Erdheim-Chester disease is a rare non-Langerhans histiocytosis, with particular tropism for connective and adipose tissues. The infiltrative process preferentially involves the axial skeleton, pituitary, orbit, retroperitoneum, lung, mediastinum and central nervous system. To date, there is no standard treatment for this rare disorder and about two-thirds of the patients succumb to their disease within three years. IFN might be a valuable first-line therapy for prolonged treatment of ECD. However, the efficacy of IFN varies among patients and according to the sites of disease involvement, and symptoms may fail to respond to treatment, especially in patients with severe multisystemic forms of ECD with central nervous system and cardiovascular involvement. There are only case reports and series to support this treatment modality.
Braiteh F, Boxrud C, Esmaeli B, Kurzrock R.Successful treatment of Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with interferon-alpha.Blood. 2005 Nov 1;106(9):2992-4. Epub 2005 Jul 14.
Julien Haroche et al, Variability in the efficacy of interferon- in Erdheim-Chester disease by patient and site of involvement: Results in eight patients Arthritis Rheum Volume 54, Issue 10 , Pages 3330 – 3336