Refractory Henoch-Schönlein purpura: atypical aetiology and management.

BACKGROUND: A 27-year-old white male was referred with recently diagnosed refractory Henoch-Schönlein purpura nephritis (HSPN). He had a past medical history of hypertension of four years duration and was prescribed lisinopril. He was switched from a brand to a generic lisinopril two days before the onset of first symptoms. On two occasions symptoms had worsened while trying to switch back to the generic drug. He had 9 g/day protein in his urine with haematuria and serum creatinine was up to 123.76 µmol/l (normal value < 106 µmol/l). TREATMENT: The patient underwent two renal biopsies; the first was carried out early after having HSP symptoms and the second was performed a month later. The first biopsy showed mild segmental endocapillary hypercellularity with IgA deposits consistent with HSPN. The second biopsy showed proliferative IgA nephropathy with focal active cellular crescent formation in 38% of the glomeruli.
RESULTS: He did not respond to steroids, cyclophosphamide and plamapheresis. However, his renal and extra-renal manifestations eventually improved after he had received three doses of 1,000 mg rituximab by infusion, two weeks apart.
CONCLUSION: Rituximab might be efficacious in treatment of hspn, more studies are needed to assess long-term safetey and efficacy.