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Erdheim-Chester Disease:A Rare Multisystem Histiocytic Disorder Associated with Interstitial Lung Disease

 

作者: Robert D. Shamburek,   H. Bryan Brewer,   Bernadette Gochuico,  

 

期刊: The American Journal of the Medical Sciences  (OVID Available online 2001)
卷期: Volume 321, issue 1  

页码: 66-75

 

ISSN:0002-9629

 

年代: 2001

 

出版商: OVID

 

关键词: Histiocytosis;Cholesterol;Erdheim-Chester;Interstitial lung disease;Non-Langerhans cells.

 

数据来源: OVID

 

摘要:

Erdheim-Chester disease (ECD) is a rare multisystem histiocytosis syndrome of unknown cause that usually affects adults. Histiocytic infiltration of multiple end organs produces bone pain, xanthelasma and xanthoma, exophthalmos, diabetes insipidus, and interstitial lung disease. Differential diagnosis includes Langerhans cell histiocytosis, metabolic disorders, malignancy, and sarcoidosis. ECD can be diagnosed using a combination of clinical and histopathologic findings. Sites of involvement include lung, bone, skin, retro-orbital tissue, central nervous system, pituitary gland, retroperitoneum, and pericardium. Symmetrical long bone pain with associated osteosclerotic lesions, xanthomas around the eyelids, exophthalmos, and/or diabetes insipidus suggest ECD. Approximately 35% of patients have associated lung involvement, characterized by interstitial accumulations of histiocytic cells and fibrosis in a predominantly perilymphangitic and subpleural pattern. This pattern distinguishes ECD from other histiocytic disorders involving the lung. The diagnosis is confirmed by tissue biopsies that contain histiocytes with non-Langerhans cell features. In general, the clinical course of patients with this disease varies, and the prognosis can be poor despite treatment. Clinical trials for treatment of ECD have not been conducted and treatment is based on anecdotal experience.

 

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