In patients with diffuse cutaneous systemic sclerosis (dcSSc), developing progressive skin fibrosis within 1 year is associated with a decline in lung function and worse survival, according to study results published Annals of Rheumatic Diseases.

Investigators conducted an observational study evaluating patients from the European Scleroderma Trials and Research (EUSTAR) database with dcSSc, baseline modified Rodnan skin score (mRSS) of ≥7 units, valid mRSS at 12±3 months following baseline, and ≥1 annual follow-up visit. The researchers sought to establish whether progressive skin fibrosis is associated with visceral organ disease progression and mortality during follow-up in patients with dcSSc. In this study, progressive skin fibrosis was defined as an increase in mRSS of >5 units and by ≥25% from baseline to 12±3 months. Data from patients’ visits between January 1, 2009, and August 31, 2017, were exported from the EUSTAR database. Outcomes included pulmonary, cardiovascular, and renal progression, as well as all-cause mortality.

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Of the 1021 patients included in the analysis, 7.6% (78 of 1021) exhibited progression of skin fibrosis at 1-year follow-up (skin progressors). The median follow-up was 3.4 years. Survival analyses demonstrated that skin progressors compared with nonprogressors had a significantly higher likelihood of a forced vital capacity (FVC) decline of ≥10% (53.6% vs 34.4%, respectively; P <.001) and all-cause mortality (15.4% vs 7.3%, respectively; log-rank test P =.003).

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These significant associations were also reported in subgroup analyses of patients with either low baseline mRSS (≤22 of 51 units) or short disease duration (≤15 months). Multivariable analyses showed that skin progression within 1 year was independently associated with an FVC decline of ≥10% (hazard ratio [HR], 1.79; 95% CI, 1.20-2.65; P =.004) and all-cause mortality (HR, 2.58; 95% CI, 1.31-5.09; P =.006).

The investigators concluded that the results of this study confirm that mRSS is an excellent surrogate marker for long-term disease progression in patients with dcSSc, which will be beneficial for cohort enrichment in future trials and risk stratification in clinical practice. They recommended that patients with short-term progressive skin disease be carefully monitored for disease progression in other organs over the subsequent years.


Wu W, Jordan S, Graf N, et al; on behalf of the EUSTAR Collaborators. 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. Ann Rheum Dis. 2019;78(5):648-646.

This article originally appeared on Pulmonology Advisor