Localized versus Systemic Granulomatosis with Polyangiitis: Data from the French Vasculitis Study Group Database

Michele Iudici¹, Christian Pagnoux², Delphine Courvoisier³, Pascal Cohen⁴, Mohamed Hamidou⁵, Achille Aouba⁶, François Lifermann⁷, Marc Ruivard⁸, Olivier Aumaitre⁹, Bernard Bonnotte¹⁰, Julien Campagne¹¹, Olivier Decaux¹², Eric Hachulla¹³, Alexandre Karras¹⁴, Chahéra Khouatra¹⁵, Noémie Jourde-Chiche¹⁶, Jean-François Viallard¹⁷, Pascal Godmer¹⁸, Claire Blanchard-Delaunay¹⁹, Alain Le Quellec²⁰, Thomas Quéméneur²¹, Claire de Moreuil²², Alexis Regent⁴, Benjamin Terrier⁴, Luc Mouthon⁴, Loïc Guillevin⁴ and Xavier Puechal⁴, ¹National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris-Descartes University and Geneva University Hospitals, Geneva, Switzerland, ²Division of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada, ³Division of rheumatology, Geneva University Hospitals, Geneva, Switzerland, ⁴National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris-Descartes University, Paris, France, ⁵Department of Internal Medicine, Hôtel-Dieu, CHU Nantes, Nantes, France, ⁶Department of Internal Medicine, CHU Côte de Nacre, Caen, France, ⁷Department of Internal Medicine, CH Côte d’Argent, Dax, France, ⁸Department of Internal Medicine, CHU Estaing, Clermont Ferrand, France, ⁹Department of Internal Medicine, CHU Gabriel Montpied, Clermont Ferrand, France, ¹⁰CHU Dijon, Dijon, France, ¹¹Department of Internal Medicine, Hôpitaux Privés de Metz, Metz, France, ¹²CHU Rennes, Rennes, France, ¹³Department of Internal Medicine, CHRU, Lille Cedex, France, ¹⁴Department of Nephrology, Hôpital Européen Georges Pompidou, Paris, France, ¹⁵Department of Respiratory Medicine, CHU Louis Pradel, Lyon, France, ¹⁶Department of Nephrology, CHU de la Conception, Marseille, France, ¹⁷Department of Internal Medicine, CHU Haut-Lévêque, Bordeaux, France, ¹⁸Department of Internal Medicine, CH Bretagne Atlantique, Vannes, France, ¹⁹Department of Internal Medicine, CH, Niort, France, ²⁰Department of Internal Medicine, CHU Saint Eloi, Montpellier, France, ²¹Department of Internal Medicine, CH, Valenciennes, France, ²²Department of Internal Medicine, CHU Cavale Blanche, Brest, France

Meeting: ACR Convergence 2020

Keywords: ANCA associated vasculitis, Granulomatosis with Polyangiitis (GPA)

Session Information

Date: Monday, November 9, 2020

Title: Vasculitis – ANCA-Associated (2048–2052)

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.

Reference
¹Hellmich B, et al. Ann Rheum Dis. 2007;66:605-17.

Disclosure: M. Iudici, None; C. Pagnoux, Chemocentryx, 1, GlaxoSmithKline, 1, 2, 3, Sanofi, 1, Hoffman-LaRoche, 1, 2, 3; D. Courvoisier, None; P. Cohen, None; M. Hamidou, None; A. Aouba, None; F. Lifermann, None; M. Ruivard, None; O. Aumaitre, None; B. Bonnotte, None; J. Campagne, None; O. Decaux, None; E. Hachulla, None; A. Karras, None; C. Khouatra, None; N. Jourde-Chiche, None; J. Viallard, None; P. Godmer, None; C. Blanchard-Delaunay, None; A. Le Quellec, None; T. Quéméneur, None; C. de Moreuil, None; A. Regent, None; B. Terrier, None; L. Mouthon, None; L. Guillevin, None; X. Puechal, Roche Pharma, 2.

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 .

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