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Topic: Update on ANCA-Associated Vasculitis: Towards the Best Stratification in Different Phenotypes by Genetics and Pathogenesis, Clinical Features and Treatment Options

A special issue of Vessel Plus

ISSN 2574-1209 (Online)

Submission deadline: 28 Feb 2021

Guest Editor(s)

  • Prof. Luca Quartuccio, MD, PhD
    Clinic of Rheumatology, Department of Medicine (DAME), ASUFC, University of Udine, Udine, Italy.

    Website | E-mail

Special Issue Introduction

From the original description of ANCAs by Davies et al. in 1982 in segmental necrotising glomerulonephritis, and the discovery of the antigens MPO and PR3 in 1988 and 1989, respectively, over 30 years have passed in which many efforts by clinicians from different medical areas have been made to greatly increase the amount of knowledge in ANCA-associated vasculitides (AAV). Now, on the horizon we can see clear differences emerging on genetic and pathogenic features that have crucial reflexes on clinical manifestations and, of major value, on treatments. Many clinical trials have been performed so far to clarify both the best induction and the best maintenance therapy in different subgroups of disease, and they have dramatically improved the outcome of patients affected by AAV. Recently, B-cell targeted therapy and complement activation inhibition strategy are still improving the management of AAV. Nevertheless, AAV remain difficult-to-treat diseases, very costly diseases even if they are rare, and many clinicians from different specialties are encouraged to put together forces for further proceeding into the best characterization of these diseases and, of consequence, the best treatment and outcome. Therefore, the scope of this special issue is to collect some important news regarding AAV from pathogenesis to treatment and outcome to improve the management of them in clinical practice.


Vasculitis, anti-neutrophil cytoplasmic antibody, B cell, complement, glucocorticoids, rituximab

Submission Deadline

28 Feb 2021

Submission Information

For Author Instructions, please refer to http://vpjournal.net/pages/view/author_instructions
For Online Submission, please login at https://oaemesas.com/login?JournalId=vp&SpecialIssueId=492
Submission Deadline: 28 Feb 2021
Contacts: Grace Zheng, Assistant Editor, grace@vpjournal.net

Planned Papers

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.

Published Articles

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