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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Scleroderma-Specific Autoantibodies and Disease Trajectories in Early Systemic Sclerosis: Insights From a
Marzieh Jamali1, Amisha Kaur1, Suiyuan Huang1,2
1Scleroderma Program, University of Michigan, Ann Arbor, MI, USA.
Objective:
Early diffuse cutaneous systemic sclerosis (dcSSc) is associated with substantial disease-specific mortality. Scleroderma-specific autoantibodies (SSc-Ab) may influence disease course, but their prognostic value in very early dcSSc remains unclear. We examined associations between autoantibody profiles and disease trajectories for clinical events in a prospective single-center cohort.
Methods:
We retrospectively analyzed prospectively collected data from a subset of patients enrolled in an early SSc registry between October 2012 and July 2021, including those with early dcSSc or individuals at risk for dcSSc less than two years of their first non-Raynaud phenomenon (RP) symptom. Participants were classified as anti-RNA polymerase III antibody positive (RNAP3+), anti-topoisomerase I antibody positive (Scl70+), or triple-negative (negative for RNAP3, Scl70, and anticentromere antibody [ACA]).
Results:
Among 115 patients with available SSc-Ab data (median follow-up, 5.5 years [interquartile range 2.1-7.2]), 42 were RNAP3+, 29 Scl70+, 6 ACA+, and 38 triple-negative. ACA+ patients were excluded from subgroup analyses because of the small sample size. Of the remaining 109 patients, 17 with prior clinical events at baseline were excluded from time-to-event analyses. Among the 92 included patients, 50 (54.3%) experienced one or more new clinical events during follow-up, including 59.4% of RNAP3+, 57.1% of Scl70+, and 46.9% of triple-negative patients. Among first new clinical events, RNAP3+ patients had higher frequencies of scleroderma renal crisis (6.3% vs 3.6% in Scl70+ and 0% in triple-negative), malignancy (35.7% vs 24.1% and 15.8%, respectively), mortality (9.4% vs 3.6% and 0%, respectively), and forced vital capacity decline (15.6% vs 10.7% and 12.5%, respectively), whereas modified Rodnan skin score worsening was most frequent in triple-negative patients (15.6% vs 10.7% in Scl70+ and 9.4% in RNAP3+).
Conclusion:
Scleroderma-specific autoantibodies in early SSc were associated with distinct baseline clinical phenotypes and showed numerical differences in longitudinal clinical outcomes in this single-center cohort.
