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Review
. 2023 Dec 13;8(6):e237.
doi: 10.1097/j.pbj.0000000000000237. eCollection 2023 Nov-Dec.

ANCA-associated vasculitis: overview and practical issues of diagnosis and therapy from a European perspective

Ana Catarina Duarte  1 , Rui Ribeiro  2   3 , Ana M Macedo  3   4 , Maria José Santos  1   5
Affiliations
Review

ANCA-associated vasculitis: overview and practical issues of diagnosis and therapy from a European perspective

Ana Catarina Duarte et al. Porto Biomed J. .

Abstract

Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a heterogeneous group of rare diseases characterized by necrotizing inflammation predominantly of small vessels and the presence of these circulating antibodies. AAV comprises three important diseases, namely granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis, which affect multiple organ systems, significantly affecting patients' quality of life and survival. The diagnosis is established according to the clinical manifestations, detectable ANCA, and histopathology findings. Primary treatment strategies are adapted to the severity of the disease and based on immunosuppression with corticosteroids and cyclophosphamide, with increasing adoption of new, less toxic agents aimed at sustained remission of the disease, such as rituximab, methotrexate, and mycophenolate mofetil. Several international medical organizations have proposed recommendations for diagnosing and managing these diseases to standardize the procedures. In this study, we provide an up-to-date European perspective on AAV management, compiling current and relevant information regarding its epidemiology, symptoms, diagnosis, treatment strategies, and prognosis.

Keywords: AAV; ANCA; immunosuppressants; treatment; vasculitis.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1.
Figure 1.
General diagram for diagnosis of ANCA-associated vasculitis., CT, computed tomography; CXR, chest x-ray; EGPA, eosinophilic granulomatosis with polyangiitis; MPA, microscopic polyangiitis.
Figure 2.
Figure 2.
General recommendations for the therapeutic management of ANCA-associated vasculitis, according to disease (adapted from Hellmich et al). AZA, azathioprine, CYC, cyclophosphamide, GC, corticosteroids, GPA, granulomatosis with polyangiitis, EGPA, eosinophilic granulomatosis with polyangiitis, MEPO, mepolizumab, MPA, microscopic polyangiitis, MTX, methotrexate, PLEX, plasmapheresis, RTX, rituximab. *Adjuvant prophylaxis with cotrimoxazole or trimethoprim/sulfamethoxazole should be administered.
See this image and copyright information in PMC

References

    1. Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med. 1997;337(21):1512–23. - PubMed
    1. Jennette JC, Falk RJ, Bacon PA, et al. . 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum. 2013;65(1):1–11. - PubMed
    1. Finkielman JD, Lee AS, Hummel AM, et al. . ANCA are detectable in nearly all patients with active severe Wegener's granulomatosis. Am J Med. 2007;120(7):643 e9–14. - PubMed
    1. Jennette JC, Falk RJ, Andrassy K, et al. . Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994;37(2):187–92. - PubMed
    1. Kitching AR, Anders HJ, Basu N, et al. . ANCA-associated vasculitis. Nat Rev Dis Primers. 2020;6(1):71. - PubMed