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.
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 April 13, 2021.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/granulomatosis-with-polyangiitis-data-from-the-french-vasculitis-study-group-registry/