Even when Henoch-Schönlein purpura is not accompanied by nephritis, powerful immunosuppressive therapy may be justified.
CASE HISTORY
A woman of 37 was referred after developing abdominal pain, arthralgia and a purpuric rash over her buttocks and legs a few days after an upper respiratory tract infection. She had had two similar episodes both lasting over six months. In the more recent episode, tests for autoantibodies, rheumatoid factor, hepatitis serology, antinuclear cytoplasmic antibodies (ANCA), complement and cryoglobulins, and electrophoresis, had all been normal; skin biopsy had shown a leucocytoclastic vasculitis and Henoch-Schönlein purpura (HSP) had been diagnosed. On the present admission renal biochemistry, full blood count, clotting, C-reactive protein and throat swab were all normal. A vasculitic screen was again negative. Recurrent HSP was diagnosed according to the American College of Rheumatology criteria.1
A short course of dapsone was started, initially with good effect. Unfortunately her symptoms flared with dose reduction and the treatment was ultimately withdrawn three months later because of methaemoglobinaemia. Despite standard supportive treatment followed by oral corticosteroids, she had distressing weight loss with arthralgia and continuing abdominal pain. At this point she required admission to hospital for nutritional support, intravenous methylprednisolone and intravenous immunoglobulins. She made a slow recovery and was discharged home two months later on oral corticosteroids, azathioprine (as a steroid-sparing agent) and regular intravenous immunoglobulins (2 g/kg/month).
A month after discharge the azathioprine was withdrawn because of abnormal liver function tests. Methotrexate was successfully introduced but had to be discontinued seven months into treatment because of neutropenia. Intravenous cyclophosphamide was then given two-weekly for the next six months but was stopped because high doses of prednisolone (>30 mg day) were still needed to control symptoms. She was then started on ciclosporin at an initial dose of 1 mg/kg/day, with continuation of the prednisolone. At follow-up two weeks later her abdominal and joint pains were much improved and a month later she was symptom-free. The ciclosporin was increased to 2 mg/kg/day and the prednisolone was gradually withdrawn without symptom recurrence. After a short disease-free period the patient spontaneously stopped all treatment. She has remained virtually symptom-free for seven months under regular follow-up.
COMMENT
HSP is a multisystem vasculitic disorder of unknown aetiology, typically causing a palpable purpuric rash, abdominal pain, arthralgia and nephritis. The disease usually remits spontaneously but up to a third of patients have recurrences.2 89% of adults with HSP make a complete recovery,3 most requiring only supportive treatment, but systemic steroids are recommended when abdominal pain is present to reduce symptom duration and risk of renal complications.4 In some patients severe symptoms demand a more aggressive approach, and in the case reported here treatment with ciclosporin permitted steroid withdrawal without loss of symptom control. Although we cannot fully exclude spontaneous remission, the coincidence of starting ciclosporin with symptom improvement would be consistent with a therapeutic response to the drug.
Use of ciclosporin has been reported in two children with steroid-dependent HSP. The authors speculate that T-lymphocyte regulation dysfunction has a role in pathogenesis: ciclosporin might act by inhibiting T-cell proliferation.5 There are two other case reports of ciclosporin use in HSP nephritis with nephrotic syndrome. One describes a complete and sustained remission,6 the other no benefit at all.7
The role of ciclosporin in HSP may only be clarified by a randomized trial. In the absence of definitive studies a carefully monitored therapeutic trial of this agent may be indicated in patients who have not responded to conventional treatments.
Acknowledgments
We thank Jill Firth for her contribution.
References
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