Naslov
Progressive skin fibrosis is associated with a decline in lung function and worse survival in patients with diffuse cutaneous systemic sclerosis in the European Scleroderma Trials and Research (EUSTAR) cohort

Autori
Wu, Wanlong ; Jordan, Suzana ; Graf, Nicole ; de Oliveira Pena, Janethe ; Curram, John ; Allanore, Yannick ; Matucci- Cerinic, Marco ; Pope, Janet E. ; Denton, Christopher P. ; Khanna, Dinesh ; Distler, Oliver ; ... ; Novak, Srđan ; ... ; Martinović, Duška ; ... ; Anić, Branimir ; Barešić, Marko ; Mayer, Miroslav ; ... EUSTAR Collabors

Kolaboracija
EUSTAR Coauthors

Izvornik
Annals of the rheumatic diseases (0003-4967) 78 (2019), 5; 648-656

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, znanstveni

Ključne riječi
scleroderma ; diffuse ; fibrosis ; biomarkers ; risk analysis

Sažetak
Objectives To determine whether progressive skin fibrosis is associated with visceral organ progression and mortality during follow-up in patients with diffuse cutaneous systemic sclerosis (dcssc). Methods We evaluated patients from the european scleroderma Trials and Research database with dcssc, baseline modified Rodnan skin score (mRss) ≥7, valid mRss at 12±3 months after baseline and ≥1 annual follow-up visit. Progressive skin fibrosis was defined as an increase in mRss >5 and ≥25% from baseline to 12±3 months. Outcomes were pulmonary, cardiovascular and renal progression, and all-cause death. associations between skin progression and outcomes were evaluated by Kaplan-Meier survival analysis and multivariable Cox regression. results Of 1021 included patients, 78 (7.6%) had progressive skin fibrosis (skin progressors). Median follow-up was 3.4 years. survival analyses indicated that skin progressors had a significantly higher probability of FVC decline ≥10% (53.6% vs 34.4% ; p<0.001) and all-cause death (15.4% vs 7.3% ; p=0.003) than non-progressors. These significant associations were also found in subgroup analyses of patients with either low baseline mRss (≤22/51) or short disease duration (≤15 months). in multivariable analyses, skin progression within 1 year was independently associated with FVC decline ≥10% (HR 1.79, 95% Ci 1.20 to 2.65) and allcause death (HR 2.58, 95% Ci 1.31 to 5.09). Conclusions Progressive skin fibrosis within 1 year is associated with decline in lung function and worse survival in dcssc during follow-up. These results confirm mRss as a surrogate marker in dcssc, which will be helpful for cohort enrichment in future trials and risk stratification in clinical practice.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

POVEZANOST RADA