REGRESSION OF HENOCH-SCHONLEIN DISEASE WITH INTENSIVE IMMUNOSUPPRESSIVE TREATMENT

Objective: To assess the results of a new immunosuppressive cycle, whi ch had given favorable results in other immune-mediated glomerulonephr itides, in the treatment of Henoch-Schonlein disease. Methods: Eight p atients (seven male and one female; age range, 13 to 61 years) with bi opsy-proved Henoch-Schonlein were treated with the following protocol: (1) induction with 250 to 750 mg intravenous methylprednisolone every day for 3 to 7 days plus 100 to 200 mg oral cyclophosphamide every da y, (2) maintenance with 100 to 200 mg oral prednisone on alternate day s plus cyclophosphamide, as before, for 30 to 75 days; (3) tapering, w ith prednisone reduced on average by 25 mg every month while the cyclo phosphamide dose remained the same, and (4) discontinuation, after at least 6 months, with abrupt interruption of cyclophosphamide and slow tapering of prednisone. The results were assessed in terms of remissio n, improvement, progression of disease, kidney failure, and death, una mbiguously defined. The follow-up extended up to 12 years. Results: Se ven of eight patients had a complete remission that was maintained ind efinitely thereafter. Plasma creatinine levels decreased on average fr om 211 +/- 81 to 92 +/- 27 mu mol/L (P < 0.01) and urine protein excre tion decreased from 1.9 +/- 0.8 to 0.3 +/- 0.1 gm/day (P < 0.01). One patient died of intestinal infarction caused by atherosclerotic mesent eric artery thrombosis. Conclusions: Our data suggest that an intensiv e immunosuppressive regimen that combines prednisone and cyclophospham ide at high doses can be effective in healing Henoch-Schonlein disease .