Goldman Samuel, Wilson Benjamin, Awan Kanwal, White Tara, Williams Matthias
Internal Medicine, Walter Reed National Military Medical Center, Bethesda, USA.
Pulmonary Medicine, Walter Reed National Military Medical Center, Bethesda, USA.
Cureus. 2025 Mar 10;17(3):e80365. doi: 10.7759/cureus.80365. eCollection 2025 Mar.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a multifaceted diseased vasculitis typically associated with myeloperoxidase-perinuclear-anti-neutrophil cytoplasmic antibody (+MPO-P-ANCA). Although rare, the diagnosis should be considered in patients with difficult-to-control or late-onset asthma and extrathoracic disease. We present the case of a 37-year-old male with adult-onset asthma and chronic rhinitis hospitalized with pancreatitis and hypoxemia. Blood investigations demonstrated eosinophilia with elevated lipase, and bronchoscopy demonstrated multiple endobronchial lesions with elevated eosinophils on bronchoalveolar lavage. In addition, labs showed proteinase 3-specific antineutrophil cytoplasmic antibody (PR3-C-ANCA) autoantibodies, and the patient was diagnosed with EGPA. Additional differential diagnoses of parasitic infection, sarcoidosis, chronic eosinophilic pneumonia, and granulomatosis with polyangiitis (GPA) were entertained, but ultimately, the multisystemic involvement, anti-neutrophil cytoplasmic autoantibody (ANCA) positivity, bronchoscopy, and imaging findings clinched the diagnosis. This case highlights the spectrum of possible EGPA presentations and a rare case of PR3-C-ANCA with gastrointestinal manifestations.
嗜酸性肉芽肿性多血管炎（EGPA）是一种多方面表现的疾病性血管炎，通常与髓过氧化物酶-核周抗中性粒细胞胞浆抗体（+MPO-P-ANCA）相关。尽管罕见，但对于难以控制或迟发性哮喘及胸外疾病的患者应考虑进行诊断。我们报告一例37岁成年起病哮喘和慢性鼻炎男性患者，因胰腺炎和低氧血症住院。血液检查显示嗜酸性粒细胞增多伴脂肪酶升高，支气管镜检查显示支气管内多发病变，支气管肺泡灌洗显示嗜酸性粒细胞增多。此外，实验室检查显示蛋白酶3特异性抗中性粒细胞胞浆抗体（PR3-C-ANCA）自身抗体，该患者被诊断为EGPA。还考虑了寄生虫感染、结节病、慢性嗜酸性粒细胞性肺炎和肉芽肿性多血管炎（GPA）等其他鉴别诊断，但最终，多系统受累、抗中性粒细胞胞浆自身抗体（ANCA）阳性、支气管镜检查及影像学表现确诊了该诊断。本病例突出了EGPA可能的表现谱以及一例罕见的伴有胃肠道表现的PR3-C-ANCA病例。
