Shrimukta Sahoo, Puneet Saxena, Akhil K Ravi, Saurabh Tiwari, Valliappan Muthu, Ravi Charan Avala, Vishwanath Gella, Raghava Rao Gandra, Robin Choudhary
{"title":"自身免疫性肺泡蛋白沉积症的自发消退:一个病例系列。","authors":"Shrimukta Sahoo, Puneet Saxena, Akhil K Ravi, Saurabh Tiwari, Valliappan Muthu, Ravi Charan Avala, Vishwanath Gella, Raghava Rao Gandra, Robin Choudhary","doi":"10.1016/j.chest.2025.01.030","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":9782,"journal":{"name":"Chest","volume":"167 6","pages":"e177-e181"},"PeriodicalIF":8.6000,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Spontaneous Resolution in Autoimmune Pulmonary Alveolar Proteinosis: A Case Series.\",\"authors\":\"Shrimukta Sahoo, Puneet Saxena, Akhil K Ravi, Saurabh Tiwari, Valliappan Muthu, Ravi Charan Avala, Vishwanath Gella, Raghava Rao Gandra, Robin Choudhary\",\"doi\":\"10.1016/j.chest.2025.01.030\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":9782,\"journal\":{\"name\":\"Chest\",\"volume\":\"167 6\",\"pages\":\"e177-e181\"},\"PeriodicalIF\":8.6000,\"publicationDate\":\"2025-06-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Chest\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1016/j.chest.2025.01.030\",\"RegionNum\":1,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"CRITICAL CARE MEDICINE\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Chest","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1016/j.chest.2025.01.030","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"CRITICAL CARE MEDICINE","Score":null,"Total":0}
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.
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.