{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,8]],"date-time":"2026-05-08T21:25:27Z","timestamp":1778275527658,"version":"3.51.4"},"reference-count":31,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2021,8,22]],"date-time":"2021-08-22T00:00:00Z","timestamp":1629590400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100004826","name":"Natural Science Foundation of Beijing Municipality","doi-asserted-by":"publisher","award":["7202160"],"award-info":[{"award-number":["7202160"]}],"id":[{"id":"10.13039\/501100004826","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2021,9]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Erdheim\u2013Chester disease (ECD) is a rare form of non\u2010Langerhans cell histiocytosis that typically affects many organs, including the lung and pleura. However, there are few studies concerning pulmonary involvement in ECD patients, as well as the difference of pulmonary involvement between ECD and Langerhans cell histiocytosis (LCH). We performed a retrospective study of 54 ECD patients, and compared the pulmonary manifestations with those of adult LCH patients in our centre. The median age of diagnosis of the 54 ECD patients was 48\u00a0years (range 9\u201366\u00a0years). Chest computed tomography (CT) scans revealed lung involvement in 49 (91%) patients and pleural involvement in 34 (63%). Thirty\u2010three (61%) patients had interstitial lung disease (ILD) with varying degrees of interlobular septal thickening, micronodules, and ground\u2010glass opacities. ECD and LCH patients with pulmonary involvement showed significant differences in smoking status (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00\u00b7001), respiratory symptoms (<jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b7001) such as cough and pneumothorax (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00\u00b7001), and radiological findings, including cysts (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00\u00b7001), opacities (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00\u00b7001), and pleural thickening (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00\u00b7001). With a median follow\u2010up duration of 24\u00a0months (range, 1\u201384\u00a0months), the estimated three\u2010year overall survival (OS) of this entire ECD cohort was 90\u00b72%. Patients with ILD tended to have worse progression\u2010free survival (PFS) than those with no ILD (<jats:italic>P<\/jats:italic>\u00a0=\u00a00\u00b729).<\/jats:p>","DOI":"10.1111\/bjh.17712","type":"journal-article","created":{"date-parts":[[2021,8,23]],"date-time":"2021-08-23T00:51:37Z","timestamp":1629679897000},"page":"1024-1033","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Pulmonary manifestations of Erdheim\u2013Chester disease: clinical characteristics, outcomes and comparison with Langerhans cell histiocytosis"],"prefix":"10.1111","volume":"194","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4036-7547","authenticated-orcid":false,"given":"Ji\u2010nuo","family":"Wang","sequence":"first","affiliation":[{"name":"Department of Hematology State Key Laboratory of Complex Severe and Rare Diseases Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College Beijing China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Feng\u2010dan","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Radiology State Key Laboratory of Complex Severe and Rare Diseases Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College Beijing China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jian","family":"Sun","sequence":"additional","affiliation":[{"name":"Department of Pathology State Key Laboratory of Complex Severe and Rare Diseases Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College Beijing China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhi\u2010yong","family":"Liang","sequence":"additional","affiliation":[{"name":"Department of Pathology State Key Laboratory of Complex Severe and Rare Diseases Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College Beijing China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jian","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Hematology State Key Laboratory of Complex Severe and Rare Diseases Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College Beijing China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dao\u2010bin","family":"Zhou","sequence":"additional","affiliation":[{"name":"Department of Hematology State Key Laboratory of Complex Severe and Rare Diseases Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College Beijing China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xinlun","family":"Tian","sequence":"additional","affiliation":[{"name":"Department of Pulmonary and Critical Care Medicine State Key Laboratory of Complex Severe and Rare Diseases Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College Beijing China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xin\u2010xin","family":"Cao","sequence":"additional","affiliation":[{"name":"Department of Hematology State Key Laboratory of Complex Severe and Rare Diseases Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College Beijing 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