; Bancel, D. F. ; Allanore, Y. ; Muller-Ladner, U. ; Distler, O. ; Iannone, F.
; Pellerito, R. ; Pileckyte, M. ; Miniati, I. ; Ananieva, L. ; Gurman, A. B. ; Damjanov, N. ; Mueller, A. ; Valentini, G. ; Riemekasten, G. ; Tikly, M. ; Hummers, L.
; Henriques, M. J. ; Caramaschi, P. ; Scheja, A. ; Rozman, B. ; Ton, E. ; Kumanovics, G. ; Coleiro, B. ; Feierl, E. ; Szucs, G.
; Von Muhlen, C. A. ; Riccieri, V. ; Novak, S. ; Chizzolini, C. ; Kotulska, A. ; Denton, C. ; Coelho, P. C. ; Kotter, I. ; Simsek, I. ; de la Pena Lefebvre, P. G. ; Hachulla, E. ; Seibold, J. R. ; Rednic, S. ; Stork, J.
; Morovic-Vergles, J. ; Walker, U. A. | Dokumentenart: | Artikel | ||||
|---|---|---|---|---|---|
| Titel eines Journals oder einer Zeitschrift: | Annals of the Rheumatic Diseases | ||||
| Verlag: | BMJ PUBLISHING GROUP | ||||
| Ort der Veröffentlichung: | LONDON | ||||
| Band: | 69 | ||||
| Nummer des Zeitschriftenheftes oder des Kapitels: | 10 | ||||
| Seitenbereich: | S. 1809-1815 | ||||
| Datum: | 2010 | ||||
| Institutionen: | Medizin > Lehrstuhl für Innere Medizin I | ||||
| Identifikationsnummer: |
| ||||
| Stichwörter / Keywords: | AUTOIMMUNE RHEUMATIC-DISEASES; INTERSTITIAL LUNG-DISEASE; POPULATION-BASED COHORT; PULMONARY-FIBROSIS; SURVIVAL; MORTALITY; ATHEROSCLEROSIS; MANIFESTATIONS; HYPERTENSION; ACCURACY; | ||||
| Dewey-Dezimal-Klassifikation: | 600 Technik, Medizin, angewandte Wissenschaften > 610 Medizin | ||||
| Status: | Veröffentlicht | ||||
| Begutachtet: | Ja, diese Version wurde begutachtet | ||||
| An der Universität Regensburg entstanden: | Ja | ||||
| Dokumenten-ID: | 65681 |
Zusammenfassung
Objectives To determine the causes and predictors of mortality in systemic sclerosis (SSc). Methods Patients with SSc (n=5860) fulfilling the American College of Rheumatology criteria and prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) cohort were analysed. EUSTAR centres completed a structured questionnaire on cause of death and comorbidities. Kaplan-Meier and Cox ...

Zusammenfassung
Objectives To determine the causes and predictors of mortality in systemic sclerosis (SSc). Methods Patients with SSc (n=5860) fulfilling the American College of Rheumatology criteria and prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) cohort were analysed. EUSTAR centres completed a structured questionnaire on cause of death and comorbidities. Kaplan-Meier and Cox proportional hazards models were used to analyse survival in SSc subgroups and to identify predictors of mortality. Results Questionnaires were obtained on 234 of 284 fatalities. 55% of deaths were attributed directly to SSc and 41% to non-SSc causes; in 4% the cause of death was not assigned. Of the SSc-related deaths, 35% were attributed to pulmonary fibrosis, 26% to pulmonary arterial hypertension (PAH) and 26% to cardiac causes (mainly heart failure and arrhythmias). Among the non-SSc-related causes, infections (33%) and malignancies (31%) were followed by cardiovascular causes (29%). Of the non-SSc-related fatalities, 25% died of causes in which SSc-related complications may have participated (pneumonia, sepsis and gastrointestinal haemorrhage). Independent risk factors for mortality and their HR were: proteinuria (HR 3.34), the presence of PAH based on echocardiography (HR 2.02), pulmonary restriction (forced vital capacity below 80% of normal, HR 1.64), dyspnoea above New York Heart Association class II (HR 1.61), diffusing capacity of the lung (HR 1.20 per 10% decrease), patient age at onset of Raynaud's phenomenon (HR 1.30 per 10 years) and the modified Rodnan skin score (HR 1.20 per 10 score points). Conclusion Disease-related causes, in particular pulmonary fibrosis, PAH and cardiac causes, accounted for the majority of deaths in SSc.
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