Boussoffara L, Boudawara N, Knani J, Zantour B, Khouaja I
Service de pneumologie, hôpital Tahar Sfar de Mahdia, Tunisie. leila
Rev Pneumol Clin. 2011 Jun;67(3):158-62. doi: 10.1016/j.pneumo.2010.04.004. Epub 2010 Nov 30.
Pulmonary alveolar proteinosis (PAP), a rare infiltrative disease of unknown aetiology, is characterized by an accumulation of abnormal lung surfactant in the alveoli. The diagnosis is based on the results of the bronchoalveolar lavage (BAL) and sometimes on the lung biopsy. The authors report the case of a 49-year-old woman who was hospitalized for chronic expectoration of the membranes. The chest X-ray revealed alveolar opacities in the lowest part of the right lung. The chest CT scan detected alveolar ground glass opacities with interlobular thickening involving the middle lobe. The BAL was opaque with periodic acid-Schiff stain-positive acellular material. The anatomopathology analysis of the membranes concludes as to the presence of granular eosinophilic material and the absence of neoplasic cells or hydatidous membranes. The diagnosis of PAP was established. Since functional deterioration was not detected, therapy was based on physiotherapy alone. The evolution was favourable, with the disappearance of the symptomatology and the normalisation of the chest X-ray. This observation shows an unusual presentation of PAP based on membrane expectoration and unusual localized lesions.
肺泡蛋白沉积症（PAP）是一种病因不明的罕见浸润性疾病，其特征是肺泡内异常肺表面活性物质的积聚。诊断基于支气管肺泡灌洗（BAL）结果，有时也基于肺活检。作者报告了一例49岁女性因慢性咳出膜状物而住院的病例。胸部X线显示右肺最低部位有肺泡性混浊。胸部CT扫描检测到中叶有伴有小叶间隔增厚的肺泡磨玻璃样混浊。BAL液呈不透明状，高碘酸-希夫染色显示无细胞物质阳性。对膜状物的解剖病理学分析结果为存在颗粒状嗜酸性物质，且无肿瘤细胞或包虫膜。确诊为PAP。由于未检测到功能恶化，治疗仅基于物理治疗。病情进展良好，症状消失，胸部X线恢复正常。该病例显示了基于咳出膜状物和不寻常局限性病变的PAP的不寻常表现。
