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

BMJ Publishing Group 2019-05

Wu, Wanlong
Jordan, Suzana; https://orcid.org/0000-0002-8114-8239
Graf, Nicole
de Oliveira Pena, Janethe
Curram, John
Allanore, Yannick; https://orcid.org/0000-0002-6149-0002
Matucci-Cerinic, Marco; https://orcid.org/0000-0002-9324-3161
Pope, Janet E; https://orcid.org/0000-0003-1479-5302
Denton, Christopher P; https://orcid.org/0000-0003-3975-8938
Khanna, Dinesh; https://orcid.org/0000-0003-1412-4453
Distler, Oliver; https://orcid.org/0000-0002-0546-8310
EUSTAR collaborators

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 all-cause 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.

Rheumatology Clinic and Institute of Physical Medicine; 610 Medicine & health; Journal Article; PeerReviewed; info:eu-repo/semantics/article; info:eu-repo/semantics/publishedVersion

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info:doi/10.5167/uzh-170631
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English

CHUZH oai:www.zora.uzh.ch:170631
https://www.zora.uzh.ch/id/eprint/170631/1/648.full.pdf
info:doi/10.5167/uzh-170631
info:doi/10.1136/annrheumdis-2018-213455
info:pmid/30852552
urn:issn:0003-4967
1416174607

HAUPTBIBLIOTHEK UNIV OF ZURICH
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