Biochemical markers of bone turnover, serum levels of interleukin-6/interleukin-6 soluble receptor and bisphosphonate treatment in Erdheim-Chester disease.
Erdheim-Chester disease (ECD) is a rare non-Langherans form of histiocytosis characterized radiologically by symmetrical sclerosis of the metaphysis and the diaphysis of long tubular bones. Macrophages are potent interleukin-6 (IL-6) producers and elevated IL-6 serum levels have been described in pathological conditions characterized by increased bone resorption. In a patient with ECD, during the acute phase of the disease we found high serum levels of IL-6 and IL-6 soluble receptor (sIL-6R) and high levels of bone turnover markers. After 5 years of combination therapy with oral prednisone and intravenous clodronate a significant reduction in the above mentioned biological parameters was seen. We suggest that the systemic disorders present in ECD could be related to the high serum levels of IL-6 and sIL-6R. We also propose the use of bisphosphonates in the clinical management of ECD.
Mossetti
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Department of Clinical and Experimental Medicine, Federico II University Medical School, Naples, Italy. nunziata@unina.it
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D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000963:Antimetabolites / Q000627:therapeutic use*; D015415:Biomarkers / Q000097:blood; D016723:Bone Remodeling / Q000276:immunology*; D004002:Clodronic Acid / Q000627:therapeutic use*; D004164:Diphosphonates / Q000627:therapeutic use*; D031249:Erdheim-Chester Disease / Q000097:blood / Q000188:drug therapy* / Q000276:immunology*; D006801:Humans; D015850:Interleukin-6 / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D019947:Receptors, Interleukin-6 / Q000276:immunology*; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D000963:Antimetabolites; D015415:Biomarkers; D004164:Diphosphonates; D015850:Interleukin-6; D019947:Receptors, Interleukin-6; D004002:Clodronic Acid; D011241:Prednisone