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 (CROSBI ID 274768)
Prilog u časopisu | izvorni znanstveni rad | međunarodna recenzija
Podaci o odgovornosti
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
EUSTAR Coauthors
engleski
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
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.
scleroderma ; diffuse ; fibrosis ; biomarkers ; risk analysis
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Podaci o izdanju
78 (5)
2019.
648-656
objavljeno
0003-4967
1468-2060
10.1136/annrheumdis-2018-213455
Povezanost rada
Kliničke medicinske znanosti