一例采用肺段灌洗和口服他汀类药物治疗的自身免疫性肺泡蛋白沉积症伴严重呼吸衰竭病例。

A case of autoimmune pulmonary alveolar proteinosis with severe respiratory failure treated with segmental lung lavage and oral statin therapy.

作者信息

Takano Tomotsugu, Takeda Keisuke, Nakamura Satoshi, Akiyama Genta, Ando Nobuhisa, Komori Masashi

机构信息

Department of Respiratory Medicine, Steel Memorial Yawata Hospital, Fukuoka, Japan.

Department of Respiratory Medicine, Japan Community Health Care Organization Kyushu Hospital, Fukuoka, Japan.

出版信息

Respir Med Case Rep. 2022 Jun 6;38:101684. doi: 10.1016/j.rmcr.2022.101684. eCollection 2022.

DOI:10.1016/j.rmcr.2022.101684

PMID:35707405

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9190053/

Abstract

Pulmonary alveolar proteinosis (PAP) is a diffuse lung disease characterized by the accumulation of alveolar surfactants due to dysfunction of granulocyte-macrophage colony-stimulating factor-dependent cholesterol clearance. Whole-lung lavage is the current standard of care for PAP, but it can lead to the exacerbation of hypoxia. A medication targeting cholesterol homeostasis is a promising therapy for refractory PAP. We present a case of autoimmune PAP with severe hypoxia that was successfully treated with segmental lung lavage (SLL). Following SLL for disease relapse, statin treatment for dyslipidemia was started. After initiating statin treatment, the patient did not require bronchoalveolar lavage for 10 months.

摘要

肺泡蛋白沉积症（PAP）是一种弥漫性肺部疾病，其特征是由于粒细胞-巨噬细胞集落刺激因子依赖性胆固醇清除功能障碍，导致肺泡表面活性物质积聚。全肺灌洗是目前PAP的标准治疗方法，但它可能导致缺氧加重。针对胆固醇稳态的药物是治疗难治性PAP的一种有前景的疗法。我们报告一例伴有严重缺氧的自身免疫性PAP患者，经节段性肺灌洗（SLL）成功治疗。在SLL治疗疾病复发后，开始使用他汀类药物治疗血脂异常。开始他汀类药物治疗后，患者10个月未需要进行支气管肺泡灌洗。