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Successful treatment with bosentan for severe digital ischaemia in limited cutaneous systemic sclerosis
  1. Emma P K Yu1,
  2. Andrew J K Ostor1,
  3. Frances C Hall2
  1. 1Addenbrooke’s Hosptial, Cambridge, UK
  2. 2University of Cambridge Department of Clinical Medicine, Addenbrooke’s Hospital, Cambridge, UK
  1. Correspondence to:
    Dr F C Hall
    Box 157, University of Cambridge Department of Clinical Medicine, Addenbrooke’s Hospital, Cambridge CB2 2QQ, UK; fch22{at}

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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 …

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  • 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.