摘要
Abstract
Objective To investigate the clinical features of the lung involvement of eosinophilic granulomatosis with polyangiitis ( EGPA) patients, in order to raise the physicians' understanding of this condition. Methods The clinical data of 12 EGPA patients with lung involvement who were hospitalized in Peking Union Medical College Hospital from January 2014 to January 2017 were retrospectively ana-lyzed. At the same time, we manually selected 17 EGPA cases without lung involvement as controls. Results Twelve lung involvement EGPA subjects including 6 males and 6 females with the median age of 46. 5 ( range 25-73 ) years old and median prodromal stage duration of 31 ( range 3-120 ) months were included in this study. 25. 0% cases had positive ANCA test. 83. 3% had constitutional symptoms. Extrathoracic organs involvement included ear-nose-throat ( ENT ) ( 83. 3%) , skin ( 75. 0%) , nervous system ( 75. 0%) , gastrointestinal system ( 41. 7%) , cardiac ( 33. 3%) and kidney ( 8. 3%) . In the lung involved EGPA patients, the occurrence rate of constitutional symptoms ( 83. 3% vs. 29. 4%, P=0. 004) and ENT involvement (83. 3% vs. 35. 3%, P=0. 012) were significantly higher than the control group. The frequency of vascular involvement was significantly lower than the control group ( 0 vs. 52. 9%, P = 0. 003 ). Respiratory symptoms of lung involved EGPA patients included dyspnea ( 100%) , cough and sputum ( 100%) , nasal congestion, runny nose ( 66. 7%) , haemoptysis ( 16. 7%) and chest pain ( 8. 3%) . In lung function test, 55. 6% demonstrated obstructive ventilation dysfunction, 11. 1% were restrictive ventilation dysfunction, 11. 1% were mixed ventilation dysfunction. CT findings included ground-glass opacities ( 75. 0%) , bronchiolitis associated opacities ( 83. 3%) , reticulointerstitial opacities ( 16. 7%) , consolidation ( 8. 3%) . Conclusions With long period of prodromal stage and low ANCA positive rate, EGPA is hard to diagnose in its early stages. EGPA pa-tients with lung involvement have more constitutional symptoms and ENT involvement as well as less pe-ripheral vascular involvement compare to those who without lung involvement. The comprehension of the clinical features especially the CT imaging characteristic in different stages is very important to improve early diagnosis.关键词
嗜酸性肉芽肿性多血管炎/肺部受累/影像学特征/临床特征Key words
eosinophilic granulomatosis with polyangiitis/lung involvement/imaging manifestations/clinical features分类
医药卫生
