bonella, F., additional, Kathrin, K., additional, Henes, J., additional, Siegert, E., additional, Riemekasten, G., additional, Blank, N., additional, Pfeiffer, C., additional, Müller-Ladner, U., additional, Kreuter, A., additional, Korsten, P., additional, Juche, A., additional, Schmalzing, M., additional, Worm, M., additional, Jandova, I., additional, Susok, L., additional, Schmeiser, T., additional, Guenther, C., additional, Keyszer, G., additional, Ehrchen, J., additional, Ramming, A., additional, Kötter, I., additional, Lorenz, H. M., additional, Moinzadeh, P., additional, and Hunzelmann, N., additional

Published
2021
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37. POS0321 USE OF HYDROXYCHLOROQUINE AND SYSTEMIC SCLEROSIS: RESULTS FROM A PROSPECTIVE OBSERVATIONAL STUDY ON THE EUSTAR COHORT

Author

Bellando Randone, S., primary, Wilhalme, H., additional, Bruni, C., additional, Siegert, E., additional, Airò, P., additional, Irace, R., additional, Distler, O., additional, Doria, A., additional, Ananieva, L. P., additional, Czirják, L., additional, Denton, C., additional, Allanore, Y., additional, Riccieri, V., additional, Vacca, A., additional, Foeldvari, I., additional, Hoffmann-Vold, A. M., additional, Gabrielli, A., additional, Matucci-Cerinic, M., additional, and Furst, D., additional

Published
2021
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38. POS0317 THE PERFORMANCE OF DIFFUSING CAPACITY FOR MONOXIDE CARBON (DLCO) AND FORCED VITAL CAPACITY (FVC) IN PREDICTING THE ONSET OF SYSTEMIC SCLEROSIS (SSc)-INTERSTITIAL LUNG DISEASE (ILD) IN THE EUROPEAN SCLERODERMA TRIALS AND RESEARCH (EUSTAR) DATABASE

Author

Lepri, G., primary, Bruni, C., additional, Tofani, L., additional, Moggi Pignone, A., additional, Orlandi, M., additional, Sara, T., additional, Hughes, M., additional, Del Galdo, F., additional, Irace, R., additional, Distler, O., additional, Riccieri, V., additional, Allanore, Y., additional, Gheorghiu, A. M., additional, Siegert, E., additional, De Vries-Bouwstra, J., additional, Hachulla, E., additional, Tikly, M., additional, Damjanov, N., additional, Spertini, F., additional, Mouthon, L., additional, Hoffmann-Vold, A. M., additional, Gabrielli, A., additional, Guiducci, S., additional, Matucci-Cerinic, M., additional, Furst, D., additional, and Bellando Randone, S., additional

Published
2021
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39. 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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42. Health Assessment Questionnaire-Disability Index (HAQ-DI) use in modelling disease progression in diffuse cutaneous systemic sclerosis: an analysis from the EUSTAR database

Author

Allanore Y., Bozzi S., Terlinden A., Huscher D., Amand C., Soubrane C., Siegert E., Czirjak L., Carreira P. E., Hachulla E., Zanatta E., Li M., Airo P., Mendoza F. A., Rosato E., Distler O. Svetlana Agachi, Walid Ahmed Abdel Atty Mohamed, Paolo Airò, Sabine Adler, Juan Jose Alegre-Sancho, Yannick Allanore, Lidia P. Ananieva, Codrina Ancuta, Branimir Anic, Fabiola Atzeni, Gianluigi Bajocchi, Alexandra Balbir-Gurman, Marko Baresic, Radim Becvar, Laura Belloli, Vera Bernardino, Washington Bianchi, Breno Valdetaro Bianchi, Otylia Kowal Bielecka, Francesco Paolo Cantatore, Patricia E. Carreira, Ivan Castellví, Marco Matucci Cerinic, Carlo Chizzolini, Lorinda S. Chung, Bernard Coleiro, Maura Couto, Mary Ellen Csuka, Giovanna Cuomo, Maurizio Cutolo, László Czirják, Lorenzo Dagna, Nemanja Damjanov, Ellen De Langhe, Francesco Del Galdo, Carlos de la Puente, Christopher Denton, Y., Allanore, S., Bozzi, A., Terlinden, D., Huscher, C., Amand, C., Soubrane, E., Siegert, L., Czirjak, P. E., Carreira, E., Hachulla, E., Zanatta, M., Li, P., Airo, F. A., Mendoza, E., Rosato, Svetlana Agachi, Distler O., Ahmed Abdel Atty Mohamed, Walid, Airò, Paolo, Adler, Sabine, Jose Alegre-Sancho, Juan, Allanore, Yannick, Ananieva, Lidia P., Ancuta, Codrina, Anic, Branimir, Atzeni, Fabiola, Bajocchi, Gianluigi, Balbir-Gurman, Alexandra, Baresic, Marko, Becvar, Radim, Belloli, Laura, Bernardino, Vera, Bianchi, Washington, Valdetaro Bianchi, Breno, Kowal Bielecka, Otylia, Paolo Cantatore, Francesco, Carreira, Patricia E., Castellví, Ivan, Matucci Cerinic, Marco, Chizzolini, Carlo, Chung, Lorinda S., Coleiro, Bernard, Couto, Maura, Ellen Csuka, Mary, Cuomo, Giovanna, Cutolo, Maurizio, Czirják, László, Dagna, Lorenzo, Damjanov, Nemanja, De Langhe, Ellen, Del Galdo, Francesco, de la Puente, Carlo, Denton, Christopher, Publica, and University of Zurich

Subjects
0301 basic medicine, Longitudinal study, lcsh:Diseases of the musculoskeletal system, medicine.medical_treatment, 2745 Rheumatology, HAQ-DI score, computer.software_genre, Severity of Illness Index, 0302 clinical medicine, Quality of life, Surveys and Questionnaires, Medicine and Health Sciences, Longitudinal Studies, 610 Medicine & health, skin and connective tissue diseases, Database, Mortality rate, 10051 Rheumatology Clinic and Institute of Physical Medicine, EUSTAR registry, Diffuse cutaneous systemic sclerosis, Health Assessment Questionnaire-Disability Index, HAQ-DI score, 2723 Immunology and Allergy, Disease Progression, Hemodialysis, ARTHRITIS, CLINICAL-TRIALS, Research Article, musculoskeletal diseases, medicine.medical_specialty, Diffuse cutaneous systemic sclerosis, PREDICTING MORTALITY, EUSTAR registry, CLASSIFICATION, 03 medical and health sciences, Internal medicine, SCORE, medicine, Humans, 030203 arthritis & rheumatology, 2403 Immunology, Scleroderma, Systemic, business.industry, Proportional hazards model, Diffuse cutaneous systemic sclerosi, Health Assessment Questionnaire-Disability Index, Rheumatology, Clinical trial, 030104 developmental biology, Scleroderma, Diffuse, Quality of Life, FUNCTIONAL DISABILITY, Observational study, lcsh:RC925-935, Mitoxantrone, business, computer
Abstract

BackgroundPatients with diffuse cutaneous systemic sclerosis (dcSSc) have a poor prognosis. The importance of monitoring subjective measures of functioning and disability, such as the Health Assessment Questionnaire-Disability Index (HAQ-DI), is important as dcSSc is rated by patients as worse than diabetes or hemodialysis for quality of life impairment. This European Scleroderma Trials and Research (EUSTAR) database analysis was undertaken to examine the importance of impaired functionality in dcSSc prognosis. The primary objectives were to identify predictors of death and HAQ-DI score progression over 1 year. HAQ-DI score, major advanced organ involvement, and death rate were also used to develop a comprehensive model to predict lifetime dcSSc progression.MethodsThis was an observational, longitudinal study in patients with dcSSc registered in EUSTAR. Death and HAQ-DI scores were, respectively, analyzed by Cox regression and linear regression analyses in relation to baseline covariates. A microsimulation Markov model was developed to estimate/predict natural progression of dcSSc over a patient’s lifetime.ResultsThe analysis included dcSSc patients with (N = 690) and without (N = 4132) HAQ-DI score assessments from the EUSTAR database. Baseline HAQ-DI score, corticosteroid treatment, and major advanced organ involvement were predictive of death on multivariable analysis; a 1-point increase in baseline HAQ-DI score multiplied the risk of death by 2.7 (p p p ConclusionsHAQ-DI score and major advanced organ involvement were comparable predictors of mortality risk in dcSSc. Baseline mRSS and baseline HAQ-DI score were predictive of HAQ-DI score progression at 1 year, indicating a correlation between these endpoints in monitoring disease progression. It is hoped that this EUSTAR analysis may change physician perception about the importance of the HAQ-DI score in dcSSc.

Published
2020
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43. Long term outcomes of immunmodulatory drugs in SSc-ILD - data rom the German SSc network

Author

Kreuter, M., Bonella, F., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Guenther, C., Susok, L., Zeidler, G., Koetter, I, Mueller-Ladner, U., Krieg, T., Juche, A., Schmeiser, T., Riemekasten, G., Aberer, E., Gaebelein-Wissing, N., Distler, J. H. W., Sardy, M., Pfeiffer, C., Kuhr, K., Lorenz, H. M., Moinzadeh, P., Hunzelmann, N., Kreuter, M., Bonella, F., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Guenther, C., Susok, L., Zeidler, G., Koetter, I, Mueller-Ladner, U., Krieg, T., Juche, A., Schmeiser, T., Riemekasten, G., Aberer, E., Gaebelein-Wissing, N., Distler, J. H. W., Sardy, M., Pfeiffer, C., Kuhr, K., Lorenz, H. M., Moinzadeh, P., and Hunzelmann, N.

Published
2020

44. Does anti-acid treatment influence disease progression in SSc-ILD? Data form the German SSc-network

Author

Kreuter, M., Bonella, F., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Guenther, C., Susok, L., Zeidler, G., Koetter, I, Mueller-Ladner, U., Krieg, T., Juche, A., Schmeiser, T., Riemekasten, G., Aberer, E., Gaebelein-Wissing, N., Distler, J. H. W., Sardy, M., Pfeiffer, C., Kuhr, K., Lorenz, H. M., Moinzadeh, P., Hunzelmann, N., Kreuter, M., Bonella, F., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Guenther, C., Susok, L., Zeidler, G., Koetter, I, Mueller-Ladner, U., Krieg, T., Juche, A., Schmeiser, T., Riemekasten, G., Aberer, E., Gaebelein-Wissing, N., Distler, J. H. W., Sardy, M., Pfeiffer, C., Kuhr, K., Lorenz, H. M., Moinzadeh, P., and Hunzelmann, N.

Published
2020

45. Large Variability of Frequency and Type of Physical Therapy in Patients in the German Network for Systemic Sclerosis

Author

Belz, D., Moinzadeh, P., Riemekasten, G., Henes, J., Mueller-Ladner, U., Blank, N., Koetter, I, Siegert, E., Pfeiffer, C., Schmalzing, M., Zeidler, G., Schmeiser, T., Worm, M., Guenther, C., Susok, L., Kreuter, A., Sunderkoetter, C., Juche, A., Aberer, E., Gaebelein-Wissing, N., Ramming, A., Kuhr, K., Hunzelmann, N., Belz, D., Moinzadeh, P., Riemekasten, G., Henes, J., Mueller-Ladner, U., Blank, N., Koetter, I, Siegert, E., Pfeiffer, C., Schmalzing, M., Zeidler, G., Schmeiser, T., Worm, M., Guenther, C., Susok, L., Kreuter, A., Sunderkoetter, C., Juche, A., Aberer, E., Gaebelein-Wissing, N., Ramming, A., Kuhr, K., and Hunzelmann, N.

Abstract

Objective To determine the type and frequency of physical therapy (PT) prescribed by physicians for patients in the registry of the German Network for Systemic Sclerosis. Methods The data for 4,252 patients were analyzed using descriptive statistics, chi-square tests, and odds ratios (ORs). Results Overall, 37.4% of patients (1,590 of 4,252) receivedPTat the end of a yearly follow-up. The most frequently used type ofPTwas lymphatic drainage (n = 1,061, 36.8%), followed by exercise therapy (n = 1,047, 36.3%) and heat therapy (n = 689, 23.9%). More than three-fourths of treated patients (82%) received 1 or 2 different forms ofPTsimultaneously. The prescription ofPTwas associated with the extent of skin fibrosis as measured by the modified Rodnan skin thickness score (<10 [41.8% of patients], 11-20 [55.8% of patients], and >21 [63.9% of patients];P< 0.001). Patients with musculoskeletal involvement (e.g., arthritis, muscle weakness, joint contractures, tendon friction rubs) had a higher chance of receivingPTthan patients without these symptoms, with correspondingORs ranging from 1.96 (95% confidence interval [95%CI] 1.69-2.28) for joint contractures to 3.83 (95%CI2.89-5.08) for arthritis. When comparing the type ofPTprescription across the initial and all follow-up visits from 2003 to 2017, significant alterations with a decreasing frequency of patients receivingPTcould be observed (P= 0.001). Conclusion To our knowledge, this is the first study reporting the use ofPTin patients with systemic sclerosis (SSc) in a large cohort. AlthoughSSc is characterized by considerable disability and restriction of motion, PT.

Published
2020

48. Large Variability of Frequency and Type of Physical Therapy in Patients in the German Network for Systemic Sclerosis

Author

Belz, D., primary, Moinzadeh, P., additional, Riemekasten, G., additional, Henes, J., additional, Müller‐Ladner, U., additional, Blank, N., additional, Koetter, I., additional, Siegert, E., additional, Pfeiffer, C., additional, Schmalzing, M., additional, Zeidler, G., additional, Schmeiser, T., additional, Worm, M., additional, Guenther, C., additional, Susok, L., additional, Kreuter, A., additional, Sunderkoetter, C., additional, Juche, A., additional, Aberer, E., additional, Gaebelein‐Wissing, N., additional, Ramming, A., additional, Kuhr, K., additional, and Hunzelmann, N., additional

Published
2020
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50. AB0584 DOES ANTI-ACID TREATMENT INFLUENCE DISEASE PROGRESSION IN SYSTEMIC SCLEROSIS INTERSTITIAL LUNG DISEASE (SSC-ILD)? DATA FROM THE GERMAN SSC-NETWORK

Author

Kreuter, M., primary, Bonella, F., additional, Riemekasten, G., additional, Müller-Ladner, U., additional, Henes, J., additional, Siegert, E., additional, Guenther, C., additional, Koetter, I., additional, Blank, N., additional, Pfeiffer, C., additional, Schmalzing, M., additional, Zeidler, G., additional, Korsten, P., additional, Susok, L., additional, Juche, A., additional, Worm, M., additional, Jandova, I., additional, Ehrchen, J., additional, Sunderkoetter, C., additional, Keyszer, G., additional, Ramming, A., additional, Schmeiser, T., additional, Kreuter, A., additional, Kuhr, K., additional, Lorenz, H. M., additional, Moinzadeh, P., additional, and Hunzelmann, N., additional

Published
2020
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