Overlapping Forms of Eosinophilic Granulomatosis with Polyangiitis and Granulomatosis with Polyangiitis: Presentation, Management and Outcomes

Federica Pallotti¹, Camille Mettler², Roberto Padoan³, Francesca Regola⁴, Franco Franceschini⁵, Sergey Moiseev⁶, Pavel Novikov⁷, Mario Andrea Piga⁸, Gianluca Moroncini⁹, Silke Brix¹⁰, Abdul Hadi Kafagi¹¹, Samuel Deshayes¹², Achille Aouba¹², Julien Campagne¹³, Paolo Delvino¹⁴, Jan Willem Cohen Tervaert¹⁵, Luisa Brussino¹⁶, Martin Michaud¹⁷, Nils Venhoff¹⁸, Federico Alberici¹⁹, Claudia Iannone²⁰, Sophie Rosenstingl²¹, Marin Moutel²², Jean-Marc Galempoix²³, Vincent Cottin²⁴, Clara Jaccard²⁵, Diane Riehl²⁶, Paul Legendre²⁷, Anne-Claire Billet²⁸, Paola Parronchi²⁹, Luca Quartuccio³⁰, Vítor Silvestre Teixeira³¹, Allyson Egan³², David Jayne³², Enrico Tombetti³³, Marco Caminati³⁴, Christian Pagnoux³⁵, Alexis Régent³⁶, Marc Ruivard³⁷, Loïc Guillevin³⁸, Xavier Puéchal³⁶ and Benjamin Terrier³⁹, and the French Vasculities Study Group and European EGPA Study Group, ¹Internal Medicine, Centre Hospitalier Universitaire de Caen, Caen, France, ²Département de Médecine Interne, Centre de Référence National pour les maladies auto-immunes systémiques rares, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France, ³Department of Medicine DIMED, Division of Rheumatology, University of Padua, Padua, Italy, ⁴Unit of Rheumatology and Clinical Immunology, ASST Spedali Civili Brescia and University of Brescia, Brescia, Italy, ⁵Scleroderma Unit, Rheumatology and Clinical Immunology Unit, ERN ReCONNET, ASST Spedali Civili, Brescia, Italy, Brescia, Italy, ⁶Tareev Clinic of Internal Diseases, Sechenov First Moscow State Medical University, Moscow, Russia, ⁷Sechenov First Moscow State Medical University, Moscow, Russia, ⁸Postgraduate School of Allergy and Clinical Immunology, Università Politecnica delle Marche, Ancona, Italy, ⁹Department of Clinical and Molecular Sciences, Marche Polytechnic University & Department of Internal Medicine, Marche University Hospital, Ancona, Italy, ¹⁰Division of Cell Matrix Biology and Regenerative Medicine, School of Biological Sciences, The University of Manchester, Manchester, United Kingdom, ¹¹Division of Cardiovascular Sciences, School of Medical Sciences, University of Manchester, Manchester, United Kingdom, ¹²Service d'immunologie clinique-médecine interne, CHU de Caen Normandie, Caen, France, ¹³Hôpital Robert Schuman - Competence center for autoimmune diseases, Internal Medicine, Metz, France, ¹⁴University of Milano-Bicocca, Milan, Milan, Italy, ¹⁵University of Alberta, Edmonton, Canada, ¹⁶SSDDU Immunologia Clinica ed Allergologia, AO Mauriziano, Turin, Italy, ¹⁷Department of Internal Medicine, Clinique Saint-Exupery, Toulouse, France., Toulouse, France, ¹⁸Medical Center - University of Freiburg, Internal Medicine, Department of Rheumatology and Clinical Immunology, Freiburg, Germany, ¹⁹Nephrology Unit, ASST Spedali Civili di Brescia, Brescia, Italy, ²⁰Department of Rheumatology, University of Milan, and Rheumatology Department, ASST Pini-CTO, Milan, Italy, ²¹Service Médecine Interne, Centre hospitalier intercommunal Compiègne Noyon, Compiègne Noyon, France, ²²Department of Internal Medicine, Infectious Diseases, and Clinical Immunology, Reims University Hospital, Reims, France, ²³J.M. Galempoix, MD, Department of Internal Medicine, Nord Ardennes Hospital, Charleville-Mézières, France, ²⁴Hôpital Louis Pradel, Centre de Référence des Maladies Pulmonaires Rares, Hospices Civils de Lyon, Université Claude Bernard Lyon 1, Lyon, France, Lyon, France, ²⁵Gaston Bourret Hospital, Nouméa, New Caledonia, ²⁶Centre hospitalier intercommunal Toulon- La Seyne Sur Mer, Toulon, France, ²⁷Service Médecine Interne et Polyvalente, Centre Hospitaliers Le Mans, Le Mans, France, ²⁸Department of Internal Medicine, Édouard Herriot Hospital, Hospices Civils de Lyon, Lyon, France, ²⁹University of Florence, Florence, Florence, Italy, ³⁰Division of Rheumatology, Department of Medicine, University of Udine, Udine, Italy, ³¹Department of Rheumatology, Faro Hospital, Algarve, Portugal, ³²University of Cambridge, Cambridge, United Kingdom, ³³Internal Medicine and Rheumatology, Department of Biomedical and Clinical Sciences, Sacco and Fatebenefratelli Hospitals, Milan, Italy, Milan, Italy, ³⁴Department of Medicine, Asthma, Allergy and Clinical Immunology Section, University of Verona, Verona, Italy, ³⁵Mount Sinai Hospital, Toronto, ON, Canada, ³⁶National Referral Center For Rare Systemic Autoimmune Diseases, Paris, France, ³⁷Internal Medicine Department, Estaing University Hospital, CHU Clermont-Ferrand, Clermonnt-Ferrand, Italy, ³⁸National Referral Center For Rare Systemic Autoimmune Diseases, Paris, Ile-de-France, France, ³⁹Service de Médecine interne, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France

Meeting: ACR Convergence 2024

Keywords: ANCA associated vasculitis, Cohort Study, Eosinophilic Granulomatosus with Polyangiitis (Churg-Strauss), Granulomatosis with Polyangiitis (GPA)

Session Information

Date: Sunday, November 17, 2024

Title: Vasculitis – ANCA-Associated Poster II

Session Type: Poster Session B

Session Time: 10:30AM-12:30PM

Background/Purpose: ANCA-associated vasculitis (AAV) include granulomatosis with polyangiitis (GPA), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (EGPA). Although these entities are often easily distinguished in daily practice, some cases may be more difficult to identify. Hence, the identification of overlapping forms between GPA and EGPA may be crucial due to different organ damage and therapeutic approaches. We aimed to describe the existence of overlapping forms of EGPA and GPA, phenotype, severity and therapeutic management.

Methods: We conducted a European multicenter retrospective study in 34 centers to include patients with overlapping forms of EGPA and GPA. Patients were defined as follows: 1) patients fulfilling both ACR/EULAR 2022 criteria for GPA and EGPA; 2) patients fulfilling ACR/EULAR 2022 criteria for EGPA with PR3-ANCA and/or pulmonary nodules; 3) patients fulfilling ACR/EULAR 2022 criteria for GPA with eosinophilic count >1000/mm³; 4) patients with AAV who do not meet ACR/EULAR 2022 criteria for EGPA and GPA but have both PR3-ANCA and eosinophilia >1000/mm³.

Results: A total of 137 patients with overlapping forms (males in 62.8%, median age 52.5 [IQR 42.2-63] years) were analyzed. The main clinical manifestations were constitutional symptoms (70.8%), ENT involvement (83.9%) (mainly sinusitis, nasal crusts or polyposis), lung nodules (55.5%) sometimes excavated, asthma (55.5%), alveolar hemorrhage (19.7%), cutaneous involvement (51.8%). Renal injury was observed in 48.2%, peripheral neuropathy in 39.4%, cardiac involvement in 24.1%, gastrointestinal involvement in 16.8% and central nervous system involvement in 8% (mainly ischemic stroke). ANCA were found in 80.3%, as PR3-ANCA in 61.3% and MPO-ANCA in 19%, and median eosinophil count was 6000/mm³ [IQR 2500-10000]. Five factor score was ≥1 for 17 patients (12.4%). Induction therapy consisted of high-dose glucocorticoids in 134 patients (97.8%), preceded by methylprednisolone pulses in 47.4%, combined with cyclophosphamide in 42.3%, rituximab in 16.8%, plasma exchange in 8%, and mepolizumab in 2.2%. Remission was achieved in 93.4%, and maintenance therapy consisted in azathioprine (29.2%), methotrexate (13.9%), rituximab (12.4%) and mepolizumab (4.4%). Six (4.4%) more patients received mepolizumab during the follow-up. Relapse-free survival was 84.7% at 1 year and 54.7% at 5 years, and relapses occurred after a median of 24.9 months [IQR 8.5-47]. Relapses were mainly pulmonary in 55.4%, ENT in 45.9%, and neurological in 24.3%. In these EGPA/GPA forms, ENT signs was the only variable associated with relapse (OR 4.7 [1.3-19.8]), while age < 65 years, female, PR3-ANCA, and asthma tended to be not associated. Death occurred in 20 patients (14.6%).

Conclusion: Overlapping forms of EGPA and GPA may occur, sharing complications of both primary forms. Relapses seem to be frequent and mainly affect lungs, ENT and nerves. Rituximab has rarely been used in these forms despite the features of GPA, probably due to the frequent diagnosis of EGPA. A cluster analysis is underway to better characterize the patient population within this group and potentially different prognosis.

Disclosures: F. Pallotti: None; C. Mettler: None; R. Padoan: None; F. Regola: None; F. Franceschini: None; S. Moiseev: None; P. Novikov: None; M. Piga: None; G. Moroncini: None; S. Brix: None; A. Hadi Kafagi: None; S. Deshayes: None; A. Aouba: None; J. Campagne: None; P. Delvino: GlaxoSmithKlein(GSK), 6; J. Cohen Tervaert: None; L. Brussino: None; M. Michaud: None; N. Venhoff: AbbVie/Abbott, 1, 5, 6, AstraZeneca, 1, 6, Boehringer-Ingelheim, 1, 6, Bristol-Myers Squibb(BMS), 6, Celgene, 6, Chugai, 6, GlaxoSmithKlein(GSK), 1, 6, Janssen, 1, 6, Medac, 5, Novartis, 1, 5, 6, Pfizer, 1, 5, 6, Roche, 1, 6, UCB, 1, 6, Vifor, 1, 6; F. Alberici: None; C. Iannone: None; S. Rosenstingl: None; M. Moutel: None; J. Galempoix: None; V. Cottin: AstraZeneca, 2, Boehringer Ingelheim, 1, 5, 6, Celgene/BMS, 2, CSL Behring, 12, Jury for CSL grant, Ferrer/ United Therapeutics, 2, 6, Fibrogen, 4, GSK, 4, 6, Molecure, 4, Pliant, 2, PureTech, 2, Roche, 2, Sanofi, 6, Shionogi, 2, Vifor, 2; C. Jaccard: None; D. Riehl: None; P. Legendre: None; A. Billet: None; P. Parronchi: None; L. Quartuccio: None; V. Teixeira: None; A. Egan: None; D. Jayne: Amgen, 2, 6, AstraZeneca, 2, 6, Aurinia, 4, Boehringer Ingelheim, 2, 6, Bristol-Myers Squibb(BMS), 2, 6, ChemoCentryx, 2, 6, Chinook, 1, CSL Vifor, 2, GSK, 1, 2, 6, Novartis, 2, 6, Roche, 2, 6, Takeda, 1, 2, 6, Vifor Pharma, 2, 6; E. Tombetti: None; M. Caminati: None; C. Pagnoux: AstraZeneca, 1, GlaxoSmithKline, 1, 2, 5, 6, Otsuka, 1, 2, 5, 6, Pfizer, 2, 5, 6, Roche, 1, 2, 5, 6; A. Régent: None; M. Ruivard: None; L. Guillevin: None; X. Puéchal: None; B. Terrier: AstraZeneca, 2, GlaxoSmithKline, 2, Novartis, 2, Vifor Pharma, 2.

To cite this abstract in AMA style:

Pallotti F, Mettler C, Padoan R, Regola F, Franceschini F, Moiseev S, Novikov P, Piga M, Moroncini G, Brix S, Hadi Kafagi A, Deshayes S, Aouba A, Campagne J, Delvino P, Cohen Tervaert J, Brussino L, Michaud M, Venhoff N, Alberici F, Iannone C, Rosenstingl S, Moutel M, Galempoix J, Cottin V, Jaccard C, Riehl D, Legendre P, Billet A, Parronchi P, Quartuccio L, Teixeira V, Egan A, Jayne D, Tombetti E, Caminati M, Pagnoux C, Régent A, Ruivard M, Guillevin L, Puéchal X, Terrier B. Overlapping Forms of Eosinophilic Granulomatosis with Polyangiitis and Granulomatosis with Polyangiitis: Presentation, Management and Outcomes [abstract]. Arthritis Rheumatol. 2024; 76 (suppl 9). https://acrabstracts.org/abstract/overlapping-forms-of-eosinophilic-granulomatosis-with-polyangiitis-and-granulomatosis-with-polyangiitis-presentation-management-and-outcomes/. Accessed .

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