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Erdheim–Chester disease

Erdheim–Chester disease REVIEW Erdheim–Chester disease a,b a,c a,b,c Julien Haroche , Laurent Arnaud , and Zahir Amoura Purpose of review Erdheim–Chester disease (ECD) is a rare, non-Langerhans form of histiocytosis first described in 1930 with a wide range of manifestations. The number of new cases has dramatically increased over the past 10 years because of the better recognition of this condition. The natural evolution is variable, but the spontaneous prognosis is severe. In this review, we describe the relevant clinical, radiological, prognostic, and therapeutic features of this orphan disease. Recent findings Compelling evidence demonstrates the efficacy of treatment by interferon alpha (IFNa) which has been reported to be a major independent predictor of survival among ECD patients. Alternative treatments remain to be defined. Recent studies have highlighted the central nervous system involvement as an independent predictor of death. Pathophysiology is better understood with a complex network of cytokines and chemokines and a systemic immune Th-1-oriented perturbation. Summary ECD, although a rare and orphan disease, has been overlooked and numerous new cases are currently diagnosed because of general better knowledge of this histiocytosis. First-line treatment is IFNa. We have recently described a unique cytokine signature that may provide further clues to understand http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Current Opinion in Rheumatology Wolters Kluwer Health

Erdheim–Chester disease

Amoura, Zahir
Current Opinion in Rheumatology , Volume 24 (1) – Jan 1, 2012
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Copyright
Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
ISSN
1040-8711
eISSN
1531-6963
DOI
10.1097/BOR.0b013e32834d861d
pmid
22089098
Publisher site
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Abstract

REVIEW Erdheim–Chester disease a,b a,c a,b,c Julien Haroche , Laurent Arnaud , and Zahir Amoura Purpose of review Erdheim–Chester disease (ECD) is a rare, non-Langerhans form of histiocytosis first described in 1930 with a wide range of manifestations. The number of new cases has dramatically increased over the past 10 years because of the better recognition of this condition. The natural evolution is variable, but the spontaneous prognosis is severe. In this review, we describe the relevant clinical, radiological, prognostic, and therapeutic features of this orphan disease. Recent findings Compelling evidence demonstrates the efficacy of treatment by interferon alpha (IFNa) which has been reported to be a major independent predictor of survival among ECD patients. Alternative treatments remain to be defined. Recent studies have highlighted the central nervous system involvement as an independent predictor of death. Pathophysiology is better understood with a complex network of cytokines and chemokines and a systemic immune Th-1-oriented perturbation. Summary ECD, although a rare and orphan disease, has been overlooked and numerous new cases are currently diagnosed because of general better knowledge of this histiocytosis. First-line treatment is IFNa. We have recently described a unique cytokine signature that may provide further clues to understand

Journal

Current Opinion in Rheumatology – Wolters Kluwer Health

Published: Jan 1, 2012

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