Early accrual of organ damage in systemic sclerosis: Rationale for development of a disease damage index

Abstract

Introduction: Systemic sclerosis (SSc) is characterized by irreversible organ damage rather than fluctuating disease activity. However, there is no validated measure of damage in SSc. We aimed to quantify the accrual of organ damage in patients with early SSc. Methods: Patients enrolled in the Australian Scleroderma Cohort Study with less than 2 years of SSc since the onset of the first non-Raynaud’s symptom were included. Organ damage was defined by a group of six experts as substantial and permanent loss of organ function due to SSc. Results: We identified 278 patients with early SSc. Among these, 38% had diffuse SSc. Damage was more common in the diffuse than in the limited disease subtyp..