<abstract xmlns="http://www.w3.org/1999/xhtml"><p>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.</p></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.

What Lies Behind A Pleurisy?

Cardiology Clinic, “PhD C.C. Iliescu” Emergency Institute for Cardiovascular Diseases

Medical Clinic 2, Central Military Emergency University Hospital “Dr. Carol Davila”

Internal Medicine

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

{"article-title":"What Lies Behind A Pleurisy?"}

Erdheim-Chester syndrome, a non-Langerhans histiocytosis, is a very rare disease, in the present approximately 500 cases being reported in literature. It is...

What Lies Behind A Pleurisy?

Published Online: May 14, 2018

Page range: 69 - 74

DOI: https://doi.org/10.2478/inmed-2018-0016

Keywords
Erdheim-Chester syndrome, pleurisy, dyspnea

© 2018 Mihaela Anton et al., published by De Gruyter Open

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

What Lies Behind A Pleurisy?

Published Online: May 14, 2018

Page range: 69 - 74

DOI: https://doi.org/10.2478/inmed-2018-0016

KeywordsErdheim-Chester syndrome, pleurisy, dyspnea

© 2018 Mihaela Anton et al., published by De Gruyter Open

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Keywords
Erdheim-Chester syndrome, pleurisy, dyspnea