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Roles of the autoantibody against GM-CSF in the pathogenesis, diagnosis, and treatment for idiopathic pulmonary alveolar proteinosis

GM-CSF自身抗体在特发性肺泡蛋白沉积症发病机制、诊断和治疗中的作用

基本信息

批准号：
16390239
负责人：
NAKATA Koh
金额：
$ 9.34万
依托单位：
Niigata University
依托单位国家：
日本
项目类别：
Grant-in-Aid for Scientific Research (B)
财政年份：
2004
资助国家：
日本
起止时间：
2004 至 2005
项目状态：
已结题

项目摘要

Pulmonary alveolar proteinosis (PAP) is a rare clinical syndrome with currently three recognised forms ; congenital, secondary and acquired. More than 90% of cases are of the latter type, which recently has been characterized as an acquired autoimmune disorder. In PAP excess accumulation of surfactant leads to characteristic bilateral airspace opacities on chest radiograph and extensive alveolar densities with thickened interlobular septa on computed tomography scan. PAP typically appears in previously healthy individuals, who present with several months of dyspnoea and impaired pulmonary gas transfer of variable severity. "Milky" appearing, lipid-rich fluid recovered at bronchoalveolar lavage is characteristic, while lung biopsy, when performed, shows minimally inflamed alveoli uniformly filled with amorphous eosinophilic material that on electron microscopy contains characteristic lamellar bodies. Current concepts of pathogenesis in acquired PAP are that the excess surfactant results from impaired clearance by alveolar macrophages, as a result of inhibition by specific polyclonal autoantibodies of trophic physiological signals from the endogenous cytokine granulocyte-macrophage colony-stimulating factor (GM-CSF). Current therapy consists of whole-lung lavage under general anesthesia, repeated as needed by clinical status, while some adult patients also respond to the repeated administration of recombinant human GM-CSF.

肺泡蛋白沉积症(PAP)是一种罕见的临床综合征，目前公认的三种类型：先天性、继发性和获得性。超过90%的病例是后一种类型，最近被描述为获得性自身免疫性疾病。在PAP中，表面活性物质的过度积聚导致胸部X线片上特征性的双侧空域混浊和CT扫描上广泛的肺泡密度和小叶间隔增厚。PAP通常出现在以前健康的人身上，他们有几个月的呼吸困难和不同严重程度的肺气体转运障碍。“乳白色”出现，在支气管肺泡灌洗中恢复的富脂液体是特征，而肺活检时，肺活检显示轻度炎症的肺泡均匀填充无定形嗜酸性物质，在电子显微镜下含有特征性的板层小体。目前关于获得性PAP发病机制的观点是，肺泡巨噬细胞对内源性细胞因子粒细胞-巨噬细胞集落刺激因子(GM-CSF)的营养生理信号的特异性多克隆自身抗体抑制，导致肺泡巨噬细胞清除功能受损。目前的治疗方法是在全身麻醉下进行全肺灌洗，根据临床情况重复使用，而一些成人患者也对重复使用重组人GM-CSF有反应。

项目成果

期刊论文数量（19）

专著数量（0）

科研奖励数量（0）

会议论文数量（0）

专利数量（0）

DNA sequence diversity of intergenic spacer I region. in the non-lipid-dependent species Malassezia pachydermatis isolated from animals.

基因间间隔区 I 区的 DNA 序列多样性。

DOI：
发表时间：
2005
期刊：
Medical Mycology 43・1
影响因子：
0
作者：
Nakata K.Kanazawa H;Watanabe M.;Seymour JF;Nakata K;Cho k;Tazawa R;Sugita T
通讯作者：
Sugita T

A case of idiopathic pulmonary alveolar proteinosis accompanied by T-cell receptor gene rearrangement in bronchoalveolar lavage fluid cells

伴有支气管肺泡灌洗液细胞T细胞受体基因重排的特发性肺泡蛋白沉积症一例

DOI：
发表时间：
2004
期刊：
Respirology 9・2
影响因子：
0
作者：
Tazawa R;Hamano E;Nakata K(corresponding author);Nukiwa T.;Tazawa R;Sugita T;Presneill JJ;Uchida K;Hosokawa T
通讯作者：
Hosokawa T

Granulocyte-macrophage colony-stimulating factor and lung immunity in pulmonary alveolar proteinosis