Successful treatment of Erdheim-Chester disease by interleukin-1 receptor antagonist protein.

Erdheim-Chester disease is a rare non-langerhans systemic histiocytosis of unknown origin, associated with bone diseases and severe visceral complications. Therapies have been disappointing. A recombinant form of interleukin-1 receptor antagonist (anakinra) has been used in a few cases when usual treatment fails. We report a new case of successfully interleukin-1 receptor antagonist treatment in Erdheim-Chester disease.