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Clinical features and prognosis of microscopic polyangiitis with usual interstitial pneumonia compared with idiopathic pulmonary fibrosis

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单位: [1]Beijing Key Laboratory of Respiratory and Pulmonary Circulation Disorders, Department of Respiratory and Critical Care Medicine, Beijing Chao‐Yang Hospital‐Beijing Institute of Respiratory Medicine, Capital Medical University, Beijing, P.R. China [2]Department of Respiratory Medicine, Beijing Ditan Hospital, Capital Medical University, Beijing, P.R. China [3]Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China–Japan Friendship Hospital, National Clinical Research Centre for Respiratory Medicine, Beijing, P.R. China [4]Department of Radiology and Image, Beijing Chao‐Yang Hospital, Capital Medical University, Beijing, P.R. China
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关键词: ANCA-associated vasculitis idiopathic pulmonary fibrosis microscopic polyangiitis usual interstitial pneumonia

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Introduction Pulmonary involvement in microscopic polyangiitis (MPA) is common, little is known about the clinical features of MPA with interstitial pneumonia (MPA-IP). Objectives This study aimed to explore the prevalence of microscopic polyangiitis associated usual interstitial pneumonia (UIP)(MPA-UIP) and compare its clinical features and prognosis with those of MPA-non-UIP and idiopathic pulmonary fibrosis (IPF). Methods A total of 73 patients with MPA-IP were identified and divided into MPA-UIP patients and MPA-non-UIP patients. The clinical characteristics and survival of MPA-UIP patients were analysed and compared with those of MPA-non-UIP patients and 68 patients with IPF. Results The results showed that 34/73 (47%) MPA-IP patients were classified as MPA-UIP patients. Compared with MPA-non-UIP patients, MPA-UIP patients tend to have longer duration of symptoms prior to diagnosis and usually have pulmonary involvement as the initial presentation. However, they were less likely to have proteinuria and/or hematuria. Compared with IPF patients, MPA-UIP patients usually had multisystem damage, positive anti-neutrophil cytoplasmic autoantibodies and elevated levels of nonspecific inflammatory markers. MPA-UIP death was concentrated mainly in the first 3 months after diagnosis and resulted in a higher early mortality compared with IPF. Conclusion UIP is the most frequent type of MPA-IP. These patients tend to have longer duration of symptoms prior to diagnosis and usually have pulmonary involvement as the first presentation. However, they are less likely to have proteinuria and/or hematuria. MPA patients with UIP can be differentiated from IPF patients through comprehensive analysis of clinical and laboratory findings.

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出版当年[2018]版:
大类 | 3 区 医学
小类 | 4 区 呼吸系统
最新[2025]版:
大类 | 4 区 医学
小类 | 4 区 呼吸系统
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出版当年[2017]版:
Q3 RESPIRATORY SYSTEM
最新[2023]版:
Q3 RESPIRATORY SYSTEM

影响因子: 最新[2023版] 最新五年平均[2021-2025] 出版当年[2017版] 出版当年五年平均[2013-2017] 出版前一年[2016版] 出版后一年[2018版]

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第一作者单位: [1]Beijing Key Laboratory of Respiratory and Pulmonary Circulation Disorders, Department of Respiratory and Critical Care Medicine, Beijing Chao‐Yang Hospital‐Beijing Institute of Respiratory Medicine, Capital Medical University, Beijing, P.R. China [2]Department of Respiratory Medicine, Beijing Ditan Hospital, Capital Medical University, Beijing, P.R. China
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通讯机构: [1]Beijing Key Laboratory of Respiratory and Pulmonary Circulation Disorders, Department of Respiratory and Critical Care Medicine, Beijing Chao‐Yang Hospital‐Beijing Institute of Respiratory Medicine, Capital Medical University, Beijing, P.R. China [3]Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China–Japan Friendship Hospital, National Clinical Research Centre for Respiratory Medicine, Beijing, P.R. China [*1]Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China– Japan Friendship Hospital, National Clinical Research Centre for Respiratory Medicine, 2 Yinghuayuan Dongjie, Beijing 100029, P.R. China.
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