An observational cohort study of patients with newly diagnosed digital ulcer disease secondary to systemic sclerosis registered in the EUSTAR database

摘要

Systemic sclerosis (SSc) is a multisystem autoimmune disease characterised by an occlusive vasculopathy of small arteries with intimal hyperplasia and endothelial dysfunction (1). Patients with SSc develop digital ulcers (DU) as a clinical correlate of this macroangiop-athy, although repetitive microtrauma, pressure, and calcinosis have been also implicated (2). DU are painful, necrotic lesions located at the distal tips of digits or overlying bony prominences, are typically slow to heal and are often complicated by infections (3, 4). Up to one-half of patients with SSc will develop DU at some time during their disease (5) and among these almost three-quarters will have developed their first ulcer within 5 years of SSc diagnosis (6). Severe digital vasculopathy contributes significantly to the morbidity of SSc patients (7).