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.
Department of Pulmonology, Hôpital Edouard Herriot, Lyon, France.
Autoimmun Rev. 2017 Sep;16(9):963-969. doi: 10.1016/j.autrev.2017.07.007. Epub 2017 Jul 12.
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.
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.
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.
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.
尽管嗜酸性粒细胞性肉芽肿伴多血管炎（EGPA）常累及周围神经系统，但中枢神经系统（CNS）表现描述甚少。本研究旨在描述 EGPA 中的 CNS 受累。
本回顾性、观察性、多中心研究纳入了 EGPA 合并影响颅神经、脑和/或脊髓的 CNS 受累患者。我们还进行了系统文献回顾。
我们分析了 26 例个人病例和 62 例既往报道的病例。在 EGPA 诊断时，97%有哮喘，98%有嗜酸性粒细胞增多，55%有周围神经病，41%有心脏受累。38/71（54%）为 ANCA 阳性，核周型和/或抗 MPO 特异性。86%的 EGPA 患者在 EGPA 诊断时即有 CNS 受累，2%在 EGPA 之前，12%在中位随访 24 个月后发生。主要神经系统表现为缺血性脑血管病变 46 例（52%），颅内出血和/或蛛网膜下腔出血 21 例（24%），视力丧失 28 例（33%）（15 例视神经炎，9 例视网膜中央动脉阻塞，4 例皮质盲），颅神经麻痹 18 例（21%），25 例患者有≥1 种这些临床 CNS 表现。在可评估神经反应的 81 例患者中，43%无后遗症完全缓解，43%有长期后遗症的部分缓解，14%为难治性疾病。平均随访 36 个月后，11 例患者死亡，其中 5 例死于颅内出血。
EGPA 相关的 CNS 表现可分为 4 种不同的神经表现：缺血性病变、颅内出血、颅神经麻痹和视力丧失。这些表现应促使医生在这些情况下考虑 EGPA。长期神经后遗症常见，颅内出血预后最差。
