Granulomatosis with Polyangiitis: Data from the French Vasculitis Study Group Registry

Michele Iudici¹, Christian Pagnoux ², Delphine Courvoisier ³, Pascal Cohen ⁴, Mohamed Hamidou ⁵, Achille Aouba ⁶, François Lifermann ⁷, Marc Ruivard ⁸, Olivier Aumaitre ⁹, Bernard Bonnotte ¹⁰, François Maurier ¹¹, Olivier Decaux ¹², Eric Hachulla ¹³, Alexandre Karras ¹⁴, Chahéra Khouatra ¹⁵, Noémie Jourde-Chiche ¹⁶, Jean-François Viallard ¹⁷, Claire Blanchard-Delaunay ¹⁸, Pascal Godmer ¹⁹, Alain Le Quellec ²⁰, Thomas Quémeneur ²¹, Claire de Moreuil ²², Alexis Régent ⁴, Benjamin Terrier ⁴, Luc Mouthon ⁴, Loic Guillevin ⁴ and Xavier Puéchal for the French Vasculitis Study Group ⁴, ¹National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, France and Division of Rheumatology, Department of Internal Medicine Specialties, Geneva University Hospitals, Switzerland, Geneva, Geneve, Switzerland, ²National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin and Toronto Canada, Paris, France, ³Division of Rheumatology, Geneva University Hospital, Switzerland, Geneva, Switzerland, ⁴National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, Paris, France, ⁵CHU Nantes, Nantes, France, ⁶CHU Caen, Caen, France, ⁷CH Dax, Dax, France, ⁸CHU Clermont-Ferrand, Clermont-Ferrand, France, ⁹CHU Clermont Ferrand, Clermont Ferrand, France, ¹⁰Service de Médecine Interne et Immunologie Clinique, CHU Dijon Bourgogne, Hôpital François Mitterrand, Dijon ; Université Bourgogne-Franche Comté, INSERM, EFS BFC, UMR1098, F-21000 Dijon, Dijon, France, ¹¹Service de Médecine Interne, Hôpital Belle Isle, Metz, Metz, France, ¹²CHU Rennes, Rennes, France, ¹³CHU Lille, Lille, France, ¹⁴Paris HEGP, Paris, France, ¹⁵CHU Lyon, Lyon, France, ¹⁶APHM, Marseille, Marseille, France, ¹⁷CHU Bordeaux, Bordeaux, France, ¹⁸CH Niort, Niort, France, ¹⁹CH Bretagne-Atlantique, Vannes, France, ²⁰CHU Montpellier, Montpellier, France, ²¹CH Valenciennes, Valenciennes, France, ²²CHU Brest, Brest, France

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: Wegener's granulomatosis and systemic vasculitides

Session Information

Date: Monday, November 11, 2019

Title: Vasculitis – ANCA-Associated Poster II

Session Type: Poster Session (Monday)

Session Time: 9:00AM-11:00AM

Background/Purpose: The long-term features of granulomatosis with polyangiitis (GPA), a systemic small-vessel ANCA-associated necrotizing vasculitis, have been mainly reported in small patient series. The aim of this study was to describe the main characteristics and long-term outcomes of patients entered in the nationwide French Vasculitis Study Group (FVSG) database.

Methods: Clinical and laboratory characteristics of patients with GPA, satisfying 1990 ACR criteria and/or revised Chapel Hill Nomenclature, enrolled in the FVSG cohort were collected. All patients had ANCA detection by immunofluorescence and/or ELISA determination of ANCA specificity at diagnosis or during follow-up. Estimated patients’ overall and relapse-free survival rates were analyzed by Kaplan–Meier curves and Cox regression analysis.

Results: Between May 1983 and April 2018, 795 GPA patients were entered in the Registry: 181 (23%) before 2000, 419 (53%) 2000–2010 and 195 (24%) after 2010. They were followed for a mean ± SD of 4.6 ± 3.9 years. At diagnosis, mean ± SD age was 53 ± 16 years, with 52 (7%) >75 years old; their main clinical manifestations were: fever >38°C (43%); weight loss (45%); arthralgias/arthritis (52%); ENT involvement (80%), including rhinitis (54%), sinusitis (42%) and/or otitis (23%); lung involvement (68%), including nodules (41%) and alveolar hemorrhage (18%); neurologic (29%) involvement, including peripheral (21%) and central (10%) manifestations; and renal (56%) involvement. Median creatinine at diagnosis was 93 µmol/L, when 177/609 (29%) patients’ had levels >150 µmol/L; between baseline and diagnosis those levels had already risen >30% for 193 (24%) patients. At diagnosis, 13 (2%) patients had subglottic stenosis; 55 (7%) had GPA limited to ENT and/or lungs (localized). Among the 728 available ELISA results, 546 (75.0%) patients were anti-PR3+ and 120 (16.5%) anti-MPO. Mean BVAS was 17.4 ± 8.8. To induce remission, glucocorticoids (GCs) were prescribed for 772 (97%) patients (median dose 60 [IQR 50–70] mg/day), combined with intravenous (76%) or oral (7%) cyclophosphamide, rituximab (6%) or methotrexate (4.5%). Among 729 (92%) patients achieving remission, 394 (54%) relapsed a mean of 2.7 ± 2.2 years after diagnosis. Kaplan–Meier estimated 5- and 10-year relapse-free survival rates, respectively, were 37% and 17% for the entire cohort, 35% and 14% for PR3+ patients, and 46% and 25% for those MPO+ (P=0.11). Univariable analysis identified PR3 positivity (HR 1.30; P=0.04) as the only factor associated with relapse, while oral cyclophosphamide use lowered relapse probability (HR 0.60; P=0.05). Multivariable analysis retained PR3 positivity (HR 1.29; P=0.05) as the only factor independently associated with relapse. Five- and 10-year overall survival rates were 90% and 85%, respectively. Among the 90 main severe side effects were 34 (4%) cancers, 32 (4%) opportunistic infections and 20 (3%) cardiovascular events.

Conclusion: Findings based on this large series showed good survival of GPA patients, with a low rate of side effects, and confirmed the higher probability of relapse among those PR3-ANCA+ at diagnosis.

Disclosure: M. Iudici, None; C. Pagnoux, None; D. Courvoisier, None; P. Cohen, None; M. Hamidou, Roche, 8; A. Aouba, None; F. Lifermann, None; M. Ruivard, None; O. Aumaitre, None; B. Bonnotte, None; F. Maurier, None; O. Decaux, None; E. Hachulla, None; A. Karras, Roche, 8; C. Khouatra, None; N. Jourde-Chiche, None; J. Viallard, None; C. Blanchard-Delaunay, None; P. Godmer, None; A. Le Quellec, None; T. Quémeneur, None; C. de Moreuil, None; A. Régent, None; B. Terrier, Grifols, 8, GSK, 8, LFB, 8, Roche, 8; L. Mouthon, None; L. Guillevin, None; X. Puéchal for the French Vasculitis Study Group, LFB, 8, Pfizer, 2, 8, Roche, 8.

To cite this abstract in AMA style:

Iudici M, Pagnoux C, Courvoisier D, Cohen P, Hamidou M, Aouba A, Lifermann F, Ruivard M, Aumaitre O, Bonnotte B, Maurier F, Decaux O, Hachulla E, Karras A, Khouatra C, Jourde-Chiche N, Viallard J, Blanchard-Delaunay C, Godmer P, Le Quellec A, Quémeneur T, de Moreuil C, Régent A, Terrier B, Mouthon L, Guillevin L, Puéchal for the French Vasculitis Study Group X. Granulomatosis with Polyangiitis: Data from the French Vasculitis Study Group Registry [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/granulomatosis-with-polyangiitis-data-from-the-french-vasculitis-study-group-registry/. Accessed .

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