Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.

Efficacy of High Doses of Interferon-a-2a (IFNa) in Erdheim-Chester Disease.

Hervier,  Baptiste, Arnaud,  Laurent, Wechsler,  Bertrand, Amoura,  Zahir, Haroche,  Julien


Erdheim-Chester disease (ECD) is a rare non-langerhans form of histiocytosis, characterized by a foamy histiocyte tissue infiltration. Treatments are not standardized. Efficacy of low doses of Interferon-a-2a (IFNa) have been suggested in small series but with variation depending of the organs involved. Indeed, central nervous system (CNS) and perivascular infiltration were reported to be resistant to low doses of IFNa.

Our aim is to report our single-center experience about the use of high doses of IFNa in ECD.


Twenty patients with biopsy-proven ECD have received high doses of IFNa (IFNa>= 18 MUI/week or PEG-IFNa>= 180 mg/week).

IFNa efficiency was systematically evaluated both clinically and morphologically (CT-scan or MRI for vessels & CNS, cardiac MRI, bone scintigraphy or MRI. 18FFDG-PET)


Sixteen men & four women were included (median age at diagnosis: 56 years (29–77). The median follow-up was 18 months (6–60). Treatments were IFNa 18 millions MUI/week (n=4), IFNa 27 MUI/week (n=8), PEG-IFNa 180 mg/week (n=8).

The treatment was started because of: CNS involvement (n=5), heart involvement (n=4), association of CNS & heart involvement (n=5), severe exophtalmos (n=1) and other (n=5). High doses of IFNa were started as first line treatment (n=12) or after a low dose course of IFNa (n=8), which was insufficient.

High doses of IFNa were efficient in 17 cases: incomplete remission (n=14, 70%) & stabilization (n=3, 15%). However, two patients did not respond to this treatment (10%) and another patient could not be evaluated (early treatment interruption). For these three patients the following treatments were inefficient (deaths, n=3).

Improvement concerned CNS (60%), lung (67%), bone (64%) and heart (56%). Median time before clinical improvement was 6 months (6–24). Nevertheless, improvement of sinusal involvement (33%), vascular involvement (20%), exophthalmos (33%) and retro-peritoneal fibrosis (20%) were rare.

High doses of IFNa were well-tolerated (n=14, 70%). However, six patients displayed side effects: severe asthenia (n=3), depression (n=2), eczema (n=1) requiring an interrupted treatment course (n=2) and an early definitive interruption (n=1). One responder patient died under IFNa after 24 months of follow-up.


High doses of IFNa may control severe ECD (85% of the cases) and are well tolerated (70%).

To cite this abstract, please use the following information:
Hervier, Baptiste, Arnaud, Laurent, Wechsler, Bertrand, Amoura, Zahir, Haroche, Julien; Efficacy of High Doses of Interferon-a-2a (IFNa) in Erdheim-Chester Disease. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1658
DOI: 10.1002/art.29423

Abstract Supplement

Meeting Menu