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Report on intensive treatment of extracapillary glomerulonephritis with focus on crescentic IgA nephropathy
D Roccatello1, M Ferro, R Coppo
1Divisione di Nefrologia e Dialisi dell'Ospedale G. Bosco, Torino, Italy.
Insights
Intensive treatment for IgA glomerulonephritis (IgAGN) showed initial clinical benefits but failed to reverse crescents. This IgAGN treatment may delay dialysis but is less effective than in vasculitis-associated glomerulonephritis.
Area of Science:
- Nephrology
- Immunology
- Glomerular Diseases
Background:
- Crescentic IgA glomerulonephritis (IgAGN) is a severe form of kidney disease.
- Histological criteria for IgAGN include >40% glomerular crescents.
- Treatment options for severe IgAGN are limited.
Purpose of the Study:
- To evaluate the efficacy of combined steroid pulses, cyclophosphamide, and plasma exchange in IgAGN.
- To compare treatment outcomes in IgAGN with other forms of crescentic glomerulonephritis.
Main Methods:
- Retrospective analysis of six IgAGN patients treated with a 2-month protocol.
- Standardized treatment: methylprednisolone boli, oral prednisone, oral cyclophosphamide, and plasma exchange.
- Comparison with three untreated IgAGN patients and 12 vasculitis patients.
Main Results:
- Combined therapy led to initial clinical improvement in IgAGN and vasculitis patients.
- Persistent florid crescents were observed in IgAGN post-treatment.
- Long-term follow-up showed loss of clinical improvement in half of treated IgAGN cases, though dialysis was delayed.
Conclusions:
- Short-term reversal of crescents is less likely in IgAGN compared to vasculitis.
- Intensive treatment can arrest, but not reverse, active lesions in IgAGN before chronicity.
- Further research is needed to optimize IgAGN treatment strategies.
Unlabelled:
PURPOSE AND DESIGN OF STUDY: In this retrospective analysis the effects of combined treatment with steroid pulses, cyclophosphamide and plasma exchange on six crescentic IgA glomerulonephritis (IgAGN) patients, selected on a histological basis, were examined. The histological criteria included involvement of more than 40% of glomeruli by cellular crescents. The effects of this treatment were compared to those observed in three untreated crescentic IgAGN patients and 12 treated patients who had extracapillary glomerulonephritis of different origins, i.e. ANCA-associated systemic or renal-limited vasculitis. All patients, except the three crescentic untreated IgAGN patients, received the same 2-month treatment according to a standardized protocol: steroid boli 15 mg/kg methylprednisolone for 3 consecutive days by intravenous infusion, followed by prednisone per 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 therapy, substantial clinical improvement was observed in both IgAGN and vasculitis patients. However, a second biopsy revealed that florid crescents persisted in IgAGN patients and, unlike the vasculitis group, during the long-term the initial clinical amelioration disappeared in one-half of the treated IgAGN cases. Nevertheless, even in the progressive 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 in crescentic IgAGN than in vasculitis-associated crescentic GN. Intensive treatment seems sufficient to arrest, but inadequate to reverse, phlogistic lesions in IgAGN before development of chronic changes.