Clinical burden of digital vasculopathy in limited and diffuse cutaneous systemic sclerosis.
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 with 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 hospitalisation 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.
Centre for Rheumatology, Royal Free Hospital, Pond Street, London NW3 2QG, UK.
S I
SI
Nihtyanova
G M
GM
Brough
C M
CM
Black
C P
CP
Denton
D000328:Adult; D001157:Arterial Occlusive Diseases / Q000150:complications* / Q000473:pathology; D001424:Bacterial Infections / Q000150:complications / Q000473:pathology; D005260:Female; D005385:Fingers / Q000098:blood supply* / Q000473:pathology; D005734:Gangrene / Q000150:complications / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D045743:Scleroderma, Diffuse / Q000150:complications / Q000473:pathology; D012594:Scleroderma, Localized / Q000150:complications / Q000473:pathology; D012595:Scleroderma, Systemic / Q000150:complications* / Q000473:pathology; D012883:Skin Ulcer / Q000150:complications / Q000473:pathology