Article Dans Une Revue RMD Open Année : 2025

Concordance between presenting features and relapse in granulomatosis with polyangiitis: implications for risk assessment and counselling

1 HUG - Hôpitaux Universitaires de Genève = University Hospital of Geneva [Genève]
2 UNIGE - Université de Genève = University of Geneva
3 Hôpital Cochin [AP-HP]
4 PhyMedExp - Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046]
5 CHU Nantes - Centre Hospitalier Universitaire de Nantes = Nantes University Hospital
6 U1064 Inserm - CR2TI - Centre de Recherche en Transplantation et Immunologie - Center for Research in Transplantation and Translational Immunology
7 CHU Caen Normandie - CHU Caen Normandie – Centre Hospitalier Universitaire de Caen Normandie
8 CH Dax - Centre Hospitalier Dax - Côte d'Argent
9 CHU Clermont-Ferrand
10 CHU Dijon - Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand
11 CHR Metz-Thionville - Centre hospitalier régional de Metz-Thionville
12 MOBIDIC - Microenvironment and B-cells: Immunopathology, Cell Differentiation, and Cancer
13 Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Pontchaillou]
14 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
15 INFINITE - Institute for Translational Research in Inflammation - U 1286
16 HEGP - Hôpital Européen Georges Pompidou [APHP]
17 UPCité - Université Paris Cité
18 Hôpital Louis Pradel [CHU - HCL]
19 UCBL - Université Claude Bernard Lyon 1
20 LA CONCEPTION - Hôpital de la Conception [CHU - APHM]
21 C2VN - Centre recherche en CardioVasculaire et Nutrition = Center for CardioVascular and Nutrition research
22 Hôpital Haut-Lévêque [CHU Bordeaux]
23 UB - Université de Bordeaux
24 CH Georges Renon Niort - Centre Hospitalier Georges Renon [Niort]
25 CHBA - Centre hospitalier Bretagne Atlantique (Morbihan)
26 CHV - Centre Hospitalier de Valenciennes
27 Hôpital de la Cavale Blanche
Claire de Moreuil
Benjamin Terrier

Résumé

Objective: To investigate the concordance between organ involvement at diagnosis and relapse in granulomatosis with polyangiitis and factors associated with new disease features at relapse. Methods: Data from a national database of newly diagnosed patients was analysed. Clinical features were recorded at diagnosis and relapse, grouped by organ system. ORs and HRs were used to assess associations between baseline features and first relapse. Factors independently associated with new organ involvement at relapse were identified using multivariable logistic regression. Results: Among 795 patients (median follow-up 3.5 years), 394 (50%) relapsed; organ involvement at relapse was available for 376 patients. Relapses most often affected ear, nose and throat (ENT), lungs and kidneys. Organ involvement at diagnosis was associated with a higher likelihood of relapse in the same organ: eyes (OR 6.69), lungs (OR 3.35), kidneys (OR 3.58), nervous system (OR 2.90), and mucocutaneous (OR 4.53). Major manifestations associated with a higher likelihood of recurrence were scleritis, pachymeningitis, subglottic stenosis and worsening renal function. For 56% of patients, the first relapse affected only the initially involved organs. Of the 165 patients with new organ manifestations, these were rarely isolated (n=34) and usually occurred alongside involvement of at least one previously affected organ (n=131). In multivariable analysis, systemic, ENT and lung manifestations at diagnosis were associated with a lower risk of new organ disease at relapse. Conclusion: Although new features can still emerge, organ involvement at diagnosis is associated with a higher likelihood of relapse in the same organ.

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hal-05437521 , version 1 (01-01-2026)
hal-05437521 , version 2 (17-02-2026)

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Michele Iudici, Zubeyir Salis, Pascal Cohen, Antoine Néel, Achille Aouba, et al.. Concordance between presenting features and relapse in granulomatosis with polyangiitis: implications for risk assessment and counselling. RMD Open, 2025, 11 (4), pp.e006434. ⟨10.1136/rmdopen-2025-006434⟩. ⟨hal-05437521v2⟩
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