Localized versus systemic granulomatosis with polyangiitis: data from the French Vasculitis Study Group Registry.

Abstract

OBJECTIVE:

To describe the main features at diagnosis and evolution over time of patients with localized granulomatosis with polyangiitis (L-GPA) compared with those of systemic GPA (S-GPA).

METHODS:

EULAR definitions of L-GPA, i.e. upper and/or lower respiratory tract involvement, and S-GPA were applied to patients from the French Vasculitis Study Group Registry. L-GPA and S-GPA patients' characteristics at diagnosis and long-term outcomes were analysed and compared.

RESULTS:

Among the 795 Registry patients, 79 (10%) had L-GPA. Their main clinical manifestations were rhinitis, lung nodules, sinusitis and otitis. L-GPA vs S-GPA patients at diagnosis, respectively, were younger, more frequently had saddle nose deformity or subglottic stenosis and were less often PR3-ANCA-positive. L-GPA vs S-GPA induction therapy less frequently included CYC but more often a combination of MTX and glucocorticoids; 64% of MTX-treated patients experienced disease progression within 18 months post-diagnosis. L- and S-GPA patients' estimated relapse-free-survival probabilities, relapse rates and refractory disease rates at each time point were comparable, but L-GPA patients had more frequent ENT and lung relapses, and higher overall survival rates (P<0.02). Over a median follow-up of 3.5 years, 18 (22.8%) L-GPA progressed to S-GPA, either as a relapse after a period in remission or more frequently in the context of refractory disease. L-GPA patients experienced more ENT-related damage.

CONCLUSIONS:

The relapse risks of L-GPA and S-GPA were similar, but relapse patterns differed and L-GPA overall survival rate was higher. About one-quarter of L-GPA patients developed S-GPA over time, but without end-stage organ involvement.

• Authors: Michele Iudici, Christian Pagnoux, Delphine S Courvoisier, Pascal Cohen, Antoine Néel, Achille Aouba, François Lifermann, Marc Ruivard, Olivier Aumaître, Bernard Bonnotte, François Maurier, Thomas Le Gallou, Eric Hachulla, Alexandre Karras, Chahéra Khouatra, Noémie Jourde-Chiche, Jean-François Viallard, Claire Blanchard-Delaunay, Pascal Godmer, Alain Le Quellec, Thomas Quéméneur, Claire de Moreuil, Alexis Régent, Benjamin Terrier, Luc Mouthon, Loïc Guillevin, Xavier Puéchal, French Vasculitis Study Group
• Journal: Rheumatology (Oxford, England) (Rheumatology (Oxford)) Vol. 61 Issue 6 Pg. 2464-2471 (05 30 2022) ISSN: 1462-0332 [Electronic] England
• PMID: 34542599 (Publication Type: Journal Article)
• Copyright: © The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: [email protected].

Chemical References

• Antibodies, Antineutrophil Cytoplasmic

Topics

• Antibodies, Antineutrophil Cytoplasmic
• Granulomatosis with Polyangiitis (diagnosis)
• Humans
• Recurrence
• Registries
• Retrospective Studies