门诊环境中嗜酸性肉芽肿性多血管炎的早期识别：呼吸系统疾病之外的肌肉骨骼和胃肠道线索。

文献信息

中文摘要

背景： 嗜酸性肉芽肿性多血管炎（EGPA）是一种多系统免疫介导性疾病，以成人起病哮喘、嗜酸性炎症和小至中等血管的坏死性血管炎为特征。诊断常因临床上明显的神经系统、心脏、肾脏、皮肤或胃肠道受累而被提示。然而，可能具有提示意义的表现或许在更早期就已可识别，尤其是当哮喘、伴鼻息肉的慢性鼻窦炎和血嗜酸性粒细胞增多与其它非特异性肺外表现共存时。大多数队列描述的是诊断时或已确立疾病期间的表现，对其在不同专科中早期时间性出现的信息有限。这一空白与关节痛和肌痛尤为相关，这些症状常见但特征描述不足；也与嗜酸性胃肠道表现相关，后者最初可能被归类为器官局限的嗜酸性胃肠道疾病。活检证实的肌炎罕见，但偶尔有在就诊时或临床可识别的神经病之前被报道。本综述考察EGPA在门诊环境中早期且可能可识别的表现，特别关注肌肉骨骼症状、客观肌肉受累、嗜酸性胃肠道疾病，以及区分器官局限嗜酸性疾病与系统性EGPA的治疗意义。

英文摘要

Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystem immune-mediated disease characterized by adult-onset asthma, eosinophilic inflammation, and necrotizing vasculitis of small-to-medium-sized vessels. Diagnosis is frequently prompted by clinically overt neurological, cardiac, renal, cutaneous, or gastrointestinal involvement. However, potentially informative manifestations may already be recognizable earlier, particularly when asthma, chronic rhinosinusitis with nasal polyps, and blood eosinophilia coexist with otherwise nonspecific extrapulmonary findings. Most cohorts describe manifestations at diagnosis or during established disease and provide limited information on their early temporal appearance across different specialties. This gap is particularly relevant to arthralgia and myalgia, which are common but poorly characterized, and to eosinophilic gastrointestinal manifestations that may initially be classified as organ-restricted eosinophilic gastrointestinal disorders. Biopsy-proven myositis is rare but has occasionally been reported at presentation or before clinically recognizable neuropathy. This review examines early and potentially recognizable manifestations of EGPA across outpatient settings, with particular attention to musculoskeletal symptoms, objective muscle involvement, eosinophilic gastrointestinal disease, and the therapeutic implications of distinguishing organ-restricted eosinophilic disease from systemic EGPA.