Despite the availability of different classifications for rapidly progressive glomerulonephritis (RPGN), patients with 'idiopathic crescentic GN' have not been yet inserted as a precisely defined subgroup, pointing to their probable heterogenicity. Trying to better define their characteristic, we retrospectively analyzed the clinical, histological and immunopathological features of 41 patients diagnostically labelled 'idiopathic RPGN' because they had no evidence of systemic disease (including systemic vasculitis), no anti-GBM mediated glomerulonephritis and no clearly defined primary glomerulopathy. Starting by a thorough morphological review, 2 subgroups were defined: group I (25 patients) with variable degrees of intraglomerular necrosis, and group II (16 patients) with no intracapillary necrotizing lesions. Group I showed no or minimal endocapillary proliferation, intense interstitial infiltrates with periglomerular localization, frequent ruptures of Bowman's capsule and mild degree of glomerular and/or interstitial sclerosis. 16 patients in this group (64%) had irregular deposits of complement C3 at immunofluorescence while the remaining 9 (36%) had no immune deposits. Clinically they had no previous history of preceeding urinary abnormalities, had a mean of 1.8 g/day proteinuria and a positivity for ANCA in 92% (12/13). In group II there was frequently marked mesangial proliferation, scarce interstitial infiltrates, no ruptures of Bowman's capsule and marked degrees of glomerulosclerosis and interstitial fibrosis. All patients in this group had clearly defined immune deposits of C3 and/or IgG. Clinically 50% of these patients had a history of recurrent microhematuria and/or proteinuria, a mean of 4.5 g/day proteinuria and negativity for ANCA in all 8 patients tested. Despite having a comparable mean percentage of crescents and S. creatinine at the time of renal biopsy, both groups differed significantly in their response to almost the same therapy, with 18 patients (72%) in group I showing remarkable improvement (> 50% decrease in their S. creatinine), while 9 patients (57%) in group II showed no response to therapy and progressed to end-stage renal disease. We conclude that, 'idiopathic' RPGN can be in fact the outcome of one of two pathogenetic mechanisms: the first is an acute necrotizing inflammation, with many features in common with the systemic vasculitides and actually represents a form of 'renal-limited' vasculitis, while the other is the result of extracapillary proliferation acutely complicating an underlaying primary chronic glomerulopathy

Ferrario, F., Tadros, M., Napodano, P., Sinico, R., Fellin, G., D'Amico, G. (1994). Critical re-evaluation of 41 cases of "idiopathic" crescentic glomerulonephritis. CLINICAL NEPHROLOGY, 41(1), 1-9.

Critical re-evaluation of 41 cases of "idiopathic" crescentic glomerulonephritis

SINICO, RENATO ALBERTO;
1994

Abstract

Despite the availability of different classifications for rapidly progressive glomerulonephritis (RPGN), patients with 'idiopathic crescentic GN' have not been yet inserted as a precisely defined subgroup, pointing to their probable heterogenicity. Trying to better define their characteristic, we retrospectively analyzed the clinical, histological and immunopathological features of 41 patients diagnostically labelled 'idiopathic RPGN' because they had no evidence of systemic disease (including systemic vasculitis), no anti-GBM mediated glomerulonephritis and no clearly defined primary glomerulopathy. Starting by a thorough morphological review, 2 subgroups were defined: group I (25 patients) with variable degrees of intraglomerular necrosis, and group II (16 patients) with no intracapillary necrotizing lesions. Group I showed no or minimal endocapillary proliferation, intense interstitial infiltrates with periglomerular localization, frequent ruptures of Bowman's capsule and mild degree of glomerular and/or interstitial sclerosis. 16 patients in this group (64%) had irregular deposits of complement C3 at immunofluorescence while the remaining 9 (36%) had no immune deposits. Clinically they had no previous history of preceeding urinary abnormalities, had a mean of 1.8 g/day proteinuria and a positivity for ANCA in 92% (12/13). In group II there was frequently marked mesangial proliferation, scarce interstitial infiltrates, no ruptures of Bowman's capsule and marked degrees of glomerulosclerosis and interstitial fibrosis. All patients in this group had clearly defined immune deposits of C3 and/or IgG. Clinically 50% of these patients had a history of recurrent microhematuria and/or proteinuria, a mean of 4.5 g/day proteinuria and negativity for ANCA in all 8 patients tested. Despite having a comparable mean percentage of crescents and S. creatinine at the time of renal biopsy, both groups differed significantly in their response to almost the same therapy, with 18 patients (72%) in group I showing remarkable improvement (> 50% decrease in their S. creatinine), while 9 patients (57%) in group II showed no response to therapy and progressed to end-stage renal disease. We conclude that, 'idiopathic' RPGN can be in fact the outcome of one of two pathogenetic mechanisms: the first is an acute necrotizing inflammation, with many features in common with the systemic vasculitides and actually represents a form of 'renal-limited' vasculitis, while the other is the result of extracapillary proliferation acutely complicating an underlaying primary chronic glomerulopathy
Articolo in rivista - Articolo scientifico
Adult; Aged; Antibodies, Antineutrophil Cytoplasmic; Autoantibodies; Blood Pressure; Complement C3; Creatinine; Female; Fluorescent Antibody Technique; Follow-Up Studies; Glomerular Mesangium; Glomerulonephritis; Humans; Male; Middle Aged; Retrospective Studies
English
1994
41
1
1
9
none
Ferrario, F., Tadros, M., Napodano, P., Sinico, R., Fellin, G., D'Amico, G. (1994). Critical re-evaluation of 41 cases of "idiopathic" crescentic glomerulonephritis. CLINICAL NEPHROLOGY, 41(1), 1-9.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10281/139484
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