Zeinab El Mawla, Ghinwa Hammoud, Racha Abed El Hamid, Abbas Zreik, Ali Tfayli, Bassam Mansour
{"title":"孤立性保奇免疫性肺毛细血管炎的致命结局:病例报告","authors":"Zeinab El Mawla, Ghinwa Hammoud, Racha Abed El Hamid, Abbas Zreik, Ali Tfayli, Bassam Mansour","doi":"10.1002/rcr2.70051","DOIUrl":null,"url":null,"abstract":"<p><p>Isolated Pauci-immune pulmonary capillaritis (IPIPC) is a rare form of small vessel vasculitis that affects only the lungs, causing inflammation of pulmonary capillaries and potentially leading to severe outcomes like alveolar haemorrhage. A 23-year-old woman with a prior diagnosis of rheumatoid arthritis presented with hemoptysis and respiratory distress, ultimately diagnosed with IPIPC. Despite treatment with high-dose steroids and intravenous immunoglobulin, her condition deteriorated, resulting in respiratory failure and death. IPIPC often lacks systemic symptoms and ANCA positivity, complicating diagnosis and treatment. Imaging, bronchoscopy, and histopathology are key for diagnosis, while management typically involves corticosteroids and possibly immunosuppressives. The case underscores the challenges in identifying and treating IPIPC, highlighting the importance of early intervention to improve prognosis, even though complications can still lead to significant respiratory issues and mortality.</p>","PeriodicalId":45846,"journal":{"name":"Respirology Case Reports","volume":"12 10","pages":"e70051"},"PeriodicalIF":0.8000,"publicationDate":"2024-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11486526/pdf/","citationCount":"0","resultStr":"{\"title\":\"Fatal outcome in isolated Pauci-immune pulmonary capillaritis: A case report.\",\"authors\":\"Zeinab El Mawla, Ghinwa Hammoud, Racha Abed El Hamid, Abbas Zreik, Ali Tfayli, Bassam Mansour\",\"doi\":\"10.1002/rcr2.70051\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Isolated Pauci-immune pulmonary capillaritis (IPIPC) is a rare form of small vessel vasculitis that affects only the lungs, causing inflammation of pulmonary capillaries and potentially leading to severe outcomes like alveolar haemorrhage. A 23-year-old woman with a prior diagnosis of rheumatoid arthritis presented with hemoptysis and respiratory distress, ultimately diagnosed with IPIPC. Despite treatment with high-dose steroids and intravenous immunoglobulin, her condition deteriorated, resulting in respiratory failure and death. IPIPC often lacks systemic symptoms and ANCA positivity, complicating diagnosis and treatment. Imaging, bronchoscopy, and histopathology are key for diagnosis, while management typically involves corticosteroids and possibly immunosuppressives. The case underscores the challenges in identifying and treating IPIPC, highlighting the importance of early intervention to improve prognosis, even though complications can still lead to significant respiratory issues and mortality.</p>\",\"PeriodicalId\":45846,\"journal\":{\"name\":\"Respirology Case Reports\",\"volume\":\"12 10\",\"pages\":\"e70051\"},\"PeriodicalIF\":0.8000,\"publicationDate\":\"2024-10-17\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11486526/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Respirology Case Reports\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1002/rcr2.70051\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/10/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q4\",\"JCRName\":\"RESPIRATORY SYSTEM\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Respirology Case Reports","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1002/rcr2.70051","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/10/1 0:00:00","PubModel":"eCollection","JCR":"Q4","JCRName":"RESPIRATORY SYSTEM","Score":null,"Total":0}
Fatal outcome in isolated Pauci-immune pulmonary capillaritis: A case report.
Isolated Pauci-immune pulmonary capillaritis (IPIPC) is a rare form of small vessel vasculitis that affects only the lungs, causing inflammation of pulmonary capillaries and potentially leading to severe outcomes like alveolar haemorrhage. A 23-year-old woman with a prior diagnosis of rheumatoid arthritis presented with hemoptysis and respiratory distress, ultimately diagnosed with IPIPC. Despite treatment with high-dose steroids and intravenous immunoglobulin, her condition deteriorated, resulting in respiratory failure and death. IPIPC often lacks systemic symptoms and ANCA positivity, complicating diagnosis and treatment. Imaging, bronchoscopy, and histopathology are key for diagnosis, while management typically involves corticosteroids and possibly immunosuppressives. The case underscores the challenges in identifying and treating IPIPC, highlighting the importance of early intervention to improve prognosis, even though complications can still lead to significant respiratory issues and mortality.
期刊介绍:
Respirology Case Reports is an open-access online journal dedicated to the publication of original clinical case reports, case series, clinical images and clinical videos in all fields of respiratory medicine. The Journal encourages the international exchange between clinicians and researchers of experiences in diagnosing and treating uncommon diseases or diseases with unusual presentations. All manuscripts are peer-reviewed through a streamlined process that aims at providing a rapid turnaround time from submission to publication.