Zhao Wen, Dai Huaping, Liu Yan, Zhu Min, Bao Na, Ban Chengjun, Zhang Shu, Ren Yanhong, Ye Qiao, Wang Chen
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.
Department of Respiratory Medicine, Beijing Ditan Hospital, Capital Medical University, Beijing, P.R. China.
Clin Respir J. 2019 Jul;13(7):460-466. doi: 10.1111/crj.13032. Epub 2019 May 16.
Pulmonary involvement in microscopic polyangiitis (MPA) is common, little is known about the clinical features of MPA with interstitial pneumonia (MPA-IP).
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).
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.
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.
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.
显微镜下多血管炎（MPA）累及肺部很常见，但关于合并间质性肺炎的MPA（MPA-IP）的临床特征知之甚少。
本研究旨在探讨显微镜下多血管炎合并普通型间质性肺炎（UIP）（MPA-UIP）的患病率，并将其临床特征和预后与MPA-非UIP和特发性肺纤维化（IPF）进行比较。
共纳入73例MPA-IP患者，分为MPA-UIP患者和MPA-非UIP患者。分析MPA-UIP患者的临床特征和生存率，并与MPA-非UIP患者和68例IPF患者进行比较。
结果显示，73例MPA-IP患者中有34例（47%）被归类为MPA-UIP患者。与MPA-非UIP患者相比，MPA-UIP患者在诊断前症状持续时间往往更长，且通常以肺部受累为首发表现。然而，他们出现蛋白尿和/或血尿的可能性较小。与IPF患者相比，MPA-UIP患者通常有多系统损害、抗中性粒细胞胞浆自身抗体阳性以及非特异性炎症标志物水平升高。MPA-UIP死亡主要集中在诊断后的前3个月，与IPF相比早期死亡率更高。
UIP是MPA-IP最常见的类型。这些患者在诊断前症状持续时间往往更长，且通常以肺部受累为首发表现。然而，他们出现蛋白尿和/或血尿的可能性较小。通过对临床和实验室检查结果的综合分析，MPA合并UIP患者可与IPF患者相鉴别。
