Response of crescentic Henoch-Schoenlein purpura nephritis to corticosteroid and azathioprine therapy.

The benefits of treating severe Henoch-Schoenlein Purpura (HSP) glomerulonephritis have not been established. In this study, we evaluate the outcome of 21 children with severe HSP nephritis treated with corticosteroids and azathioprine. Between 1977 and 1995, 78 children (age range 1 to 16 years) were seen for evaluation of HSP. Thirty-one underwent kidney biopsy; indications included nephritic and/or nephrotic onset (15 patients), persistently decreased creatinine clearance (5 patients), or proteinuria > 4 g/24 h (11 patients). Twenty treated patients had diffuse mesangial proliferation with crescents in 6-100% (mean 40%) of glomeruli. One treated patient, not biopsied due to extreme obesity, had a creatinine clearance of 49 ml/min/1.73 m2 and proteinuria of 21.3 g/24 h. These 21 patients were initially treated with azathioprine and daily oral prednisone (13 patients) or i.v. methyl-prednisolone (8 patients), followed by azathioprine and alternate-day prednisone for 9-24 (mean 15) months. The average follow-up was 32 months. Over the course of follow-up, 19 treated patients showed a decline in hematuria (> 5 red blood cells/high power field) from 100% to 16% (p < 0.01), a fall in the serum creatinine from 1.71 +/- 2.20 to 0.78 +/- 0.25 mg/dl (p < 0.01), an increase in creatinine clearance from 76 +/- 43 to 122 +/- 26 ml/min/1.73 m2 (p < 0.01), and a reduction in proteinuria from 8.8 +/- 7.5 to 0.47 +/- 0.39 g/24 h (p < 0.01). Two treated patients progressed to end-stage renal failure. There was no difference in outcome comparing patients initially treated with prednisone versus methyl-prednisolone. These observations suggest that corticosteroid and azathioprine therapy is effective in crescentic HSP nephritis.