Comparative study of granulomatosis with polyangiitis subsets according to ANCA status: data from the French Vasculitis Study Group Registry Article Swipe
Xavier Puéchal
,
Michele Iudici
,
Christian Pagnoux
,
Pascal Cohen
,
M. Hamidou
,
Achille Aouba
,
François Lifermann
,
M. Ruivard
,
O. Aumaître
,
Bernard Bonnotte
,
F. Maurier
,
Thomas Le Gallou
,
É. Hachulla
,
Alexandre Karras
,
C. Khouatra
,
Noémie Jourde‐Chiche
,
Jean‐François Viallard
,
Claire Blanchard‐Delaunay
,
Pascal Godmer
,
Alain Le Quellec
,
T. Quéméneur
,
Claire de Moreuil
,
Luc Mouthon
,
Benjamin Terrier
,
Loı̈c Guillevin
·
YOU?
·
· 2022
· Open Access
·
· DOI: https://doi.org/10.1136/rmdopen-2021-002160
YOU?
·
· 2022
· Open Access
·
· DOI: https://doi.org/10.1136/rmdopen-2021-002160
Objective To investigate whether antineutrophil cytoplasm antibody (ANCA)-negative and myeloperoxidase (MPO)-ANCA–positive granulomatosis with polyangiitis (GPA) differ from proteinase-3 (PR3)-ANCA–positive GPA. Methods Diagnostic characteristics and outcomes of newly diagnosed French Vasculitis Study Group Registry patients with ANCA-negative, MPO-ANCA–positive or PR3-ANCA–positive GPA satisfying American College of Rheumatology criteria and/or Chapel Hill Conference Consensus Nomenclature were compared. Results Among 727 GPA, 62 (8.5%) were ANCA-negative, 119 (16.4%) MPO-ANCA–positive and 546 (75.1%) PR3-ANCA–positive. ANCA-negative patients had significantly (p<0.05) more limited disease (17.7% vs 5.8%) and less kidney involvement (35.5% vs 58.9%) than those PR3-ANCA–positive or MPO-ANCA–positive, with comparable relapse-free (RFS) and overall survival (OS). MPO-ANCA–positive versus PR3-ANCA–positive and ANCA-negative patients were significantly more often female (52.9% vs 42.1%), older (59.8 vs 51.9 years), with more frequent kidney involvement (65.5% vs 55.2%) and less arthralgias (34.5% vs 55.1%), purpura (8.4% vs 17.1%) or eye involvement (18.5% vs 28.4%); RFS was similar but OS was lower before age adjustment. PR3-positive patients’ RFS was significantly lower than for ANCA-negative and MPO-positive groups combined, with OS higher before age adjustment. PR3-ANCA–positivity independently predicted relapse for all GPA forms combined but not when comparing only PR3-ANCA–positive versus MPO-ANCA–positive patients. Conclusions Based on this large cohort, ANCA-negative versus ANCA-positive patients more frequently had limited disease but similar RFS and OS. MPO-ANCA–positive patients had similar RFS but lower OS due to their older age. PR3-ANCA–positive GPA patients’ RFS was lower than those of the two other subsets combined but that difference did not persist when comparing only PR3 versus MPO-ANCA–positive patients.
Related Topics
Concepts
Medicine
Proteinase 3
Internal medicine
Granulomatosis with polyangiitis
Microscopic polyangiitis
Anti-neutrophil cytoplasmic antibody
Rheumatology
Gastroenterology
Vasculitis
Cohort
Disease
Metadata
- Type
- article
- Language
- en
- Landing Page
- https://doi.org/10.1136/rmdopen-2021-002160
- https://rmdopen.bmj.com/content/rmdopen/8/1/e002160.full.pdf
- OA Status
- gold
- Cited By
- 24
- References
- 38
- Related Works
- 10
- OpenAlex ID
- https://openalex.org/W4220935597
All OpenAlex metadata
Raw OpenAlex JSON
- OpenAlex ID
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https://openalex.org/W4220935597Canonical identifier for this work in OpenAlex
- DOI
-
https://doi.org/10.1136/rmdopen-2021-002160Digital Object Identifier
- Title
-
Comparative study of granulomatosis with polyangiitis subsets according to ANCA status: data from the French Vasculitis Study Group RegistryWork title
- Type
-
articleOpenAlex work type
- Language
-
enPrimary language
- Publication year
-
2022Year of publication
- Publication date
-
2022-03-01Full publication date if available
- Authors
-
Xavier Puéchal, Michele Iudici, Christian Pagnoux, Pascal Cohen, M. Hamidou, Achille Aouba, François Lifermann, M. Ruivard, O. Aumaître, Bernard Bonnotte, F. Maurier, Thomas Le Gallou, É. Hachulla, Alexandre Karras, C. Khouatra, Noémie Jourde‐Chiche, Jean‐François Viallard, Claire Blanchard‐Delaunay, Pascal Godmer, Alain Le Quellec, T. Quéméneur, Claire de Moreuil, Luc Mouthon, Benjamin Terrier, Loı̈c GuillevinList of authors in order
- Landing page
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https://doi.org/10.1136/rmdopen-2021-002160Publisher landing page
- PDF URL
-
https://rmdopen.bmj.com/content/rmdopen/8/1/e002160.full.pdfDirect link to full text PDF
- Open access
-
YesWhether a free full text is available
- OA status
-
goldOpen access status per OpenAlex
- OA URL
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https://rmdopen.bmj.com/content/rmdopen/8/1/e002160.full.pdfDirect OA link when available
- Concepts
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Medicine, Proteinase 3, Internal medicine, Granulomatosis with polyangiitis, Microscopic polyangiitis, Anti-neutrophil cytoplasmic antibody, Rheumatology, Gastroenterology, Vasculitis, Cohort, DiseaseTop concepts (fields/topics) attached by OpenAlex
- Cited by
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24Total citation count in OpenAlex
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2025: 6, 2024: 9, 2023: 6, 2022: 3Per-year citation counts (last 5 years)
- References (count)
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38Number of works referenced by this work
- Related works (count)
-
10Other works algorithmically related by OpenAlex
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