Background: Vascular damage is a key pathological process in systemic sclerosis (SSc) and accounts for significant disease-related morbidity. To determine the clinical burden of severe digital vasculopathy (SDV) we have reviewed hospital-based treatment for this important complication of SSc in a large single centre cohort.
Methods: Cases were identified from a cohort of 1168 patients with a diagnosis of SSc who were reviewed during an 18 month period. Patients with recorded episodes of SDV-related complications (digital ulceration, critical digital ischaemia or digital gangrene), requiring surgical amputation, digital sympathectomy or admissions for intravenous prostacyclin or calcitonin gene related peptide (CGRP) and/or intravenous antibiotic treatment were identified.
Results: From this large SSc cohort 17.4% had SDV-related complications. Contrary to expectation, their frequency was significantly higher among the patients with the diffuse cutaneous subset of SSc (27.5%) compared to 13% among the patients with limited cutaneous SSc (p<0.0001). 16.6% had at least one recorded episode of digital ulcers and 12% required at least one hospitalization during the 18 months for treatment with intravenous prostacyclin/CGRP. Overall there were 242 admissions with a mean duration of 6 days.
Conclusions: Digital vasculopathy is a serious complication of SSc contributing significant morbidity and often requiring hospital-based management.
- Raynauds phenomenon
- digital ulcers
- systemic sclerosis