Clinical characteristics of 33 patients with eosinophilic granulomatosis with polyangiitis
10.3969/j.issn.1672-8467.2025.01.010
- VernacularTitle:33例嗜酸性肉芽肿性多血管炎患者的临床特征分析
- Author:
Ming-lang ZHAN
1
;
Lin-di JIANG
;
Yun LIU
Author Information
1. 复旦大学附属中山医院厦门医院风湿免疫科 厦门 361015
- Publication Type:Journal Article
- Keywords:
eosinophilic granulomatosis with polyangiitis(EGPA);
antineutrophil cytoplasmic antibody(ANCA);
tofacitinib
- From:
Fudan University Journal of Medical Sciences
2025;52(1):77-82
- CountryChina
- Language:Chinese
-
Abstract:
Objective To provide a comprehensive description and summary of the clinical characteristics of eosinophilic granulomatosis with polyangiitis(EGPA)in order to enhance understanding of this disease.Methods A total of 33 EGPA patients treated in Zhongshan Hospital,Fudan University,between Jan 2017 and Aug 2022 were included in this retrospective analysis.The diagnosis was based on the 1990 American College of Rheumatology(ACR)classification criteria for EGPA.Clinical manifestations,laboratory examinations,and treatment outcomes of the patients were analyzed.Results Among the 33 EGPA patients,there were 22 males(66.7%)and 11 females(33.3%),with an average age of diagnosis being(47.42±15.83)years old.The most common initial department visited by patients was the rheumatology department(23 cases,69.7%),followed by the respiratory medicine department(6 cases,18.2%).Skin involvement manifested as rash,ulcers,necrosis or gangrene was observed in most cases(23 cases,69.7%),followed by asthma(17 cases,51.5%),infiltrative pneumonia(14 cases,42.4%),peripheral neuropathy(9 cases,27.3%),thrombosis formation(9 cases,27.3%).The mean absolute value of eosinophils in all patients was measured as(3.43±3.52)×109/L,with eight patients(24.2%)testing positive for antineutrophil cytoplasmic antibody(ANCA).Compared with ANCA-negative patients,ANCA-positive individuals exhibited significantly higher Birmingham Vasculitis Activity Score(BVAS)and eosinophil count,as well as a higher incidence rate of renal involvement(P<0.05).Glucocorticoid therapy was administered in thirty-two patients(97%),while biologics or tofacitinib were given to eleven patients(33.3%),among them six received tofacitinib treatment,of which five achieved disease remission.Conclusion EGPA exhibits a wide range of clinical manifestations,and ANCA-positive patients tend to exhibit higher disease activity levels.A multidisciplinary diagnosis and treatment system for EGPA should be established.
