Document Detail


Successful treatment of Erdheim-Chester disease with combination of interleukin-1-targeting drugs and high-dose glucocorticoids.
MedLine Citation:
PMID:  24450524     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Erdheim-Chester disease (ECD) is a rare histocytic disorder. We report a case of a 45-year-old male ECD 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-1A receptor antagonist (Anakinra, 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 Anakinra and glucocorticoids. Furthermore, current literature about ECD and treatment options are discussed.
Authors:
F Darstein; S Kirschey; S Heckl; F Rahman; A Schwarting; M Schuchmann; P R Galle; T Zimmermann
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Internal medicine journal     Volume:  44     ISSN:  1445-5994     ISO Abbreviation:  Intern Med J     Publication Date:  2014 Jan 
Date Detail:
Created Date:  2014-01-23     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101092952     Medline TA:  Intern Med J     Country:  Australia    
Other Details:
Languages:  eng     Pagination:  90-2     Citation Subset:  IM    
Copyright Information:
© 2014 The Authors; Internal Medicine Journal © 2014 Royal Australasian College of Physicians.
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