Zeleke Sinen Tadesse, Zaidi Syeda Ramsha, Gebremedhen Ademsegd Isac, Becker Melinda
Marshall University Joan C. Edwards School of Medicine, Huntington, WV, USA.
J Investig Med High Impact Case Rep. 2025 Jan-Dec;13:23247096251363027. doi: 10.1177/23247096251363027. Epub 2025 Jul 30.
Granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA) are distinct yet overlapping forms of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, each with unique clinical features. GPA typically involves necrotizing granulomatous inflammation of the upper and lower respiratory tracts and is strongly associated with cytoplasmic ANCA (c-ANCA) and PR3-ANCA positivity, while EGPA is characterized by eosinophilia, asthma, and vasculitis. Rare cases of GPA can present with eosinophilia, complicating the distinction between these entities and raising the possibility of a GPA/EGPA overlap syndrome. We present the case of a 40-year-old female with a history of rheumatoid arthritis and recurrent pneumonia, admitted with worsening dyspnea, peripheral eosinophilia, and high titers of anti-PR3-ANCA. Bronchoalveolar lavage confirmed eosinophilic infiltration, leading to an initial diagnosis of GPA with eosinophilia. The patient responded well to corticosteroids, rituximab, and avacopan, with significant clinical improvement. This case underscores the importance of integrating clinical, serologic, and histopathologic findings when diagnosing ANCA-associated vasculitis, particularly in patients with eosinophilia and asthma-like symptoms. Recognizing GPA with eosinophilia as distinct from true GPA/EGPA overlap is crucial for prognosis and treatment decisions.
肉芽肿性多血管炎（GPA）和嗜酸性肉芽肿性多血管炎（EGPA）是抗中性粒细胞胞浆抗体（ANCA）相关血管炎的不同但有重叠的形式，每种都有独特的临床特征。GPA通常累及上、下呼吸道的坏死性肉芽肿性炎症，与胞浆型ANCA（c-ANCA）和蛋白酶3-ANCA（PR3-ANCA）阳性密切相关，而EGPA的特征是嗜酸性粒细胞增多、哮喘和血管炎。罕见的GPA病例可出现嗜酸性粒细胞增多，这使得区分这些实体变得复杂，并增加了GPA/EGPA重叠综合征的可能性。我们报告一例40岁女性，有类风湿关节炎和复发性肺炎病史，因呼吸困难加重、外周血嗜酸性粒细胞增多和高滴度抗PR3-ANCA入院。支气管肺泡灌洗证实有嗜酸性粒细胞浸润，最初诊断为伴有嗜酸性粒细胞增多的GPA。患者对糖皮质激素、利妥昔单抗和阿伐可泮反应良好，临床有显著改善。该病例强调了在诊断ANCA相关血管炎时整合临床、血清学和组织病理学结果的重要性，特别是在有嗜酸性粒细胞增多和哮喘样症状的患者中。认识到伴有嗜酸性粒细胞增多的GPA与真正的GPA/EGPA重叠不同，对预后和治疗决策至关重要。
