Background/Purpose: Systemic sclerosis is an autoimmune disease characterized by inflammation, vasculopathy and fibrosis of the skin, vasculature and internal organs along with disease specific autoantibody formation. Patients are subtyped as limited or diffuse scleroderma based on extent of skin involvement and autoantibody profile. Recent retrospective studies have shown that autoantibodies may precede a clinical diagnosis of scleroderma by several years. However, some individuals with scleroderma specific autoantibodies and no symptoms never progress to a clinical diagnosis of scleroderma. The purpose of this study is to investigate the characteristics of asymptomatic scleroderma antibody positive (ASAP) individuals in a longitudinal biorepository.

Methods: All subjects gave written informed consent for longitudinal collection of their data. Data is collected on demographics, baseline disease phenotype, baseline disease activity scores including modified Rodnan skin score (mRSS), scleroderma health assessment questionnaire disability index (HAQ-DI), gastrointestinal score, physician global assessment and Medsger severity score. Data were analyzed using GraphPad Prism (version 7.0).

Results: At the time of data-lock, there were 90 patients with data available for analysis. At the baseline visit, 41 fulfilled ACR criteria for limited scleroderma, 19 met criteria for diffuse scleroderma, and 30 patients were in the ASAP group. There were no statistically significant differences in age, gender or race in the limited, diffuse of ASAP patients. Patients with limited scleroderma were more commonly centromere antibody positive (p 0.0031). As expected, baseline mRSS was significantly higher in the diffuse scleroderma subset. Over a mean follow up of 1.27 years, patients in the ASAP group demonstrated no significant change in mRSS indicating lack of progression of disease.

Conclusion: The current study investigated a cohort of asymptomatic scleroderma antibody positive individuals enrolled in the STOP scleroderma biorepository who do not exhibit diagnostic criteria for scleroderma at baseline and did not demonstrate progression to clinically diagnosable scleroderma. This cohort of patients with autoantibody positivity but no progression of disease is of interest to investigate molecular biomarkers of non-progression in patients with scleroderma specific antibody positivity.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/asymptomatic-scleroderma-antibody-positivity-and-progression-to-systemic-sclerosis/