Presentation: Hospital-Based Management of Severe Digital Vasculopathy in Systemic Sclerosis (2007)

9 Hospital-Based Management of Severe Digital Vasculopathy in Systemic Sclerosis

Purpose
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 incidence and hospital-based treatment for this complication of SSc in a large single centre cohort.
Methods
SSc cases with SDV (Raynaud’s phenomenon complicated by digital ulceration (DU), critical digital ischaemia, or gangrene, or requiring sympathectomy) were identified. Demographic and clinical characteristics were recorded and patients who needed hospitalization were identified.
Results
From a cohort of 1168 SSc patients reviewed during an 18 month period we identified 203 patients (17.4%) with SDV. 78% of them were female and 53% had limited cutaneous SSc (lcSSc). The frequency of SDV among the patients with diffuse cutaneous SSc was significantly higher than that among the lcSSc patients (27.5% and 13% respectively, p<0.0001). Their median ± SD age was 53±14 years while the duration of their disease was 104±102 months. One third (n=65) of the SDV cohort was on active immunosuppressive treatment and three quarters were receiving at least one vasodilator for Raynaud’s phenomenon. In addition 19 % (n=38) of the patients were on anti-platelet agents.
We found 194 patients (16.6% of the SSc cohort) with at least one recorded episode of DU. The average number of DU episodes over the 18 month period in these patients was 1.5±0.9 while the average number of ulcers they had was 2.5±1.9. 184 patients (16%) had finger and 18 (1.5%) had toe ulcers. 19 patients (1.6%) developed critical digital ischaemia and 16 (1.4%) developed digital gangrene. In 11 (0.9%) patients this resulted in auto- or required surgical amputation and 5 (0.4%) of the patients had a history of amputation prior to the 18 month follow-up period. 13 patients (1%) were referred for digital sympathectomy.
Of the SSc cohort 141 patients (12%) were admitted in hospital at least once to receive intravenous prostacyclin or calcitonic gene related peptide (CGRP) during the 18 month window. Of them 77 had at least one recorded episode of SDV-related complication and the remaining 64 were receiving the treatment as a preventative measure. In the whole SSc cohort the frequency of admissions was significantly higher among the SDV patients (38%) compared to patients with no recorded SDV episodes (7%; p<0.0001). 63 patients (5.4% of the SSc cohort) required multiple admissions. There were a total of 242 admissions with a mean duration of 6 days and each patient was hospitalised on average twice, which accounted for a total of 1346 bed-days (9.5 days in hospital per patient on average). In addition 18 patients (1.5%) required parenteral and 56 (5%) oral antibiotic treatment for infected digits and 20 (1.7%) needed opioid analgesics for SDV-related pain.
Conclusions
Digital vasculopathy is a serious complication of SSc contributing significant morbidity and often requiring hospital-based management.

 S.I. Nihtyanova, None; G.M. Brough, None; C.M. Black, Honoraria and research grants from Actelion pharmaceuticals, 2; C.P. Denton, Honoraria and research grants from Actelion pharmaceuticals, 2.