Ohta Ryuichi, Fujimori Taichi, Sano Chiaki
Communiy Care, Unnan City Hospital, Unnan, JPN.
Internal Medicine, Shimane University, Izumo, JPN.
Cureus. 2024 Feb 24;16(2):e54832. doi: 10.7759/cureus.54832. eCollection 2024 Feb.
This case report elucidates the diagnosis and management of eosinophilic granulomatosis with polyangiitis (EGPA), a form of systemic vasculitis, in a 32-year-old female presenting with progressive dermal, respiratory, and gastrointestinal symptoms following multiple pregnancies. The patient's history of allergic reactions and pregnancies suggested a gradual progression of EGPA, a condition rarely associated with pregnancy. Initial symptoms were misinterpreted as allergic reactions and acute gastroenteritis, delaying the correct diagnosis. Laboratory findings included eosinophilia and elevated immunoglobulin E, while further investigations ruled out other differential diagnoses, such as chronic eosinophilic leukemia. A clinical diagnosis of EGPA was made based on symptom progression, eosinophilia, and mononeuritis multiplex, absent typical granulomatous changes in the skin biopsy. Treatment with high-dose prednisolone and rituximab halted disease progression and improved symptoms, highlighting the critical importance of prompt diagnosis and treatment in preventing irreversible complications. This case emphasizes the need for general physicians to consider pregnancy as a potential trigger for autoimmune diseases like EGPA, especially in patients presenting with multi-symptom allergic reactions and high inflammatory markers.
本病例报告阐述了一名32岁女性嗜酸性肉芽肿性多血管炎（EGPA）的诊断和管理，EGPA是一种系统性血管炎，该患者在多次妊娠后出现进行性皮肤、呼吸和胃肠道症状。患者的过敏反应和妊娠史提示EGPA病情逐渐进展，这是一种很少与妊娠相关的疾病。最初的症状被误诊为过敏反应和急性肠胃炎，延误了正确诊断。实验室检查结果包括嗜酸性粒细胞增多和免疫球蛋白E升高，而进一步检查排除了其他鉴别诊断，如慢性嗜酸性粒细胞白血病。基于症状进展、嗜酸性粒细胞增多和多发性单神经炎，在皮肤活检中未发现典型的肉芽肿性改变，从而做出了EGPA的临床诊断。高剂量泼尼松龙和利妥昔单抗治疗阻止了疾病进展并改善了症状，突出了及时诊断和治疗对预防不可逆并发症的至关重要性。本病例强调普通医生需要将妊娠视为EGPA等自身免疫性疾病的潜在触发因素，尤其是在出现多症状过敏反应和高炎症指标的患者中。
