Raphaël André1, Vincent Cottin2, Jean-Luc Saraux3, Gilles Blaison4, Boris Bienvenu5, Pascal Cathebras6, Robin Dhote7, Aurélie Foucher8, Helder Gil9, Joëlle Lapoirie10, David Launay11, Valentine Loustau12, François Maurier13, Edouard Pertuiset14, Thierry Zénone15, Jörg Seebach16, Nathalie Costedoat-Chalumeau17, Xavier Puéchal17, Luc Mouthon18, Loïc Guillevin17, Benjamin Terrier19. 1. Department of Internal Medicine, Hôpital Cochin, F-75014 Paris, France; National Referral Center for Systemic and Autoimmune Diseases, Hôpital Cochin, F-75014 Paris, France; Université Paris Descartes, Faculté de Médecine Paris Descartes, F-75014 Paris, France. 2. Department of Pulmonology, Hôpital Edouard Herriot, Lyon, France. 3. Department of Internal Medicine, Hôpital Simone Veil, Eaubonne, France. 4. Department of Internal Medicine, Hôpital Pasteur, Colmar, France. 5. Department of Internal Medicine, Centre Hospitalier Universitaire Caen, France. 6. Department of Internal Medicine, Centre Hospitalier Universitaire, Saint-Etienne, France. 7. Department of Internal Medicine, Centre Hospitalier Avicenne, Bobigny, France. 8. Department of Internal Medicine, Centre Hospitalier Universitaire, Saint-Pierre de la, Réunion. 9. Department of Internal Medicine, Crentre Hospitalier Universitaire, Besancon, France. 10. Department of Rheumatology, Centre Hospitalier, Pau, France. 11. Univ. Lille, U995, Lille Inflammation Research International Center (LIRIC), F-59000 Lille, France; Inserm, U995, F-59000 Lille, France; CHU Lille, département de médecine interne et immunologie clinique, F-59000 Lille, France; Centre national de référence maladies systémiques et auto-immunes rares (sclérodermie systémique), F-59000 Lille, France. 12. Department of Internal Medicine, Centre Hospitalier Henri Mondor, Créteil, France. 13. Department of Internal Medicine, Centre Hospitalier Saint-Blandine, Metz, France. 14. Department of Rheumatology, Centre Hospitalier René Dubos, Pontoise, France. 15. Department of Internal Medicine, Centre Hospitalier, Valence, France. 16. Department of Immunology and Allergology, Hôpitaux Universitaires de Genève, Switzerland. 17. Department of Internal Medicine, Hôpital Cochin, F-75014 Paris, France; National Referral Center for Systemic and Autoimmune Diseases, Hôpital Cochin, F-75014 Paris, France; Université Paris Descartes, Faculté de Médecine Paris Descartes, F-75014 Paris, France; Université Paris Descartes, Paris 5, Paris, France. 18. Department of Internal Medicine, Hôpital Cochin, F-75014 Paris, France; National Referral Center for Systemic and Autoimmune Diseases, Hôpital Cochin, F-75014 Paris, France; Université Paris Descartes, Faculté de Médecine Paris Descartes, F-75014 Paris, France; Université Paris Descartes, Paris 5, Paris, France; Inserm, U1016, Institut Cochin, F-75014 Paris, France. 19. Department of Internal Medicine, Hôpital Cochin, F-75014 Paris, France; National Referral Center for Systemic and Autoimmune Diseases, Hôpital Cochin, F-75014 Paris, France; Université Paris Descartes, Faculté de Médecine Paris Descartes, F-75014 Paris, France; Université Paris Descartes, Paris 5, Paris, France; Inserm, U1016, Institut Cochin, F-75014 Paris, France. Electronic address: benjamin.terrier@aphp.fr.
Abstract
BACKGROUND: Although peripheral nervous system involvement is common in eosinophilic granulomatosis with polyangiitis (EGPA), central nervous system (CNS) manifestations are poorly described. This study aimed to describe CNS involvement in EGPA. PATIENTS AND METHODS: This retrospective, observational, multicenter study included patients with EGPA and CNS involvement affecting cranial nerves, brain and/or spinal cord. We also undertook a systematic literature review. RESULTS: We analyzed 26 personal cases and 62 previously reported cases. At EGPA diagnosis, asthma was noted in 97%, eosinophilia in 98%, peripheral neuropathy in 55% and cardiac involvement in 41%. 38/71 (54%) were ANCA-positive, with a perinuclear-labeling pattern and/or anti-MPO specificity. CNS was involved in 86% at EGPA diagnosis, preceded EGPA in 2%, and occurred during follow-up in 12% after a median of 24months. Main neurological manifestations were ischemic cerebrovascular lesions in 46 (52%), intracerebral hemorrhage and/or subarachnoid hemorrhage in 21 (24%), loss of visual acuity in 28 (33%) (15 with optic neuritis, 9 with central retinal artery occlusion, 4 with cortical blindness), and cranial nerves palsies in 18 (21%), with 25 patients having ≥1 of these clinical CNS manifestations. Among the 81 patients with assessable neurological responses, 43% had complete responses without sequelae, 43% had partial responses with long-term sequelae and 14% refractory disease. After a mean follow-up of 36months, 11 patients died including 5 from intracerebral hemorrhages. CONCLUSION: EGPA-related CNS manifestations form 4 distinct neurological pictures: ischemic lesions, intracerebral hemorrhages, cranial nerve palsies and loss of visual acuity. Such manifestation should prompt practitioners to consider EGPA in such conditions. Long-term neurological sequelae were common, and intracerebral hemorrhages had the worst prognostic impact.
BACKGROUND: Although peripheral nervous system involvement is common in eosinophilic granulomatosis with polyangiitis (EGPA), central nervous system (CNS) manifestations are poorly described. This study aimed to describe CNS involvement in EGPA. PATIENTS AND METHODS: This retrospective, observational, multicenter study included patients with EGPA and CNS involvement affecting cranial nerves, brain and/or spinal cord. We also undertook a systematic literature review. RESULTS: We analyzed 26 personal cases and 62 previously reported cases. At EGPA diagnosis, asthma was noted in 97%, eosinophilia in 98%, peripheral neuropathy in 55% and cardiac involvement in 41%. 38/71 (54%) were ANCA-positive, with a perinuclear-labeling pattern and/or anti-MPO specificity. CNS was involved in 86% at EGPA diagnosis, preceded EGPA in 2%, and occurred during follow-up in 12% after a median of 24months. Main neurological manifestations were ischemic cerebrovascular lesions in 46 (52%), intracerebral hemorrhage and/or subarachnoid hemorrhage in 21 (24%), loss of visual acuity in 28 (33%) (15 with optic neuritis, 9 with central retinal artery occlusion, 4 with cortical blindness), and cranial nerves palsies in 18 (21%), with 25 patients having ≥1 of these clinical CNS manifestations. Among the 81 patients with assessable neurological responses, 43% had complete responses without sequelae, 43% had partial responses with long-term sequelae and 14% refractory disease. After a mean follow-up of 36months, 11 patients died including 5 from intracerebral hemorrhages. CONCLUSION: EGPA-related CNS manifestations form 4 distinct neurological pictures: ischemic lesions, intracerebral hemorrhages, cranial nerve palsies and loss of visual acuity. Such manifestation should prompt practitioners to consider EGPA in such conditions. Long-term neurological sequelae were common, and intracerebral hemorrhages had the worst prognostic impact.
Authors: Anthony Fong; Shahzada Ahmed; Satheesh Ramalingam; Rachel M Brown; Lorraine Harper; Susan P Mollan Journal: Neuroophthalmology Date: 2020-06-25
Authors: Rodney J Schlosser; Timothy L Smith; Jess C Mace; Jeremiah Alt; Daniel M Beswick; Jose L Mattos; Spencer Payne; Vijay R Ramakrishnan; Zachary M Soler Journal: Int Forum Allergy Rhinol Date: 2020-01 Impact factor: 3.858
Authors: George Markousis-Mavrogenis; Dimos D Mitsikostas; Loukia Koutsogeorgopoulou; Theodoros Dimitroulas; Gikas Katsifis; Panayiotis Argyriou; Dimitrios Apostolou; Stella Velitsista; Vasiliki Vartela; Dionysia Manolopoulou; Maria G Tektonidou; Genovefa Kolovou; George D Kitas; Petros P Sfikakis; Sophie I Mavrogeni Journal: J Clin Med Date: 2020-02-06 Impact factor: 4.241