Short communication, Note
Erdheim-Chester Disease and Concomitant Tuberculosis Successfully Treated with Chemotherapy and Long-Term Steroids
Saša Badžek
Ana Mišir-Krpan
Zdenko Krajina
Ivo Radman
Ranka Štern-Padovan
Snježana Dotlić
Abstract
Erdheim-Chester disease (ECD) is a rare histiocytosis usually affecting the skeletal system, but visceral organs and
central nervous system involvement are common as well. Probability exists that immunomodulatory therapies and disorders
can play a role in clinical course of the disease. Because of rarity of the disorder, it is hard to classify it and standardize
the treatment options, but, according to published material and our experience, cytotoxic chemotherapy and
long-term steroids have therapeutic benefit. Although this approach can probably be accepted as standard of care management,
novel therapeutic modalities should be explored, and pathogenesis and disorder classification should be cleared
out as well. The case of ECD affecting skeletal system and lungs and concomitant laryngeal tuberculosis successfully
treated with chemotherapy and long-term steroid therapy is presented.
Keywords
Erdheim-Chester disease; steroids; therapy; tuberculosis
Hrčak ID:
27371
URI
Publication date:
10.4.2007.
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