Evidence map›Paper›PMID 36109667›Full record

ReviewNature reviews. Rheumatology2022

Genetics of ANCA-associated vasculitis: role in pathogenesis, classification and management.

Giorgio Trivioli, Ana Marquez, Davide Martorana, Michelangelo Tesi, Andreas Kronbichler, Paul A Lyons, Augusto Vaglio

Abstract readReview
PubMed Publisher
In one paragraph

Review in Nature reviews. Rheumatology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 45 papers.

0numbers the graph read from it
0cells of the map it votes in
45citing papers in PubMed
10.6field-weighted citation impact, top 1% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

45 citing papers in PubMed, 74 citations in OpenAlex.

  1. Article
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  6. Immature leukocyte and plasma-induced cell death reveal subclinical immune activation in EGPA patients in remission.Inflammation research : official journal of the European Histamine Research Society ... [et al.] · 2026
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  11. PeerJ · 2026
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  20. A 28-Year-Old Man with Stridor and Dyspnea.Journal of clinical medicine · 2025
    Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

7 authors at 4 institutions in 3 countries.

Giorgio TrivioliDepartment of Biomedical Experimental and Clinical Sciences "Mario Serio", University of Florence, Florence, Italy.ORCID 0000-0003-1578-4402
Ana MarquezInstitute of Parasitology and Biomedicine "López-Neyra", CSIC, Granada, Spain.
Davide MartoranaMedical Genetics Unit, Department of Diagnostics, University Hospital of Parma, Parma, Italy.ORCID 0000-0002-7572-7031
Michelangelo TesiNephrology and Dialysis Unit, Meyer Children's Hospital, Florence, Italy.
Andreas KronbichlerDepartment of Medicine, University of Cambridge School of Clinical Medicine, Cambridge, UK.ORCID 0000-0002-2945-2946
Paul A LyonsDepartment of Medicine, University of Cambridge School of Clinical Medicine, Cambridge, UK.ORCID 0000-0001-7035-8997
Augusto VaglioDepartment of Biomedical Experimental and Clinical Sciences "Mario Serio", University of Florence, Florence, Italy. augusto.vaglio@unifi.it.ORCID 0000-0002-3814-9172
Meyer Children's Hospital · ITUniversity of Cambridge · GBInstituto de Parasitología y Biomedicina "López - Neyra" · ESUniversity of Parma · IT

Funding

British Heart Foundation PG/13/64/30435
6 · The paper itself

Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), that share features of pauci-immune small-vessel vasculitis and the positivity of ANCA targeting proteinase-3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA). AAV syndromes are rare, complex diseases and their aetio-pathogenesis is mainly driven by the interaction between environmental and genetic factors. In patients with GPA and MPA, the genetic associations are stronger with ANCA specificity (PR3- versus MPO-ANCA) than with the clinical diagnosis, which, in keeping with the known clinical and prognostic differences between PR3-ANCA-positive and MPO-ANCA-positive patients, supports an ANCA-based re-classification of these disorders. EGPA is also made up of genetically distinct subsets, which can be stratified on ANCA-status (MPO ANCA-positive versus ANCA-negative); these subsets differ in clinical phenotype and possibly in their response to treatment. Interestingly, MPO-ANCA-positive patients with either MPA or EGPA have overlapping genetic determinants, thus strengthening the concept that this EGPA subset is closely related to the other AAV syndromes. The genetics of AAV provides us with essential information to understand its varied phenotype. This Review discusses the main findings of genetic association studies in AAV, their pathogenic implications and their potential effect on classification, management and prognosis.

Indexed as

Anti-Neutrophil Cytoplasmic Antibody-Associated VasculitisChurg-Strauss SyndromeGranulomatosis with PolyangiitisMicroscopic PolyangiitisAntibodies, Antineutrophil CytoplasmicHumansMyeloblastinPeroxidaseAntibodies, Antineutrophil CytoplasmicMyeloblastinPeroxidase

Identifiers

PMID36109667
OpenAlexW4295923974

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.