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慢性气道疾病患者中显微镜下多血管炎的发生

Development of microscopic polyangiitis in patients with chronic airway disease.

作者信息

Takahashi K, Hayashi S, Ushiyama O, Sueoka N, Fukuoka M, Nagasawa K

机构信息

Department of Medicine, Saga University Medical School, Nabeshima 5-1-1, Saga 849-8501, Japan.

出版信息

Lung. 2005 Jul-Aug;183(4):273-81. doi: 10.1007/s00408-004-2540-1.

DOI:10.1007/s00408-004-2540-1
PMID:16211463
Abstract

Microscopic polyangiitis (MPA) is a rare systemic vasculitis syndrome, which is often accompanied by positive myeloperoxidase-specific antineutrophil cytoplasmic antibody (MPO-ANCA). While pulmonary involvement of MPA consists mainly of diffuse alveolar hemorrhage and interstitial pneumonia, bronchiectasis has been reported as a pulmonary lesion in association with MPA. To investigate the clinical features of patients with MPA, focusing on the presence or the absence of preceding chronic airway diseases (CAD), we conducted a retrospective observational study of 26 patients in the last 13 years at Saga University Hospital. The clinical records and radiologic chest examinations were reviewed retrospectively. Pulmonary manifestations were alveolar hemorrhage in 3 patients (12%) and interstitial pneumonia in 5 (19%). Bronchiectasis, defined by the findings of chest radiograph and computed tomography, was found in 9 patients (35%). Four patients (15%) with bronchiectasis and one patient (4%) with chronic bronchitis had experienced chronic bronchial suppuration prior to the onset of MPA. Ten patients were classified as having chronic airway disease (CAD) before the onset of MPA. MPO-ANCA tended to be lower in the CAD group than in the non-CAD group. None of the patients in the CAD group had pulmonary hemorrhage or interstitial pneumonia. Only one patient (10%) in the CAD group died within 90 days of the onset of MPA, while 7 (43.8%) of the non-CAD group died. Our study suggests that MPA may result in part from CAD and that the clinical course of MPA with CAD may be different from MPA without CAD.

摘要

显微镜下多血管炎(MPA)是一种罕见的系统性血管炎综合征,常伴有髓过氧化物酶特异性抗中性粒细胞胞浆抗体(MPO-ANCA)阳性。MPA的肺部受累主要包括弥漫性肺泡出血和间质性肺炎,支气管扩张也被报道为与MPA相关的肺部病变。为了研究MPA患者的临床特征,重点关注是否存在先前的慢性气道疾病(CAD),我们对佐贺大学医院过去13年中的26例患者进行了一项回顾性观察研究。对临床记录和胸部影像学检查进行了回顾性分析。肺部表现为3例(12%)肺泡出血和5例(19%)间质性肺炎。根据胸部X线片和计算机断层扫描结果定义的支气管扩张在9例患者(35%)中发现。4例(15%)支气管扩张患者和1例(4%)慢性支气管炎患者在MPA发病前有慢性支气管化脓病史。10例患者在MPA发病前被归类为患有慢性气道疾病(CAD)。CAD组的MPO-ANCA水平往往低于非CAD组。CAD组患者均无肺出血或间质性肺炎。CAD组中只有1例患者(10%)在MPA发病后90天内死亡,而非CAD组有7例(43.8%)死亡。我们的研究表明,MPA可能部分由CAD引起,并且CAD患者的MPA临床病程可能与无CAD的MPA患者不同。

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