Ranjbar Yasamin, Árokszállási Tamás, Szinay Dorottya, Nagy Edit B, Tarr Tünde, Nagy-Vincze Melinda
Division of Immunology, Department of Internal Medicine, Faculty of Medicine, University of Debrecen, 4032 Debrecen, Hungary.
Department of Neurology, Faculty of Medicine, University of Debrecen, 4032 Debrecen, Hungary.
J Clin Med. 2025 Aug 10;14(16):5652. doi: 10.3390/jcm14165652.
Central nervous system (CNS) involvement is an extremely rare manifestation in eosinophilic granulomatosis with polyangiitis (EGPA), associated with a poor prognosis. Here we present a case of 50-year-old female patient with long-term asthma treatment who presented initially with extreme eosinophilia (56%) and severe progressive ascending paresis, similar to Guillain-Barré syndrome, leading to tetraplegia. After navigating through diagnostic mazes, the diagnosis of EGPA was established based on eosinophilia, myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) positivity, asthma, eosinophil granulomatosis in the gastrointestinal tract, and severe peripheral nervous system involvement, complicated with rare central nervous granulomas and ischemia. With combined immunosuppressive and immunomodulatory treatment including high-dose corticosteroids, rituximab and intravenous immunoglobulin along with symptomatic treatment and planned rehabilitation over 6 months, our patient recovered gradually from tetraplegia and adverse events such as severe infections and osteoporotic fractures. Now, from a 2-year perspective, we can conclude a successful treatment leading to decrease in all of her symptoms. Due to persistent eosinophilia after steroid tapering, she was switched to mepolizumab maintenance treatment and demonstrated continuous improvement of motor and sensory functions. Thanks to periodically repeated rehabilitation, she became self-sufficient and returned to her previous job. Our case highlights that EGPA patients should be treated in a center of expertise due to the rarity of the disease and complexity of diagnosis and treatment. Careful multidisciplinary cooperation, the huge effort of the patient, and a supportive environment can show a way back from immune-mediated tetraplegia.
中枢神经系统(CNS)受累是嗜酸性肉芽肿性多血管炎(EGPA)极为罕见的表现,预后较差。本文报告一例50岁长期接受哮喘治疗的女性患者,最初表现为极度嗜酸性粒细胞增多(56%)和严重的进行性上升性麻痹,类似于吉兰-巴雷综合征,导致四肢瘫痪。在经过一系列诊断难题后,基于嗜酸性粒细胞增多、髓过氧化物酶抗中性粒细胞胞浆抗体(MPO-ANCA)阳性、哮喘、胃肠道嗜酸性粒细胞肉芽肿以及严重的外周神经系统受累,并伴有罕见的中枢神经肉芽肿和缺血,确诊为EGPA。通过联合免疫抑制和免疫调节治疗,包括大剂量糖皮质激素、利妥昔单抗和静脉注射免疫球蛋白,以及对症治疗和为期6个月的计划性康复治疗,我们的患者逐渐从四肢瘫痪以及严重感染和骨质疏松性骨折等不良事件中恢复。从2年的随访来看,我们可以得出治疗成功,患者所有症状均减轻的结论。由于减停激素后嗜酸性粒细胞持续增多,她转而接受美泊利单抗维持治疗,运动和感觉功能持续改善。由于定期重复进行康复治疗,她实现了自理并重返原工作岗位。我们的病例强调,由于EGPA疾病罕见且诊断和治疗复杂,患者应在专业中心接受治疗。精心的多学科合作、患者的巨大努力以及支持性的环境可为免疫介导的四肢瘫痪患者指明康复之路。