Clinical and prognostic characteristics in patients with eosinophilic granulomatosis with polyangitis
10.3760/cma.j.cn112138-20191111-00742
- VernacularTitle:嗜酸性肉芽肿性多血管炎146例患者临床特征分析
- Author:
Jing CHEN
1
;
Jing LI
;
Yunjiao YANG
;
Xinping TIAN
;
Xiaofeng ZENG
Author Information
1. 中国医学科学院 北京协和医学院 北京协和医院风湿免疫科 国家皮肤与免疫疾病临床医学研究中心 100730
- From:
Chinese Journal of Internal Medicine
2020;59(5):360-365
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To analyze the clinical and prognostic characteristics in patients with eosinophilic granulomatosis with polyangitis (EGPA).Methods:The clinical data of 146 EGPA patients hospitalized in Peking Union Medical College Hospital from 2000 to 2019 were analyzed retrospectively, including clinical manifestations, laboratory results, treatment, complications and outcome at discharge. Birmingham Vasculitis activity score-V3 (BVAS-V3) was used to evaluate disease activity.Results:The ratio of male to female was 1.8∶1 with average age (41.7±16.1) year-old. The median time from disease onset to diagnosis was 18(6, 60) months (0.5~450). The most common clinical manifestations were lung [121(82.9%)] and nose/paranasal sinuses [119(81.5%)] involvement. The positive rate of anti-neutrophil cytoplasmic antibody (ANCA) was 24.7%, mainly peripheral (P)-ANCA/myeloperoxidase (MPO)-ANCA. Compared with ANCA-negative patients, the ANCA-positive patients had a higher incidence of renal involvement and nervous system involvement (66.7% vs. 20.9%, 80.6% vs. 51.8%, P<0.001), fever and optic neuropathy (66.7% vs. 40.9%,8.3% vs. 0, P<0.05), more active disease [median BVAS-V3 25(18,30)vs. 19(14,24), P=0.001] and more elevated erythrocyte sedimentation rate [40.5(20.5,82.8)mm/1h vs. 25.0(13.3,50.8)mm/1h, P=0.006] and C-reactive protein [37.1(11.8,72.9)mg/L vs.13.5(3.4,66.1)mg/L, P=0.036]. More ANCA-negative patients had pleural effusion (20.9% vs. 5.6%, P<0.04) compared with ANCA-negative patients. Pulmonary infection was the most common complication. A total of 12 EGPA patients (8.2%)achieved remission and 6 patients (4.1%)died or discharged themselves from the hospital. Conclusion:EGPA is a rare small vessel vasculitis. The clinical manifestations and outcomes are heterogenous. The mortality rate of EGPA is high.
