Clinical Vignette
Department of Rheumatology, St Vincent's University Hospital, Dublin, Ireland
Correspondence to: C. Matthews. E-mail: cfmatthews{at}doctors.net
The patient, a 69-yr-old man, had seropositive erosive rheumatoid arthritis (RA) diagnosed in 1977. He failed on the conventional disease-modifying agents of salazopyrin and methotrexate. Complications of Felty's syndrome (splenomegaly, neutropenia) and osteoporosis developed during his disease course. In 1997 he developed skin ulceration on the right first web space, left medial malleolus and right medial malleolus.
Clinically, his lower limb pulses were palpable with no venous insufficiency. Peripheral nerves were intact. Arterial pulse pressures were normal. Blood glucose and cholesterol were within normal limits. Anti-neutrophil cytoplasmic antigens were negative. Serum inflammatory markers were elevated, with erythrocyte sedimentation rate (ESR) at 90 mm/h and C-reactive protein (CRP) at 67 mg/dl. While pyoderma granulosum was considered, following a dermatological review a clinical diagnosis of vasculitic ulceration of the skin associated with Felty's syndrome was made in the light of his previous features. Treatment was high-dose corticosteroids and oral cyclophosphamide. There was an initial improvement in the size of the ulcers. After 6 months of therapy, cyclophosphamide was switched to azathioprine. With reduction in corticosteroid, the ulcers again deteriorated. Treatment was adjusted with the administration of intravenous prostaglandins and pulse cyclophosphamide. Subsequent maintenance therapy was oral cyclophosphamide.
In 2001, due to inefficacy, treatment was changed to infliximab (dose 3 mg/kg every 8 weeks). Over the subsequent months, there was a dramatic improvement at the right medial malleolus and complete healing at the other two sites. The patient's joint symptoms also diminished, although ESR remained elevated at 30 mm/h and neutropenia persisted. Due to the development of a septic prosthetic joint, infliximab therapy was discontinued. During this time there was a significant deterioration in the areas of ulceration. Improvement was noted again when anti-TNF therapy, this time adalimumab, was reinstituted with prophylactic antibiotic cover.
Anti-TNF therapy can be an effective treatment for systemic large- and medium-vessel vasculidites. Cutaneous manifestations also respond with pyoderma gangrenosum healing in patients with inflammatory bowel disease. There appears to be increased blood flow due to improved endothelial vasomotor responses following anti-TNF therapy [1]. Adverse event reporting has shown paradoxically that these agents can be associated with the development of new-onset vasculitis [2]. The mechanism of this is unclear. Our case highlights a role for anti-TNF therapy in the treatment of cutaneous ulceration secondary to RA/Felty's syndrome.
The authors have declared no conflicts of interest.
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- Booth AD, Jayne DR, Kharbanda RK, et al. (2004) Infliximab improves endothelial dysfunction in systemic vasculidites: a model of vascular inflammation. Circulation 109:1718–23.
[Abstract/Free Full Text] - McCain ME, Quinet RJ, Davis WE. (2002) Etanercept and infliximab associated with cutaneous vasculitis. Rheumatology 41:116–7.
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