自身免疫性肺泡蛋白沉积症的自发消退:一个病例系列。

IF 8.6 1区 医学 Q1 CRITICAL CARE MEDICINE
Chest Pub Date : 2025-06-01 DOI:10.1016/j.chest.2025.01.030
Shrimukta Sahoo, Puneet Saxena, Akhil K Ravi, Saurabh Tiwari, Valliappan Muthu, Ravi Charan Avala, Vishwanath Gella, Raghava Rao Gandra, Robin Choudhary
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引用次数: 0

摘要

肺泡蛋白沉积症(PAP)是一种罕见的肺部疾病,其特征是肺泡内蛋白质物质的积累。获得性形式通常是自身免疫性的,由抗粒细胞-巨噬细胞集落刺激因子的自身抗体驱动。自身免疫性PAP的表现可能从无症状患者的偶然发现到晚期呼吸衰竭。治疗通常包括全肺灌洗或粒细胞-巨噬细胞集落刺激因子治疗;在严重的病例中,自发消退是罕见的。在这里,我们报告了3例自身免疫性PAP患者,他们表现为静息缺氧,并在未经显著治疗的情况下自行消退。3例均有职业性吸入有毒气体暴露史,持续停止接触后病情有所改善。我们假设职业性或环境性有害吸入暴露可能在某些自身免疫性PAP病例的疾病表达中起作用,并且这些病例可能在停止有害暴露后自发消退。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Spontaneous Resolution in Autoimmune Pulmonary Alveolar Proteinosis: A Case Series.

Pulmonary alveolar proteinosis (PAP) is a rare lung condition characterized by the accumulation of proteinaceous material within the alveoli. The acquired form is often autoimmune, driven by autoantibodies against granulocyte-macrophage colony-stimulating factor. Presentation of autoimmune PAP may range from incidental detection in asymptomatic patients to advanced respiratory failure. Treatment typically involves whole lung lavage or granulocyte-macrophage colony-stimulating factor therapy in symptomatic patients; spontaneous resolution is rare in severe cases. Here, we report 3 cases of autoimmune PAP who presented with resting hypoxia and exhibited spontaneous resolution without significant treatment. All 3 cases had a history of occupational inhalational exposure to noxious gases, and they improved following sustained cessation of exposure. We hypothesize that occupational or environmental noxious inhalation exposure may have a role in disease expression in some cases of autoimmune PAP, and such cases may show spontaneous resolution following cessation of the offending exposure.

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来源期刊
Chest
Chest 医学-呼吸系统
CiteScore
13.70
自引率
3.10%
发文量
3369
审稿时长
15 days
期刊介绍: At CHEST, our mission is to revolutionize patient care through the collaboration of multidisciplinary clinicians in the fields of pulmonary, critical care, and sleep medicine. We achieve this by publishing cutting-edge clinical research that addresses current challenges and brings forth future advancements. To enhance understanding in a rapidly evolving field, CHEST also features review articles, commentaries, and facilitates discussions on emerging controversies. We place great emphasis on scientific rigor, employing a rigorous peer review process, and ensuring all accepted content is published online within two weeks.
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