[Introduction: Rapidly progressive glomerulonephritis is life-threatening manifestation of antineutrophil cytoplasmatic antibody (ANCA)-associated vasculitis (AAV), often diagnosed only in advanced stage of renal failure. In 2010 a new histopathologic classification of ANCA- associated vasculitis was published by an international working group of nephropathologists. Vasculitis cases were classified in four groups: focal, crescent, mixed and sclerotic. Method: The aim of our study was to re-evaluate the predictive value of this new classification regarding renal outcome. From the 88 patients with ANCA-associated vasculitis treated in our department from 1996 to 2013, 39 were involved. Results: By retrospective evaluation of biopsy samples, patients were classified as 11 in focal, 12 in crescent, eight in mixed, eight in sclerotic group. There was no significant difference among the four groups regarding the mean age, sex, ANCAtype and initial eGFR. Due to the treatment, the eGFR values significantly increased in the focal and in the crescent groups. Eleven patients needed dialysis at presentation and three of them recovered, none of them belonged to the sclerotic group. The cumulative renal response to treatment was 100% in the focal, 87.5% in the mixed, 64% in the crescent and 62% in the sclerotic group. Renal response at one year treatment was 80%, thirty-one of the thirty-nine patients were dialysis independent. All patients were alive at one year, by year five two patients from the sclerotic group died. Conclusion: The new nephropathological classification of AAV is useful in predicting the renal response to treatment. Nephropathology can optimize the system by mentioning the specific percentage of normal glomeruli in the biopsy specimen.]
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