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Type: Original Article
Title: Kidney pathology and outcomes in ANCA-associated vasculitis
Authors: Elena V. Zakharova1, Tatyana A. Makarova1, Eugenia S. Leonova1, Elena N. Nikitina1, Ekaterina S. Stolyarevich2
Affiliations: 1Nephrology, Clinical City Hospital n.a. S.P. Botkin, Moscow, Russian Federation.
2Pathology, City Nephrology Center, Moscow, Russian Federation.
Aim: The frequency of kidney involvement in ANCA-associated vasculitis (AAV) varies from 90% in Microscopic Polyangiitis (MPA) and 80% in Granulomatosis with Poliangiitis (GPA) to 45% in Eosinophilic Granulomatosis with Poliangiitis (EGPA). Renal outcomes are mainly determined by serum creatinine (SCr) level and the need for dialysis at the time of diagnosis, and pathology findings, such as the number of normal and totally sclerosed glomeruli, and the presence of active lesions with cellular crescents and fibrinoid necrosis or chronic lesions with fibrous crescents. The current histopathologic classification of ANCA-associated glomerulonephritis distinguishes focal, crescentic, mixed and sclerosing classes with different kidney survival. We aim to evaluate pathology data in our cohort of patients with AAV according to this classification, and compare kidney survival in different classes.
Methods: Using clinical and pathology electronic database, we will search charts of all patients with AAV with biopsy-proven kidney involvement, who received immunosuppressive treatment in nephrology unit of Moscow City Clinical Hospital n.a. S.P. Botkin in 2000-2020. We expect to retrieve data for at least 60 patients, and evaluate kidney histopathology findings, serum creatinine and need for dialysis at the time of kidney biopsy and at the latest follow-up visit, and intend to compare kidney function and kidney survival between the different classes.
Results: The preliminary analysis of 40 cases, 16 males and 24 females, with the median age of 36.5 [15; 80], and 17 of them with MPA, 18 with GPA and 5 with EGPA showed 79% had 3-year estimated overall kidney survival. In the focal class 3-year estimated kidney survival is 100%, in the crescentic class – 87.5%, in the mixed class - 78%, and in the sclerotic class - 68% (Figure 1). Of note, in the crescentic class, kidney survival is as high as 87.5% despite the highest serum creatinine level and highest number of dialysis-dependent patients at the time of biopsy, and 3 out of 4 dialysis-dependent patients became dialysis-free under treatment (Table 1).
Conclusion: so far, we have concluded that the best kidney outcome is associated with the focal class of ANCA-associated glomerulonephritis, the worst – with the sclerosing class, which corresponds with the published data. In the crescentic class, timely kidney biopsy treatment improves kidney outcome in the majority of cases.