Clinical and morphological characteristics and prognosis of different histomorphological variants of rapidly progressing glomerulonephritis associated with ANCA-vasculitis

Background: anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is the most common cause of rapidly progressing glomerulonephritis (RPGN). Its prognosis has improved, however long-term patient morbidity and mortality still remain relatively high due to toxic effects of immunosuppressive treatment. Therefore, a search for optimal predictors of long-term outcome of this disease is of particular importance. Aim: to evaluate the short- and long-term outcomes of AAV-RPGN and their dependence the histopathologic classification. Materials and Methods: 115 biopsy-proven AAV-RPGN patients, aged 56±14.3 years (40% older than 60), were included in the retrospective study. The serum creatinine level by the time of presentation was 570 [380; 830] µmol/L. According to histopathology, the patients were divided into 4 groups: focal (22 pts), crescentic (34 pts), sclerotic (33 pts), and mixed (29 pts) RPGN. Induction therapy was carried out with methylprednisolone and cyclophosphamide IV pulses followed by oral prednisolone. Rituximab was used in 21 patients. Maintenance therapy was carried out with prednisolone and azathioprine or MMF. Results: improvement was observed in 77% of patients: 100% in group with focal, 80% in mixed, 57% in crescentic, and 52% in sclerotic RPGN. Five-years patient survival rate was 67.7%. It decreased to 38% in the group of patients older than 60 years versus 80% in the younger group. Five-years renal survival was 59% and was closely associated with the categories of glomerular lesions: 89% in group with focal RPGN, 78% in mixed RPGN, 56% in crescentic, and 34% in sclerotic RPGN (p<0.05). Conclusion: our results show that histopathologic category of glomerular lesions in AAV-RPGN has predictive value for renal outcome.

быстропрогрессирующий гломерулонефрит, АНЦА-ассоциированный васкулит, гистоморфологический вариант, ближайшие и отдаленные результаты лечения, anti-neutrophil cytoplasmic antibody-associated vasculitis, rapidly progressive glomerulonephritis, short-term and long-term outcomes, histopathologic category of glomerular lesions

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