Erbay Müge, Bülbül Yılmaz, Öztuna Funda, Kılıç Mehmet, Özçelik Neslihan, Ersöz Şafak
Department of Chest Diseases, Faculty of Medicine, Karadeniz Tecnical University, Trabzon, Turkey.
Department of Pathology, Faculty of Medicine, Karadeniz Tecnical University, Trabzon, Turkey.
Tuberk Toraks. 2017 Sep;65(3):249-254. doi: 10.5578/tt.50708.
Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease characterised by alveolar accumulation of surfactant composed of proteins and lipids. Three main categories of PAP have been defined depending on the aetiology: primer/idiopathic, neonatal/congenital, secondary and exogenous/environmental exposure. Radiologically diffuse ground glass opacities, interlobular and intralobular septal thickening is seen. Although open lung biopsy is accepted as the gold standard in diagnosis, it can be diagnosed by showing bronchoalveolar lavage (BAL) fluid with a milky appearance and periodic acid-schiff (PAS) positive globules in biopsy with clinical and radiological findings. Theraphy for PAP are supportive care (oxygen), total lung lavage, inhale/subcutaneous granulocyte macrophage colony stimulating factor (GM-CSF), rituximab, plasmapheresis, lung transplantation. We report a case of PAP presented with cough and shortness of breath, diagnosed with BAL and transbronchial lung biopsy, treated by total lung lavage by reviewing literature.
肺泡蛋白沉积症(PAP)是一种罕见的肺部疾病,其特征是由蛋白质和脂质组成的表面活性剂在肺泡内积聚。根据病因,PAP主要分为三类:原发性/特发性、新生儿/先天性、继发性以及外源性/环境暴露性。在影像学上可见弥漫性磨玻璃影、小叶间隔和小叶内间隔增厚。尽管开胸肺活检被认为是诊断的金标准,但结合临床和影像学表现,通过显示支气管肺泡灌洗(BAL)液呈乳状外观以及活检中高碘酸-希夫(PAS)阳性小球也可作出诊断。PAP的治疗方法包括支持治疗(吸氧)、全肺灌洗、吸入/皮下注射粒细胞巨噬细胞集落刺激因子(GM-CSF)、利妥昔单抗、血浆置换、肺移植。我们通过回顾文献报道一例以咳嗽和气短为表现的PAP病例,经BAL和经支气管肺活检确诊,并接受了全肺灌洗治疗。