Erdheim-Chester disease

PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Treatment of skeletal Erdheim-Chester disease with zoledronic acid: case report and proposed mechanisms of action” (Rheumatol Int. 2006 Aug 25). Authors are Srikulmontree T, Massey HD and Roberts WN, from the Rheumatology Section, Hunter Holmes McGuire Medical Center, Richmond, VA, USA. Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized by tissue infiltration of lipid-laden macrophages, multinucleated giant cells, and inflammatory infiltrate of lymphocytes and histiocytes. The disease typically involves long bone, but may also affect the central nervous system, the orbit, retroperitoneal organs, and the lungs. There is no proven effective treatment for ECD to date. However, recent data suggested the potential use of bisphosphonates for the treatment of this rare disease. The authors report a case of biopsy-proven skeletal ECD, who received treatment with zoledronic acid, an aminobisphosphonate, with remarkable clinical improvement. To access the full abstract of the article, click here.