Progressive skin fibrosis should trigger enhanced monitoring in dcSSc
medwireNews: Progressive skin fibrosis is significantly associated with worsening lung function and an increased risk for death among patients with diffuse cutaneous systemic sclerosis (dcSSc), an analysis of EUSTAR data shows.
Based on their findings, Oliver Distler (University Hospital Zurich, Switzerland) and co-workers say that “[p]atients with short term progressive skin disease should be carefully monitored for other organ progression in the following years.”
The study included 1021 patients (mean age 52.0 years) with dcSSc from the EUSTAR (European Scleroderma Trials and Research) database who had a mean disease duration of 7.7 years and baseline modified Rodnan skin score (mRSS) of 7 or more (mean of 16.9).
At 12 months, 7.6% of the patients had progressive skin fibrosis, defined as an increase in mRSS of more than 5 and at least a 25% increase from baseline.
During a median 3.4 years of follow-up, patients with skin progression were significantly more likely than those without to experience worsening lung function, defined as a forced vital capacity (FVC) decline of 10% or more, at rates of 53.6% versus 34.4%.
The all-cause mortality rate was also significantly higher among the patients with versus without skin progression, at 15.4% versus 7.3%, and after adjustment for multiple clinically relevant confounders, skin progression was independently associated with both FVC decline and all-cause mortality risk, at hazard ratios of 1.79 and 2.58, respectively.
Distler and team note that similar significant differences in lung progression and all-cause mortality were observed in subgroup analyses of patients with either low baseline mRSS (≤22/51 units) or short disease duration (≤15 months), which have previously been identified as independent predictors of progressive skin fibrosis.
By contrast, there was no significant difference in these outcomes between individuals with versus without skin progression among patients with a baseline mRSS above 22/51 units or a disease duration of longer than 15 months.
Writing in the Annals of the Rheumatic Diseases, the researchers say that these results “support the concept that inclusion of patients with lower mRSS or shorter disease duration can enrich clinical trials for progressive skin fibrosis, and this enrichment leads to study populations with more severe disease at higher risk of organ progression and overall death.”
They conclude that their data “show that mRSS progression is an excellent surrogate marker for long-term disease progression in SSc, which supports the use of mRSS as an end point in clinical trials.”
By Laura Cowen
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