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Article Dans Une Revue RMD Open : Rheumatic & Musculoskeletal Diseases Année : 2022

Comparative study of granulomatosis with polyangiitis subsets according to ANCA status: data from the French Vasculitis Study Group Registry

Xavier Puéchal
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Pascal Cohen
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Achille Aouba
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François Lifermann
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Marc Ruivard
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Olivier Aumaître
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Bernard Bonnotte
Francois Maurier
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Eric Hachulla
Alexandre Karras
Chahéra Khouatra
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Noémie Jourde-Chiche
Jean-François Viallard
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Claire Blanchard-Delaunay
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Pascal Godmer
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Thomas Quéméneur
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Claire de Moreuil
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Luc Mouthon
Benjamin Terrier

Résumé

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.
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Dates et versions

hal-03633811 , version 1 (30-05-2022)

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Paternité - Pas d'utilisation commerciale

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Xavier Puéchal, Michele Iudici, Christian Pagnoux, Pascal Cohen, Mohamed Hamidou, et al.. Comparative study of granulomatosis with polyangiitis subsets according to ANCA status: data from the French Vasculitis Study Group Registry. RMD Open : Rheumatic & Musculoskeletal Diseases, 2022, 8 (1), pp.e002160. ⟨10.1136/rmdopen-2021-002160⟩. ⟨hal-03633811⟩
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