Kalinova Desislava, Kukushev Georgi, Kolarov Zlatimir, Rashkov Rasho
Clinic of Rheumatology, UMHAT St. Ivan Rilski University Hospital, Sofia, Bulgaria.
Department of Otolaryngology, Military Medical Academy, Sofia, Bulgaria.
Reumatologia. 2019;57(5):288-291. doi: 10.5114/reum.2019.89522. Epub 2019 Oct 31.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis characterised by bronchial asthma, hypereosinophilia, and systemic vasculitis. History of asthma with blood eosinophilia and multiorgan involvement are the important clues to suspect EGPA. In the original paper by Churg and Strauss cardiac, gastrointestinal tract, renal, and neurological involvement were noted more frequently. The pattern of neurological involvement may be mononeuritis multiplex, and symmetrical and asymmetrical polyneuropathy. Mononeuritis multiplex was present in 78.1% while cranial nerves were involved in only 4.1% of cases. Glucocorticosteroids and immunosuppressants, especially cyclophosphamide, have considerably improved the prognosis and overall survival rates in patients with systemic vasculitis, including eosinophilic granulomatosis with polyangiitis. The authors present a clinical case of eosinophilic granulomatosis with polyangiitis with severe mononeuritis multiplex. The case reflects the successful application of a cyclophosphamide regime as a remission inducer.
嗜酸性肉芽肿性多血管炎（EGPA）是一种系统性血管炎，其特征为支气管哮喘、嗜酸性粒细胞增多以及系统性血管炎。伴有血液嗜酸性粒细胞增多和多器官受累的哮喘病史是怀疑EGPA的重要线索。在Churg和Strauss的原始论文中，心脏、胃肠道、肾脏和神经系统受累更为常见。神经系统受累的模式可能是多发性单神经炎，以及对称性和非对称性多发性神经病。78.1%的病例存在多发性单神经炎，而仅4.1%的病例累及颅神经。糖皮质激素和免疫抑制剂，尤其是环磷酰胺，已显著改善了包括嗜酸性肉芽肿性多血管炎在内的系统性血管炎患者的预后和总生存率。作者介绍了一例患有严重多发性单神经炎的嗜酸性肉芽肿性多血管炎的临床病例。该病例反映了环磷酰胺方案作为缓解诱导剂的成功应用。
