Guillaume Lefèvre1, Amélie Leurs2, Jean-Baptiste Gibier3, Marie-Christine Copin3, Delphine Staumont-Sallé4, Frédéric Dezoteux4, Cécile Chenivesse5, Benjamin Lopez6, Louis Terriou7, Eric Hachulla8, David Launay8, Nicolas Etienne8, Myriam Labalette9, Pascal DeGroote10, François Pontana11, Thomas Quemeneur12, Pierre-Yves Hatron8, Nicolas Schleinitz13, Jean-François Viallard14, Mohamed Hamidou15, Thierry Martin16, Chafika Morati-Hafsaoui17, Matthieu Groh18, Marc Lambert2, Jean-Emmanuel Kahn19. 1. Centre de Référence National des Syndromes Hyperéosinophiliques (CEREO), Lille, France; Université de Lille, CHU Lille, Institut d'Immunologie, Lille, France; Université de Lille, CHU Lille, Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Auto-immunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), Lille, France; Université de Lille, INSERM, CHU Lille, Lille Inflammation Research International Center (LIRIC), Lille, France. Electronic address: guillaume.lefevre@chru-lille.fr. 2. Université de Lille, CHU Lille, Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Auto-immunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), Lille, France. 3. Centre de Référence National des Syndromes Hyperéosinophiliques (CEREO), Lille, France; Université de Lille, CHU Lille, Institut de Pathologie, Centre de Biologie Pathologie, Lille, France. 4. Centre de Référence National des Syndromes Hyperéosinophiliques (CEREO), Lille, France; Université de Lille, CHU Lille, Département de Dermatologie, Lille, France. 5. Centre de Référence National des Syndromes Hyperéosinophiliques (CEREO), Lille, France; Université de Lille, CHU Lille, Département de Pneumologie et Immuno-Allergologie, Centre de compétence Maladies Pulmonaires Rares, Lille, France. 6. Université de Lille, CHU Lille, Institut d'Immunologie, Lille, France. 7. Centre de Référence National des Syndromes Hyperéosinophiliques (CEREO), Lille, France; Université de Lille, CHU Lille, Institut d'Immunologie, Lille, France; Université de Lille, CHU Lille, Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Auto-immunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), Lille, France. 8. Centre de Référence National des Syndromes Hyperéosinophiliques (CEREO), Lille, France; Université de Lille, CHU Lille, Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Auto-immunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), Lille, France. 9. Centre de Référence National des Syndromes Hyperéosinophiliques (CEREO), Lille, France; Université de Lille, CHU Lille, Institut d'Immunologie, Lille, France. 10. Centre de Référence National des Syndromes Hyperéosinophiliques (CEREO), Lille, France; Université de Lille, CHU Lille, Département de Cardiologie, Institut National de la Santé et de la Recherche Médicale, Lille, France. 11. Centre de Référence National des Syndromes Hyperéosinophiliques (CEREO), Lille, France; Université de Lille, CHU Lille, Département de Radiologie Cardiovasculaire, Lille, France. 12. Centre Hospitalier de Valenciennes, Département de Médecine Interne, Valenciennes, France. 13. Centre de Référence National des Syndromes Hyperéosinophiliques (CEREO), Lille, France; Aix-Marseille Université, APHM, Département Médecine Interne Hôpital de la Timone, Marseille, France. 14. Centre de Référence National des Syndromes Hyperéosinophiliques (CEREO), Lille, France; CHU Bordeaux, Service de Médecine Interne, Hôpital Haut Lévêque, Pessac, France. 15. Centre de Référence National des Syndromes Hyperéosinophiliques (CEREO), Lille, France; CHU Nantes, Service de Médecine Interne, CHU Hôtel-Dieu, Nantes, France. 16. Centre de Référence National des Syndromes Hyperéosinophiliques (CEREO), Lille, France; CHU Strasbourg, Service d'immunologie clinique Nouvel Hôpital Civil, Strasbourg, France. 17. Centre de Référence National des Syndromes Hyperéosinophiliques (CEREO), Lille, France; CH Annecy Genevois, Service de Médecine Interne, Annecy, France. 18. Centre de Référence National des Syndromes Hyperéosinophiliques (CEREO), Lille, France; Hôpital Foch, Département de Médecine Interne, Suresnes, France. 19. Centre de Référence National des Syndromes Hyperéosinophiliques (CEREO), Lille, France; Hôpital Ambroise Paré, Service de Médecine Interne, Assistance Publique-Hôpitaux de Paris, Boulogne-Billancourt, France.
Abstract
BACKGROUND: The absence of asthma may rule out a diagnosis of eosinophilic granulomatosis with polyangiitis in patients with hypereosinophilic syndrome (HES) and features of vasculitis. OBJECTIVE: To describe eosinophilic vasculitis (EoV) as a possible manifestation of HES in asthma-free patients. METHODS: We screened our hospital database and the literature for patients with HES who met the following 4 criteria: (1) histopathological or clinical features of EoV (biopsy-proven vasculitis with predominant eosinophilic infiltration of the vessel wall and/or features of vasculitis with tissue and/or blood hypereosinophilia [absolute eosinophil count >1.5 G/L]); (2) no other obvious causes of reactive eosinophilia, organ damage, and vasculitis; (3) the absence of antineutrophil cytoplasmic antibodies; and (4) the absence of current asthma. RESULTS: Ten of our 83 (12%) asthma-free patients with HES and 107 additional cases in the literature met the criteria for EoV. After a critical analysis of the patients' clinical and laboratory characteristics and outcomes, we identified 41 cases of single-organ EoV (coronary arteritis, n = 29; temporal arteritis, n = 8; cerebral vasculitis, n = 4). Of the remaining 76 patients with EoV, the most frequent manifestations (>10%) were cutaneous vasculitis (56%), peripheral neuropathy (24%), thromboangiitis obliterans-like disease (16%), fever (13%), central nervous system involvement (13%), deep venous thrombosis (12%), and nonasthma lung manifestations (12%). Blood hypereosinophilia more than 1.5 G/L was observed in 79% of patients, and necrotizing vasculitis was observed in 44%. CONCLUSIONS: Our results suggest that idiopathic EoV (HES-associated vasculitis) can be classified as an eosinophilic-rich, necrotizing, systemic form of vasculitis that affects vessels of various sizes in asthma-free patients.
BACKGROUND: The absence of asthma may rule out a diagnosis of eosinophilic granulomatosis with polyangiitis in patients with hypereosinophilic syndrome (HES) and features of vasculitis. OBJECTIVE: To describe eosinophilic vasculitis (EoV) as a possible manifestation of HES in asthma-freepatients. METHODS: We screened our hospital database and the literature for patients with HES who met the following 4 criteria: (1) histopathological or clinical features of EoV (biopsy-proven vasculitis with predominant eosinophilic infiltration of the vessel wall and/or features of vasculitis with tissue and/or blood hypereosinophilia [absolute eosinophil count >1.5 G/L]); (2) no other obvious causes of reactive eosinophilia, organ damage, and vasculitis; (3) the absence of antineutrophil cytoplasmic antibodies; and (4) the absence of current asthma. RESULTS: Ten of our 83 (12%) asthma-freepatients with HES and 107 additional cases in the literature met the criteria for EoV. After a critical analysis of the patients' clinical and laboratory characteristics and outcomes, we identified 41 cases of single-organ EoV (coronary arteritis, n = 29; temporal arteritis, n = 8; cerebral vasculitis, n = 4). Of the remaining 76 patients with EoV, the most frequent manifestations (>10%) were cutaneous vasculitis (56%), peripheral neuropathy (24%), thromboangiitis obliterans-like disease (16%), fever (13%), central nervous system involvement (13%), deep venous thrombosis (12%), and nonasthma lung manifestations (12%). Blood hypereosinophilia more than 1.5 G/L was observed in 79% of patients, and necrotizing vasculitis was observed in 44%. CONCLUSIONS: Our results suggest that idiopathic EoV (HES-associated vasculitis) can be classified as an eosinophilic-rich, necrotizing, systemic form of vasculitis that affects vessels of various sizes in asthma-freepatients.
Authors: Enrico Ammirati; Emanuele Bizzi; Giacomo Veronese; Matthieu Groh; Caroline M Van de Heyning; Jukka Lehtonen; Marc Pineton de Chambrun; Alberto Cereda; Chiara Picchi; Lucia Trotta; Javid J Moslehi; Antonio Brucato Journal: Front Med (Lausanne) Date: 2022-03-07