嗜酸性肉芽肿性多血管炎合并非结核分枝杆菌病1例并文献复习

Eosinophilic Granulomatosis with Polyangiitis Complicated by Nontuberculous Mycobacterial Disease: A Case Report and Literature Review

摘要: 目的：报道一例嗜酸性肉芽肿性多血管炎(eosinophilic granulomatosis with polyangiitis, EGPA)合并非结核分枝杆菌病，分析其临床表现、诊断标准、治疗方法，结合文献分析其临床特点。方法：回顾性分析广州市第十二人民医院2021年11月8日收治临床表现最终诊断为嗜酸性肉芽肿性多血管炎的患者。以“嗜酸性肉芽肿性多血管炎”为检索词，检索2021年1月1日至2023年12月31日期间发表在PubMed、中国知网、万方数据库、中华医学期刊全文数据库的病例报道，共获得文献71篇，其中外文62篇，中文9篇。结果：包括本病例在内共有72例，男性40例，平均年龄(53.9 ± 14.28)岁；女性32例，平均年龄(57.63 ± 17.69)岁；中位年龄58岁。其中c-ANCA、p-ANCA均阳性7例，c-ANCA、p-ANCA均阴性46例，单纯c-ANCA阳性7例，p-ANCA阳性12例，60例有病理结果支持。主要临床表现：哮喘、鼻窦炎、神经炎、嗜酸性粒细胞增多、IgE升高，对治疗敏感。结论：EGPA是罕见多系统炎症性疾病，发病率低，常因表现不特异而被误诊。关键是，在使用糖皮质激素之前，该病具有普遍致命性，50%未经治疗的患者在血管炎发作后3个月内死亡。自从全身性糖皮质激素的广泛应用以及选择性应用免疫抑制剂治疗病情较重的患者，EGPA患者的预后已显著改善。

Abstract: Objective: This paper aims to report a case of eosinophilic granulomatosis with polyangiitis (EGPA) complicated by nontuberculous mycobacterial disease, analyze its clinical manifestations, diagnostic criteria, and treatment methods, and review its clinical characteristics in light of the literature. Methods: A retrospective analysis was conducted on a case admitted to Guangzhou Twelfth People’s Hospital on November 8, 2021, whose clinical manifestations ultimately led to a diagnosis of eosinophilic granulomatosis with polyangiitis. Using “eosinophilic granulomatosis with polyangiitis” as the search term, case reports published between January 1, 2021, and December 31, 2023, in PubMed, CNKI, Wanfang Database, and the Chinese Medical Journal Full-text Database were retrieved. A total of 71 articles were obtained, including 62 in foreign languages and 9 in Chinese. Results: Including the present case, a total of 72 cases were analyzed. Among them, there were 40 males with a mean age of 53.9 ± 14.28 years, and 32 females with a mean age of 57.63 ± 17.69 years (median age: 58 years). Both c-ANCA and p-ANCA were positive in 7 cases, both negative in 46 cases, c-ANCA alone positive in 7 cases, and p-ANCA alone positive in 12 cases. Pathological results supported the diagnosis in 60 cases. The main clinical manifestations included asthma, sinusitis, neuritis, eosinophilia, elevated IgE, and sensitivity to treatment. Conclusion: EGPA is a rare multisystem inflammatory disease with low incidence. It is often misdiagnosed due to nonspecific manifestations. The key point is that before the use of glucocorticoids, the disease is generally fatal, with 50% of untreated patients dying within three months of vasculitis onset. Since the widespread application of systemic glucocorticoids and the selective use of immunosuppressants in severely ill patients, the prognosis of EGPA patients has significantly improved.