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We report the case of a patient with limited cutaneous systemic sclerosis, who developed progressive digital gangrene. This responded rapidly to bosentan.
A 38-year-old, previously well, male non-smoker presented with an ischaemic left index finger. On examination the distal left index finger was cyanosed; sclerodactyly and facial telangectasia were also noted. Investigations revealed haemoglobin 10.7 g/dl (normocytic); erythrocyte sedimentation rate 94 mm in the first hour; normal C-reactive protein levels; positive antinuclear, anticentromere and anti-Ro antibodies; normal complement components; no anticardiolipin antibodies, lupus anticoagulant activity or cryoglobulin; negative blood cultures; normal chest radiograph and ECG; arteriogram of the left arm revealed no arterial occlusion and was consistent with small vessel disease; and endoscopy …
Funding: FCH is funded by the Arthritis Research Campaign, Cambridge Arthritis Research Endeavour and The Evelyn Trust.
Competing interests: FCH has received funding from Actelion for a part-time research nurse.
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