Date: Sunday, November 5, 2017
Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Previously, we have identified short disease duration (≤15 months) and low baseline modified Rodnan skin score (mRSS) (≤22/51) as independent predictors of progressive skin fibrosis (>5 mRSS units and ≥25% increment within 1 year) in patients with diffuse cutaneous systemic sclerosis (dcSSc). While this strategy has resulted in recruitment of more patients with progression of skin fibrosis in clinical trials, it might lead to recruitment of patients with less severe internal organ disease. This study was designed to determine whether progression of skin fibrosis is associated with progression of internal organ disease on longitudinal follow-up of patients with dcSSc.
This study analyzed prospectively collected data from the EUSTAR cohort. Inclusion criteria were: diagnosis of SSc fulfilling ACR criteria, diffuse cutaneous involvement, mRSS ≥7 at baseline visit in 2009 or later, valid mRSS with a time interval of 12±3 months after baseline visit, and at least one available annual follow-up visit.
Progression of skin fibrosis was defined as an increase in mRSS >5 units and ≥25% from baseline to 12±3 months later. The outcome was defined as one of the following new events occurring at any follow-up visit based on expert group consensus: 1) renal crisis; 2) decrease in forced vital capacity (FVC) ≥10%; 3) left ventricular ejection fraction (LVEF) <45% or decrease in LVEF by >10% for patients with baseline LVEF <50%; 4) pulmonary hypertension (PH) on echocardiography as judged by the investigator; 5) death.
Kaplan–Meier analyses and log-rank tests were used to compare disease progression between progressors and non-progressors for up to 6 years of follow-up.
A total of 871 dcSSc patients were eligible. The median follow-up period was 3.26 years (IQR: 1.49–5.14). Sixty-seven (7.7%) patients had worsening of skin fibrosis within 1 year (defined as progressors). Cumulatively, 235/666 (35.3%) patients had a decrease in FVC ≥10%, 96/593 (16.2%) had new PH, 22/564 (3.9%) had worsening LVEF, 16/844 (1.9%) had a new renal crisis, and 58/871 (6.7%) died. Mean FVC% at baseline was 87.2% ± 20.4%, while 134 (20.1%) patients had baseline FVC% <70%.
Log-rank tests indicated the probability for a decrease in FVC ≥10% was significantly higher for progressors than non-progressors (53.2% vs. 33.9%, p=0.004) (Figure). There were non-significant differences in probabilities for new PH (9.5% vs. 16.7%), worsening LVEF (7.3% vs. 3.6%), new renal crisis (0.0% vs. 2.1%) and death (7.5% vs. 6.6%) between progressors and non-progressors.
Progression of skin fibrosis is associated with a decline in lung function in dcSSc patients over follow-up. Our data indicate that evidence-based criteria for cohort enrichment of progressive skin fibrosis might also be appropriate to recruit more dcSSc patients at higher risk of lung function decline in future clinical trials.
To cite this abstract in AMA style:Wu W, Jordan S, Graf N, Pena J, Curram J, Allanore Y, Matucci-Cerinic M, Pope JE, Denton C, Khanna D, Distler O. Progression of Skin Fibrosis Is Associated with Decline in Lung Function in Patients with Diffuse Cutaneous Systemic Sclerosis: A European Scleroderma Trials and Research (EUSTAR) Analysis [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/progression-of-skin-fibrosis-is-associated-with-decline-in-lung-function-in-patients-with-diffuse-cutaneous-systemic-sclerosis-a-european-scleroderma-trials-and-research-eustar-analysis/. Accessed June 4, 2020.
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