Abstract

Erdheim-Chester syndrome, a non-Langerhans histiocytosis, is a very rare disease, in the present approximately 500 cases being reported in literature. It is characterized by the histiocytic infiltration of various organs and systems, therefore clinical signs and symptoms are miscellaneous. Despite that, there are a few patognomonic elements that help recognizing the disease: symmetrical involvement in the long bones of the lower limbs, bilateral perirenal infiltration (“hairy kidneys”) and circumferential aortic infiltration (“coated aorta”). However, in order to confirm the diagnosis, biopsy is required, histopathologic examination revealing “foamy” histiocytes. Currently, interferon alpha serves as the first line of treatment, most literature data showing that it might improve survival rate in patients diagnosed with Erdheim-Chester syndrome.

Details

Title
What Lies Behind A Pleurisy?
Author
Anton, Mihaela 1 ; Militaru, Theodora 2 ; Denise-Ani Mardale 2 ; Diaconu, Roxana 2 ; Maximeasa, Camelia 1 ; Vasilescu, Florina 2 ; Beţianu, Cezar 2 ; Ștefănescu, Ioana 2 ; Jurcuţ, Ruxandra 1 ; Jurcuţ, Ciprian 2 

 Cardiology Clinic, “PhD C.C. Iliescu” Emergency Institute for Cardiovascular Diseases, Bucharest, Romania 
 Medical Clinic 2, Central Military Emergency University Hospital “Dr. Carol Davila”, Bucharest, Romania 
Pages
69-74
Publication year
2018
Publication date
2018
Publisher
De Gruyter Poland
ISSN
12205818
Source type
Scholarly Journal
Language of publication
English
ProQuest document ID
3157032677
Copyright
© 2018. This work is published under http://creativecommons.org/licenses/by-nc-nd/3.0 (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.