Session Type: Abstract Session
Session Time: 11:00AM-11:50AM
Background/Purpose: The clinical picture at onset and evolution of localized forms of granulomatosis with polyangiitis (L-GPA) have already been investigated but, to our knowledge, have not been directly compared to those of patients with initial systemic disease (S-GPA); nor have the risk factors for L-GPA progression to systemic disease (LS-GPA) or its main characteristics been examined in large samples. We undertook this study to describe the main L-GPA features at diagnosis and their evolution over time, with comparisons of L- vs S-GPA patients.
Methods: French Vasculitis Study Group Registry patients had an isolated orbital mass and/or met EULAR recommendations1 for L-GPA, ie upper and/or lower respiratory tract, and, for S-GPA, categorizing them as early systemic, generalized or severe subset. All patients’ demographics, disease manifestations at diagnosis and long-term clinical outcomes were extracted from the database for analyses and L-GPA vs S-GPA comparisons.
Results: Among the 795 FVSG-Registry GPA patients, 87 (10.9%) had L-GPA (M/F ratio 1; mean±SD age 45.8±18.0 years) involving upper and lower airways in 56 (64.4%). Most L-GPA patients were positive for anti-proteinase-3 (PR3) (56.3%) or anti-myeloperoxidase (MPO) (21.8%) ANCA. Their main clinical manifestations were rhinitis (54.0%), lung nodules (50.6%), sinusitis (42.5%) and otitis (26.4%), with 5.7% each having subglottic stenosis or saddle nose. Comparing L-GPA vs. S-GPA patients at diagnosis, respectively, they were younger (mean±SD 45.8±18.0 vs. 54.1±15.9 years; P< 0.001), more frequently had saddle nose (5.7% vs. 0.7%; P=0.001) or subglottic stenosis (5.7% vs. 1.1%; P=0.006), were less often PR3-ANCA–positive (56.3% vs. 76.6%;< 0.001) and had lower BVAS (7.2 vs. 18.6; P< 0.001). L-GPA induction therapy less frequently included intravenous cyclophosphamide (46.0% vs. 79.4%; P< 0.001) or glucocorticoids (90.8% vs. 97.9%; P< 0.001; mean dose 48.7 vs. 58.0 mg; P< 0.001) but more often methotrexate (14.9% vs. 3.2%; P< 0.001). L- and S-GPA patients’ relapse-free–survival probability estimates (P=0.97), relapse rates (P=0.927) and refractory disease rates at each visit (P=0.543) were comparable, but the L-GPA overall survival rate from diagnosis was significantly higher (P< 0.0001). During follow-up (median 42 (range 6–241) months), 21 (24.1%) L-GPA progressed to LS-GPA after a median of 21 (range 3–93) months with peripheral/central neuropathy (n=8), ocular (n=8), renal (n=6), severe pulmonary (n=1), cardiovascular (n=1) and/or skin (n=1) relapses. No patient evolved into a severe renal subset. None of the evaluated demographic, clinical or serological factors predicted progression to LS-GPA.
Conclusion: The risk of L-GPA relapse was similar to that of S-GPA but L-GPA patients’ overall survival rate was higher. About a quarter of L-GPA patients developed clinical S-GPA manifestations, but never life-threatening end-stage organ disease.
1Hellmich B, et al. Ann Rheum Dis. 2007;66:605-17.
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, Campagne J, Decaux O, Hachulla E, Karras A, Khouatra C, Jourde-Chiche N, Viallard J, Godmer P, Blanchard-Delaunay C, Le Quellec A, Quéméneur T, de Moreuil C, Regent A, Terrier B, Mouthon L, Guillevin L, Puechal X. Localized versus Systemic Granulomatosis with Polyangiitis: Data from the French Vasculitis Study Group Database [abstract]. Arthritis Rheumatol. 2020; 72 (suppl 10). https://acrabstracts.org/abstract/localized-versus-systemic-granulomatosis-with-polyangiitis-data-from-the-french-vasculitis-study-group-database/. Accessed September 24, 2021.
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