白塞病中的肺泡蛋白沉积症。

Alveolar proteinosis in Behçet's disease.

作者信息

Tetikkurt Cuneyt, Tetikkurt Seza, Ozdemir Imran, Zuhur Cigdem, Bayar Nihal

机构信息

Pulmonary Diseases Department, Cerrahpasa Medical Faculty, Istanbul University, Turkey.

出版信息

Multidiscip Respir Med. 2010 Aug 31;5(4):264-6. doi: 10.1186/2049-6958-5-4-264.

DOI:10.1186/2049-6958-5-4-264

PMID:22958601

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3436630/

Abstract

A 51-year-old man with Behçet's disease complained of fever, dry cough and dyspnea during exertion. Chest CT showed ground glass opacities with interstitial septal thickening in both lungs. Bronchoalveolar lavage (BAL) revealed amorphous and lipoproteinaceous material that was periodic acid-Schiff (PAS) stain positive. Transbronchial biopsy specimen demonstrated PAS positive alveolar eosinophilic material consistent with pulmonary alveolar proteinosis. Serum anti-granulocyte-macrophage colony stimulating factor (GM-CSF) antibody was negative. Recent studies have reported anti-GMCSF not present in the the serum of patients with secondary pulmonary alveolar proteinosis (PAP) but they have not reported so in patients with idiopathic PAP. We report a case of alveolar proteinosis in the setting of Behçet's disease with spontaneous remission.

摘要

一名51岁的白塞病男性患者，主诉发热、干咳及劳力性呼吸困难。胸部CT显示双肺磨玻璃影伴间质增厚。支气管肺泡灌洗（BAL）发现无定形和脂蛋白样物质，过碘酸-希夫（PAS）染色呈阳性。经支气管活检标本显示PAS阳性的肺泡嗜酸性物质，符合肺泡蛋白沉积症。血清抗粒细胞-巨噬细胞集落刺激因子（GM-CSF）抗体为阴性。最近的研究报道继发性肺泡蛋白沉积症（PAP）患者血清中不存在抗GM-CSF，但特发性PAP患者未见此报道。我们报告一例白塞病合并肺泡蛋白沉积症且自发缓解的病例。