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特发性肺泡蛋白沉积症发病机制研究与治疗进展
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- Author:
- No author available
- Journal Title:
- Chinese Journal of Tuberculosis and Respiratory Diseases
- Issue:
- 12
- DOI:
- 10.3760/cma.j.issn.1001-0939.2016.12.013
- Key Word:
- No keyword available
Abstract： 肺泡蛋白沉积症( pulmonary alveolar proteinosis ,PAP)是一种罕见的肺部疾病,主要病理生理基础是肺泡及细支气管腔内过碘酸雪夫( periodic acid-Schiff, PAS)染色阳性的表面活性蛋白及脂质的过量沉积. 文献报道本病发病率为0.36~0.49/100万至3.7~6.2/100万,中位发病年龄为39岁,男女比例约2.1:1[1-2]. PAP包括先天性、特发性和继发性3种类型. 约90%PAP患者血清中可检测到高滴度抗粒细胞-单核细胞集落刺激因子(GM-CSF)自身抗体[3] ,称为特发性肺泡蛋白沉积症(idiopathic PAP, iPAP)或自身免疫性PAP( autoimmune PAP ). iPAP患者常表现为呼吸困难,部分可合并爆裂音、杵状指和发绀,血清生化可见乳酸脱氢酶升高,肺功能主要表现为限制性通气功能障碍和弥散功能降低,几乎100%患者有高滴度抗GM-CSF IgG抗体[3-4]. 胸部CT可见双侧弥漫肺累及,由广泛镶嵌状磨玻璃影和特征性的多边形小叶间隔增厚构成典型"铺路石"或"霜花"图案.大部分病灶边界清晰,呈"地图征". 相当比例患者未累及肺野外周部分,包括肋膈角、肺尖和胸膜下,称"窄透明带".典型者BALF呈不透明牛奶样,静置后可出现沉淀和半透明上清液,离心后上清液呈无色透明,伴白色沉淀. BALF中包含磷脂和表面活性蛋白A、B和D,卵磷脂和磷脂酰甘油比例较低. 细胞学检查及PAS染色对诊断十分必要. 灌洗液中可见包含嗜酸颗粒的大泡沫状巨噬细胞,细胞外有PAS阳性、黏液卡红和阿尔新蓝染色阴性的均匀球状透明物质[1]. TBLB或手术取得活检为诊断PAP的金标准[5-6]. 但由于PAP并非全肺累及,有可能会出现假阴性的结果. 较为通用的评判疾病程度的方法是日本一项队列研究中提出的,依据"症状和 PaO2"进行疾病严重度评分( disease severity score, DSS) [2,7].
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