Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2024 May;295(5):651-667.
doi: 10.1111/joim.13777. Epub 2024 Mar 11.

Significance of clinical-immunological patterns and diagnostic yield of biopsies in microscopic polyangiitis and granulomatosis with polyangiitis

João Fernandes-Serodio  1   2 , Sergio Prieto-González  1 , Georgina Espígol-Frigolé  1 , Roberto Ríos-Garcés  1 , Verónica Gómez-Caverzaschi  1 , Olga Araújo  1 , Gerard Espinosa  1 , Raül Jordà-Sánchez  1 , Marco A Alba  1 , Luis Quintana  3 , Miquel Blasco  3 , Elena Guillen  3 , Odette Viñas  4 , Estíbaliz Ruiz-Ortiz  4 , Laura Pelegrín  5 , Maite Sainz de la Maza  5 , Bernardo Sánchez-Dalmau  5 , Adriana García-Herrera  6 , Manel Solé  6 , Paola Castillo  6 , Iban Aldecoa  6 , María D Cano  6 , Jacobo Sellarés  7 , Fernanda Hernández-González  7 , Carlos Agustí  7 , Carmen M Lucena  7 , Antonio López-Rueda  8 , Marcelo Sánchez  9 , Mariana Benegas  9 , Sebastián Capurro  9 , Raimon Sanmartí  10 , Josep M Grau  11 , Isabel Vilaseca  12 , Isam Alobid  12 , Maria C Cid  1 , José Hernández-Rodríguez  1
Affiliations
Free article

Significance of clinical-immunological patterns and diagnostic yield of biopsies in microscopic polyangiitis and granulomatosis with polyangiitis

João Fernandes-Serodio et al. J Intern Med. 2024 May.
Free article

Abstract

Background: Microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) are the two major antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

Objectives: To characterize a homogenous AAV cohort and to assess the impact of clinicopathological profiles and ANCA serotypes on clinical presentation and prognosis. Clinical differences in GPA patients according to ANCA serotype and the diagnostic yield for vasculitis of biopsies in different territories were also investigated.

Results: This retrospective study (2000-2021) included 152 patients with AAV (77 MPA/75 GPA). MPA patients (96.1% myeloperoxidase [MPO]-ANCA and 2.6% proteinase 3 [PR3]-ANCA) presented more often with weight loss, myalgia, renal involvement, interstitial lung disease (ILD), cutaneous purpura, and peripheral nerve involvement. Patients with GPA (44% PR3-ANCA, 33.3% MPO, and 22.7% negative/atypical ANCA) presented more commonly with ear, nose, and throat and eye/orbital manifestations, more relapses, and higher survival than patients with MPA. GPA was the only independent risk factor for relapse. Poor survival predictors were older age at diagnosis and peripheral nerve involvement. ANCA serotypes differentiated clinical features in a lesser degree than clinical phenotypes. A mean of 1.5 biopsies were performed in 93.4% of patients in different territories. Overall, vasculitis was identified in 80.3% (97.3% in MPA and 61.8% in GPA) of patients.

Conclusions: The identification of GPA presentations associated with MPO-ANCA and awareness of risk factors for relapse and mortality are important to guide proper therapeutic strategies in AAV patients. Biopsies of different affected territories should be pursued in difficult-to-diagnose patients based on their significant diagnostic yield.

Keywords: ANCA‐associated vasculitis; biopsy diagnostic yield; granulomatosis with polyangiitis; microscopic polyangiitis; mortality; relapse.

PubMed Disclaimer

Similar articles

See all similar articles

Cited by

References

    1. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised international chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65:1–11. https://doi.org/10.1002/art.37715.
    1. Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116:488–498. https://doi.org/10.7326/0003–4819‐116–6–488.
    1. Schirmer JH, Wright MN, Vonthein R, Herrmann K, Nolle B, Both M, et al. Clinical presentation and long‐term outcome of 144 patients with microscopic polyangiitis in a monocentric German cohort. Rheumatology (Oxford). 2016;55:71–79. https://doi.org/10.1093/rheumatology/kev286.
    1. Turgeon D, Balter MS, Pagnoux C. Interstitial lung disease in patients with antineutrophil cytoplasm antibody‐associated vasculitis: an update on pathogenesis and treatment. Curr Opin Pulm Med. 2023;29:436–442. https://doi.org/10.1097/MCP.0000000000000979.
    1. Comarmond C, Crestani B, Tazi A, Hervier B, Adam‐Marchand S, Nunes H, et al. Pulmonary fibrosis in antineutrophil cytoplasmic antibodies (ANCA)‐associated vasculitis: a series of 49 patients and review of the literature. Medicine (Baltimore). 2014;93:340–349. https://doi.org/10.1097/MD.0000000000000217.

Publication types

MeSH terms

Substances