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.