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Journal Articles Rheumatology Year : 2022

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

Michele Iudici
Christian Pagnoux
  • Function : Author
Delphine Courvoisier
Pascal Cohen
  • Function : Author
Antoine Néel
  • Function : Author
Achille Aouba
  • Function : Author
François Lifermann
  • Function : Author
Marc Ruivard
  • Function : Author
Olivier Aumaître
  • Function : Author
Bernard Bonnotte
  • Function : Author
François Maurier
  • Function : Author
Thomas Le Gallou
  • Function : Author
Alexandre Karras
  • Function : Author
Chahéra Khouatra
  • Function : Author
Jean-François Viallard
  • Function : Author
Claire Blanchard-Delaunay
  • Function : Author
Pascal Godmer
  • Function : Author
Alain Le Quellec
  • Function : Author
Thomas Quéméneur
  • Function : Author
Claire de Moreuil
  • Function : Author
Alexis Régent
  • Function : Author
Benjamin Terrier
  • Function : Author
Luc Mouthon
  • Function : Author
Loïc Guillevin
  • Function : Author
Xavier Puéchal

Abstract

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.
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Dates and versions

hal-03800939 , version 1 (06-10-2022)

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Michele Iudici, Christian Pagnoux, Delphine Courvoisier, Pascal Cohen, Antoine Néel, et al.. Localized versus systemic granulomatosis with polyangiitis: data from the French Vasculitis Study Group Registry. Rheumatology, 2022, 61 (6), pp.2464-2471. ⟨10.1093/rheumatology/keab719⟩. ⟨hal-03800939⟩
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