{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:17:23Z","timestamp":1784557043790,"version":"3.55.0"},"reference-count":20,"publisher":"Bioscientifica","content-domain":{"domain":["edm.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2015,3,1]]},"abstract":"Summary<\/jats:title>\n Erdheim\u2013Chester disease (ECD) is a potentially fatal condition characterized by infiltration of multiple organs by non-Langerhans histiocytes. Although endocrine dysfunction has been reported in association with ECD, to date, there have been no previous reports of empty sella syndrome (ESS) associated with it. We report the case of a patient with ECD who had symptomatic ESS. A 55-year-old man of Chinese ethnicity initially presented with symptoms of heart failure, fatigue and knee joint pain. Physical examination revealed xanthelasma, gynaecomastia, lung crepitations, hepatomegaly and diminished testicular volumes. He had laboratory evidence of hypogonadotrophic hypogonadism, secondary hypoadrenalism and GH deficiency. Imaging studies showed diffuse osteosclerosis of the long bones on X-ray, a mass in the right atrium and thickening of the pleura and of the thoracic aorta on fusion positron emission tomography\u2013computed tomography. Magnetic resonance imaging (MRI) of the brain showed an empty sella. The diagnosis of ECD was confirmed by bone biopsy.<\/jats:p>\n \n Learning points<\/jats:title>\n \n \n \n \n ECD is a multisystemic disease that can affect the pituitary and other organs. The diagnosis of ECD is based on clinical and radiological features and histology, showing lipid-laden CD68\n +<\/jats:sup>\n CD1a\n \u2212<\/jats:sup>\n S100\n \u2212<\/jats:sup>\n histiocytes surrounded by fibrosis.\n <\/jats:p>\n <\/jats:list-item>\n \n The finding of xanthelasmas especially in the presence of normal lipid levels in the presence of a multisystem infiltrative disorder should raise the suspicion of ECD.<\/jats:p>\n <\/jats:list-item>\n \n Systemic perturbation of autoimmunity may play a role in the pathogenesis of ECD and is an area that merits further research.<\/jats:p>\n <\/jats:list-item>\n <\/jats:list>\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1530\/edm-14-0122","type":"journal-article","created":{"date-parts":[[2015,3,24]],"date-time":"2015-03-24T11:06:37Z","timestamp":1427195197000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-2","source":"Crossref","is-referenced-by-count":1,"title":["Symptomatic empty sella syndrome: an unusual manifestation of Erdheim\u2013Chester disease"],"prefix":"10.1530","volume":"2015","author":[{"given":"Wann Jia","family":"Loh","sequence":"first","affiliation":[{"name":"1 Osteoporosis and Bone Metabolism Unit, Department of Endocrinology, Singapore General Hospital, Academia, 20, College Road, Singapore, 169856, Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kesavan","family":"Sittampalam","sequence":"additional","affiliation":[{"name":"2 Department of Pathology, Singapore General Hospital, Academia, 20, College Road, Singapore, 169856, Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suan Cheng","family":"Tan","sequence":"additional","affiliation":[{"name":"3 Department of Radiology, Singapore General Hospital, Academia, 20, College Road, Singapore, 169856, Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manju","family":"Chandran","sequence":"additional","affiliation":[{"name":"1 Osteoporosis and Bone Metabolism Unit, Department of Endocrinology, Singapore General Hospital, Academia, 20, College Road, Singapore, 169856, Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","reference":[{"key":"ref11","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1111\/j.1365-2796.1998.00389.x","article-title":"Endocrine manifestations of Erdheim\u2013Chester disease (a distinct form of histiocytosis)","volume":"244","year":"1998","journal-title":"Journal of Internal Medicine"},{"key":"ref131","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1111\/j.1365-2265.1992.tb01484.x","article-title":"The empty sella results of treatment in 76 successive cases and high frequency of endocrine and neurological disturbances","volume":"37","year":"1992","journal-title":"Clinical Endocrinology"},{"key":"ref121","first-page":"198","article-title":"Empty sella syndrome","volume":"2","year":"2001","journal-title":"Journal of Indian Academy of Clinical Medicine"},{"key":"ref71","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1097\/01.iop.0000197021.98742.09","article-title":"Association between Erdheim\u2013Chester disease, Hashimoto thyroiditis, and familial thrombocytopenia","volume":"22","year":"2006","journal-title":"Ophthalmic Plastic and Reconstructive Surgery"},{"key":"ref151","doi-asserted-by":"crossref","first-page":"662","DOI":"10.1001\/archneur.1984.04210080070017","article-title":"Empty sella turcica in intracranial sarcoidosis. Pituitary insufficiency, primary polydipsia, and changing neuroradiologic findings","volume":"41","year":"1984","journal-title":"Archives of Neurology"},{"key":"ref181","doi-asserted-by":"crossref","first-page":"2783","DOI":"10.1182\/blood-2010-10-313510","article-title":"Systemic perturbation of cytokine and chemokine networks in Erdheim\u2013Chester disease: a single-center series of 37 patients","volume":"117","year":"2011","journal-title":"Blood"},{"key":"ref111","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1111\/j.1365-2796.1998.00389.x","article-title":"Endocrine manifestations of Erdheim\u2013Chester disease (a distinct form of histiocytosis)","volume":"244","year":"1998","journal-title":"Journal of Internal Medicine"},{"key":"ref161","doi-asserted-by":"crossref","first-page":"472","DOI":"10.1016\/j.jocn.2004.05.022","article-title":"Empty sella hypogonadism and hypopituitarism secondary to moyamoya disease","volume":"12","year":"2005","journal-title":"Journal of Clinical Neuroscience"},{"key":"ref51","doi-asserted-by":"crossref","first-page":"662","DOI":"10.1001\/archneur.1984.04210080070017","article-title":"Empty sella turcica in intracranial sarcoidosis. Pituitary insufficiency, primary polydipsia, and changing neuroradiologic findings","volume":"41","year":"1984","journal-title":"Archives of Neurology"},{"key":"ref141","doi-asserted-by":"crossref","first-page":"bcr0720103203","DOI":"10.1136\/bcr.07.2010.3203","article-title":"Partial hypopituitarism and Langerhans cell histiocytosis","volume":"2011","year":"2011","journal-title":"BMJ Case Reports"},{"key":"ref81","doi-asserted-by":"crossref","first-page":"2783","DOI":"10.1182\/blood-2010-10-313510","article-title":"Systemic perturbation of cytokine and chemokine networks in Erdheim\u2013Chester disease: a single-center series of 37 patients","volume":"117","year":"2011","journal-title":"Blood"},{"key":"ref01","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1097\/00005792-199605000-00005","article-title":"Erdheim\u2013Chester disease. Clinical and radiologic characteristics of 59 cases","volume":"75","year":"1996","journal-title":"Medicine"},{"key":"ref171","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1097\/01.iop.0000197021.98742.09","article-title":"Association between Erdheim\u2013Chester disease, Hashimoto thyroiditis, and familial thrombocytopenia","volume":"22","year":"2006","journal-title":"Ophthalmic Plastic and Reconstructive Surgery"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1111\/j.1365-2265.1992.tb01484.x","article-title":"The empty sella results of treatment in 76 successive cases and high frequency of endocrine and neurological disturbances","volume":"37","year":"1992","journal-title":"Clinical Endocrinology"},{"key":"ref191","first-page":"3655","article-title":"Thyrotropin-receptor and thyroid peroxidase-specific T cell clones and their cytokine profile in autoimmune thyroid disease","volume":"82","year":"1997","journal-title":"Journal of Clinical Endocrinology and Metabolism"},{"key":"ref41","doi-asserted-by":"crossref","first-page":"bcr0720103203","DOI":"10.1136\/bcr.07.2010.3203","article-title":"Partial hypopituitarism and Langerhans cell histiocytosis","volume":"2011","year":"2011","journal-title":"BMJ Case Reports"},{"key":"ref21","first-page":"198","article-title":"Empty sella syndrome","volume":"2","year":"2001","journal-title":"Journal of Indian Academy of Clinical Medicine"},{"key":"ref91","first-page":"3655","article-title":"Thyrotropin-receptor and thyroid peroxidase-specific T cell clones and their cytokine profile in autoimmune thyroid disease","volume":"82","year":"1997","journal-title":"Journal of Clinical Endocrinology and Metabolism"},{"key":"ref101","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1097\/00005792-199605000-00005","article-title":"Erdheim\u2013Chester disease. 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