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特发性肺肺泡蛋白沉积症合并特发性肺纤维化及石棉肺样特征 1 例报告

A Case of Autoimmune Pulmonary Alveolar Proteinosis with Pulmonary Fibrosis and Asbestosis-Like Features.

机构信息

Division of Respirology, Neurology and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine.

Department of Radiology and Center for Diagnostic Imaging, Kurume University School of Medicine.

出版信息

Kurume Med J. 2020 Jul 1;66(1):59-63. doi: 10.2739/kurumemedj.MS661005. Epub 2020 Apr 24.

Abstract

A 78-year-old man who had worked in the building industry visited our hospital because of groundglass opacity with smoothly thickened, intralobular interstitial lines and interlobular septal lines on chest high-resolution computed tomography (HRCT). HRCT image also showed a focal area of reticulation and pleural thickening. Lung specimens obtained by surgical lung biopsy showed accumulations of intra-alveolar periodic acid-Schiffpositive materials, usual interstitial pneumonia (UIP)-like subpleural lung fibrosis and asbestos bodies (1 body/cm2 in high-power field, ×400). Serum granulocyte-macrophage colony stimulating factor autoantibody was positive. The patient was diagnosed as having autoimmune pulmonary alveolar proteinosis (PAP) and needed differential diagnosis from secondary PAP caused from pulmonary asbestosis and UIP. Careful observation of the manifestations of pulmonary asbestosis and the progression of fibrosis using HRCT will be necessary in this patient.

摘要

一位 78 岁的男性,曾从事建筑行业,因胸部高分辨率计算机断层扫描(HRCT)显示磨玻璃样混浊伴小叶内及小叶间隔增厚的线样混浊,而到我院就诊。HRCT 图像还显示局灶性网状影和胸膜增厚。手术肺活检获得的肺标本显示肺泡内周期性酸-Schiff 阳性物质堆积、通常间质性肺炎(UIP)样胸膜下肺纤维化和石棉小体(高倍视野 1 个/厘米 2 ,×400)。血清粒细胞-巨噬细胞集落刺激因子自身抗体阳性。该患者被诊断为自身免疫性肺泡蛋白沉积症(PAP),需要与由石棉肺和 UIP 引起的继发性 PAP 进行鉴别诊断。对于该患者,需要使用 HRCT 仔细观察石棉肺的表现和纤维化的进展。

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