Berivan Bitik, Mustafa Şenturk, Seda Kibaroglu, Tulin Yildirim, Mehmet Engin Tezcan, Pınar Zeyneloglu, Ahmet Eftal Yucel
{"title":"静脉注射 Anakinra 用于治疗成人型 Still's 病的巨噬细胞活化综合征。","authors":"Berivan Bitik, Mustafa Şenturk, Seda Kibaroglu, Tulin Yildirim, Mehmet Engin Tezcan, Pınar Zeyneloglu, Ahmet Eftal Yucel","doi":"10.12890/2024_004788","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disease characterized by fever, rash, arthritis, and multi-organ involvement. Macrophage activation syndrome (MAS), a serious complication of AOSD, poses significant diagnostic and therapeutic challenges.</p><p><strong>Case presentation: </strong>A 32-year-old male was diagnosed with AOSD in 2020 after being hospitalized for a fever of unknown origin and elevated liver enzymes. The patient was initially treated with corticosteroids and methotrexate but subsequently discontinued both treatment and follow-up. In September 2023, he presented with fever, sore throat, and elevated inflammatory markers. After screening for infections, methylprednisolone (MP) treatment was initiated because of AOSD activation. The following day, the patient was admitted to the intensive care unit due to an altered state of consciousness. Brain magnetic resonance imaging revealed brainstem involvement. Empirical treatments were initiated, including intravenous MP, and immunoglobulin therapy. Due to suspected macrophage activation syndrome (MAS), anakinra (ANA) infusion was initiated. Significant improvement was observed after the ANA infusion.</p><p><strong>Conclusion: </strong>This case highlights the complex management of severe AOSD complications, emphasizing the role of early recognition, aggressive therapy, and multidisciplinary care in improving outcomes.</p><p><strong>Learning points: </strong>Macrophage activation syndrome (MAS) is a serious complication of adult-onset Still's disease characterized by systemic inflammation. Early recognition and prompt initiation of treatment are crucial due to the high mortality rate associated with MAS, especially when neurologic symptoms are present.Clinicians should not delay treatment pending confirmatory diagnostic tests when MAS is suspected, as early intervention can significantly impact patient outcomes.Anakinra, an interleukin-1 inhibitor, is typically administered subcutaneously but has shown promise when administered intravenously, particularly in severe cases of MAS.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 11","pages":"004788"},"PeriodicalIF":0.0000,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11542943/pdf/","citationCount":"0","resultStr":"{\"title\":\"Intravenous Anakinra for Treating Macrophage Activation Syndrome in Adult-Onset Still's Disease.\",\"authors\":\"Berivan Bitik, Mustafa Şenturk, Seda Kibaroglu, Tulin Yildirim, Mehmet Engin Tezcan, Pınar Zeyneloglu, Ahmet Eftal Yucel\",\"doi\":\"10.12890/2024_004788\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disease characterized by fever, rash, arthritis, and multi-organ involvement. Macrophage activation syndrome (MAS), a serious complication of AOSD, poses significant diagnostic and therapeutic challenges.</p><p><strong>Case presentation: </strong>A 32-year-old male was diagnosed with AOSD in 2020 after being hospitalized for a fever of unknown origin and elevated liver enzymes. The patient was initially treated with corticosteroids and methotrexate but subsequently discontinued both treatment and follow-up. In September 2023, he presented with fever, sore throat, and elevated inflammatory markers. After screening for infections, methylprednisolone (MP) treatment was initiated because of AOSD activation. The following day, the patient was admitted to the intensive care unit due to an altered state of consciousness. Brain magnetic resonance imaging revealed brainstem involvement. Empirical treatments were initiated, including intravenous MP, and immunoglobulin therapy. Due to suspected macrophage activation syndrome (MAS), anakinra (ANA) infusion was initiated. Significant improvement was observed after the ANA infusion.</p><p><strong>Conclusion: </strong>This case highlights the complex management of severe AOSD complications, emphasizing the role of early recognition, aggressive therapy, and multidisciplinary care in improving outcomes.</p><p><strong>Learning points: </strong>Macrophage activation syndrome (MAS) is a serious complication of adult-onset Still's disease characterized by systemic inflammation. Early recognition and prompt initiation of treatment are crucial due to the high mortality rate associated with MAS, especially when neurologic symptoms are present.Clinicians should not delay treatment pending confirmatory diagnostic tests when MAS is suspected, as early intervention can significantly impact patient outcomes.Anakinra, an interleukin-1 inhibitor, is typically administered subcutaneously but has shown promise when administered intravenously, particularly in severe cases of MAS.</p>\",\"PeriodicalId\":11908,\"journal\":{\"name\":\"European journal of case reports in internal medicine\",\"volume\":\"11 11\",\"pages\":\"004788\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-10-10\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11542943/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"European journal of case reports in internal medicine\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.12890/2024_004788\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/1/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q3\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"European journal of case reports in internal medicine","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.12890/2024_004788","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/1/1 0:00:00","PubModel":"eCollection","JCR":"Q3","JCRName":"Medicine","Score":null,"Total":0}
Intravenous Anakinra for Treating Macrophage Activation Syndrome in Adult-Onset Still's Disease.
Background: Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disease characterized by fever, rash, arthritis, and multi-organ involvement. Macrophage activation syndrome (MAS), a serious complication of AOSD, poses significant diagnostic and therapeutic challenges.
Case presentation: A 32-year-old male was diagnosed with AOSD in 2020 after being hospitalized for a fever of unknown origin and elevated liver enzymes. The patient was initially treated with corticosteroids and methotrexate but subsequently discontinued both treatment and follow-up. In September 2023, he presented with fever, sore throat, and elevated inflammatory markers. After screening for infections, methylprednisolone (MP) treatment was initiated because of AOSD activation. The following day, the patient was admitted to the intensive care unit due to an altered state of consciousness. Brain magnetic resonance imaging revealed brainstem involvement. Empirical treatments were initiated, including intravenous MP, and immunoglobulin therapy. Due to suspected macrophage activation syndrome (MAS), anakinra (ANA) infusion was initiated. Significant improvement was observed after the ANA infusion.
Conclusion: This case highlights the complex management of severe AOSD complications, emphasizing the role of early recognition, aggressive therapy, and multidisciplinary care in improving outcomes.
Learning points: Macrophage activation syndrome (MAS) is a serious complication of adult-onset Still's disease characterized by systemic inflammation. Early recognition and prompt initiation of treatment are crucial due to the high mortality rate associated with MAS, especially when neurologic symptoms are present.Clinicians should not delay treatment pending confirmatory diagnostic tests when MAS is suspected, as early intervention can significantly impact patient outcomes.Anakinra, an interleukin-1 inhibitor, is typically administered subcutaneously but has shown promise when administered intravenously, particularly in severe cases of MAS.
期刊介绍：
The European Journal of Case Reports in Internal Medicine is an official journal of the European Federation of Internal Medicine (EFIM), representing 35 national societies from 33 European countries. The Journal''s mission is to promote the best medical practice and innovation in the field of acute and general medicine. It also provides a forum for internal medicine doctors where they can share new approaches with the aim of improving diagnostic and clinical skills in this field. EJCRIM welcomes high-quality case reports describing unusual or complex cases that an internist may encounter in everyday practice. The cases should either demonstrate the appropriateness of a diagnostic/therapeutic approach, describe a new procedure or maneuver, or show unusual manifestations of a disease or unexpected reactions. The Journal only accepts and publishes those case reports whose learning points provide new insight and/or contribute to advancing medical knowledge both in terms of diagnostics and therapeutic approaches. Case reports of medical errors, therefore, are also welcome as long as they provide innovative measures on how to prevent them in the current practice (Instructive Errors). The Journal may also consider brief and reasoned reports on issues relevant to the practice of Internal Medicine, as well as Abstracts submitted to the scientific meetings of acknowledged medical societies.
求助内容：
应助结果提醒方式：
京公网安备 11010802042870号
