{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T20:59:51Z","timestamp":1780088391003,"version":"3.54.0"},"reference-count":18,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2014,1,23]],"date-time":"2014-01-23T00:00:00Z","timestamp":1390435200000},"content-version":"vor","delay-in-days":22,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Internal Medicine Journal"],"published-print":{"date-parts":[[2014,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:styled-content style=\"fixed-case\">E<\/jats:styled-content>rdheim\u2013<jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>hester disease (<jats:styled-content style=\"fixed-case\">ECD<\/jats:styled-content>) is a rare histocytic disorder. We report a case of a 45\u2010year\u2010old male <jats:styled-content style=\"fixed-case\">ECD<\/jats:styled-content> patient with severe clinical manifestation (urinary obstruction due to retroperitoneal mass with hydronephrosis, involvement of long bones) and central nervous system involvement (hemiparesis, aphasia and diabetes insipidus). Diagnosis was confirmed by typical clinical, radiological and histological findings. Under immunosuppressive therapy with prednisolone and interleukin\u2010<jats:styled-content style=\"fixed-case\">1A<\/jats:styled-content> receptor antagonist (<jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>nakinra, Kineret, Swedish Orphan Biovitrum AB, Stockholm, Sweden), a rapid improvement of the patients' symptoms and condition was observed. This is the first report of a successful combination therapy of <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>nakinra and glucocorticoids. Furthermore, current literature about <jats:styled-content style=\"fixed-case\">ECD<\/jats:styled-content> and treatment options are discussed.<\/jats:p>","DOI":"10.1111\/imj.12329","type":"journal-article","created":{"date-parts":[[2014,1,23]],"date-time":"2014-01-23T08:30:03Z","timestamp":1390465803000},"page":"90-92","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Successful treatment of <scp>E<\/scp>rdheim\u2013<scp>C<\/scp>hester disease with combination of interleukin\u20101\u2010targeting drugs and high\u2010dose glucocorticoids"],"prefix":"10.1111","volume":"44","author":[{"given":"F.","family":"Darstein","sequence":"first","affiliation":[{"name":"I. Medizinische Klinik Universit\u00e4tsmedizin Mainz  Bad Kreuznach Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Kirschey","sequence":"additional","affiliation":[{"name":"III. Medizinische Klinik Universit\u00e4tsmedizin Mainz  Bad Kreuznach Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Heckl","sequence":"additional","affiliation":[{"name":"I. Medizinische Klinik Universit\u00e4tsmedizin Mainz  Bad Kreuznach Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"Rahman","sequence":"additional","affiliation":[{"name":"I. Medizinische Klinik Universit\u00e4tsmedizin Mainz  Bad Kreuznach Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Schwarting","sequence":"additional","affiliation":[{"name":"I. 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