{"title":"Multifocal sclerotic lesions in Erdheim-Chester disease successfully treated with tocilizumab.","authors":"Henk-Jan Boiten, E J Libourel","doi":"10.1080/1750743X.2025.2561393","DOIUrl":null,"url":null,"abstract":"<p><p>A 58-year-old woman presented to the outpatient clinic with invalidating bone pain in the proximal lower extremities. Laboratory testing showed increased inflammation parameters with a normocytic anemia. Bone scintigraphy revealed increased uptake in both radii, distal femurs, tibiae, and fibulae. PET/CT scanning showed irregular sclerotic bone lesions without other abnormalities. Multiple bone biopsies showed histiocytes expressing CD68 and Factor XIIIa. Metastatic or hematopoietic neoplasms were ruled out. A diagnosis of Erdheim-Chester disease (ECD) with solely osseous involvement was made. Treatment with tocilizumab weekly, an IL-6 receptor antibody, was successful. Tocilizumab appears to be a promising agent for the treatment of patients with Erdheim-Chester disease with limited organ involvement.</p>","PeriodicalId":13328,"journal":{"name":"Immunotherapy","volume":" ","pages":"1-4"},"PeriodicalIF":2.3000,"publicationDate":"2025-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Immunotherapy","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1080/1750743X.2025.2561393","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"IMMUNOLOGY","Score":null,"Total":0}
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Abstract
A 58-year-old woman presented to the outpatient clinic with invalidating bone pain in the proximal lower extremities. Laboratory testing showed increased inflammation parameters with a normocytic anemia. Bone scintigraphy revealed increased uptake in both radii, distal femurs, tibiae, and fibulae. PET/CT scanning showed irregular sclerotic bone lesions without other abnormalities. Multiple bone biopsies showed histiocytes expressing CD68 and Factor XIIIa. Metastatic or hematopoietic neoplasms were ruled out. A diagnosis of Erdheim-Chester disease (ECD) with solely osseous involvement was made. Treatment with tocilizumab weekly, an IL-6 receptor antibody, was successful. Tocilizumab appears to be a promising agent for the treatment of patients with Erdheim-Chester disease with limited organ involvement.
期刊介绍：
Many aspects of the immune system and mechanisms of immunomodulatory therapies remain to be elucidated in order to exploit fully the emerging opportunities. Those involved in the research and clinical applications of immunotherapy are challenged by the huge and intricate volumes of knowledge arising from this fast-evolving field. The journal Immunotherapy offers the scientific community an interdisciplinary forum, providing them with information on the most recent advances of various aspects of immunotherapies, in a concise format to aid navigation of this complex field.
Immunotherapy delivers essential information in concise, at-a-glance article formats. Key advances in the field are reported and analyzed by international experts, providing an authoritative but accessible forum for this vitally important area of research. Unsolicited article proposals are welcomed and authors are required to comply fully with the journal''s Disclosure & Conflict of Interest Policy as well as major publishing guidelines, including ICMJE and GPP3.
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