Case Studies
Case 9.7 Henoch-Schönlein nephritis
A 12-year-old boy presented with a 1-week history of pain in the left loin. This was diagnosed as a urinary tract infection and treated with amoxycillin. One week later, he developed a purpuric rash around the ankles, accompanied by some blistering and superficial necrosis. Shortly afterwards, he developed pain in the left elbow joint. On admission to hospital, he was noted to have haematuria and proteinuria and a blood pressure of 130/90. Over the next month, he suffered further episodes of abdominal colic and purpura. His haemoglobin was 95g/l with a normal white-cell count. Antinuclear antibodies were negative and total haemolytic complement, C4 and C3 levels were normal. Although his blood urea was normal, his creatinine clearance was low at 31ml/min/m2 with proteinuria of 4.5g/day.
A skin biopsy of a purpuric lesion showed vasculitic changes in the dermis, with IgA and C3 deposition in the blood-vessel walls. A renal biopsy, containing 21 glomeruli per section, showed epithelial crescents and diffuse mesangial hypercellularity in seven glomeruli. On immunofluorescence, granular deposits of IgA and C3 and, to a lesser extent, IgG and properdin were present in the mesangium. The clinical and histological features were those of Henoch-Schönlein nephritis. Because of the heavy proteinuria and diminished creatinine clearance, he was treated with cyclophosphamide (as part of an international study of treatment in this condition). Over a 9-month follow-up period, the purpura and episodes of abdominal colic subsided, his creatinine clearance increased to 47ml/min, but he continued to have moderately heavy proteinuria (3.2g/day). The prognosis is uncertain.
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