REPORT ON INTENSIVE TREATMENT OF EXTRACAPILLARY GLOMERULONEPHRITIS WITH FOCUS ON CRESCENTIC IGA NEPHROPATHY

Purpose and design of study. In this retrospective analysis the effect s of combined treatment with steroid pulses, cyclophosphamide and plas ma exchange on six crescentic IgA glomerulonephritis (IgAGN) patients, selected on a histological basis, were examined. The histological cri teria included involvement of more than 40% of glomeruli by cellular c rescents. The effects of this treatment were compared to those observe d in three untreated crescentic IgAGN patients and 12 treated patients who had extracapillary, glomerulo-nephritis of different origins, i.e . ANCA-associated systemic or renal-limited vasculitis. All patients, except the three crescentic untreated IgAGN patients, received the sam e 2-month treatment according to a standardized protocol: steroid boli 15 mg/kg methylprednisolone for 3 consecutive days by intravenous inf usion, followed by prednisone pei os (1 mg/kg/day for 4 weeks, 0.75 mg /kg/day for 4 more weeks), cyclophosphamide per os 2.5 mg/kg/day for 8 weeks, and plasma exchange. Results. After this 2-month course of the rapy, substantial clinical improvement was observed in both IgAGN and vasculitis patients. However, a second biopsy revealed that florid cre scents persisted in IgAGN patients and, unlike the vasculitis group, d uring the long-term the initial clinical amelioration disappeared in o ne-half of the treated IgAGN cases. Nevertheless, even in the progress ive cases, intensive treatment seemed to substantially delay the onset of dialysis. Conclusions. Despite some clinical benefits of therapy, short-term reversal of active crescents appears less likely to occur i n crescentic IgAGN than in vasculitis-associated crescentic GN. Intens ive treatment seems sufficient to arrest, but inadequate to reverse, p hlogistic lesions in IgAGN before development of chronic changes.