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.


Erdheim-Chester Disease: Prospective Follow-Up of a Single-Center Cohort of 48 Patients Reveals Improved Survival in Patients Treated with Interferon-alpha.

Arnaud,  Laurent, Haroche,  Julien, Hervier,  Baptiste, Wechsler,  Bertrand, Costedoat-Chalumeau,  Nathalie, Cacoub,  Patrice, Saadoun,  David

Background:

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis, with poor prognosis and non-codified therapeutic management. This study was undertaken to evaluate the impact of treatment with interferon alpha on survival in ECD patients.

Methods:

Forty-eight patients (36 men and 12 women; median age: 59 years; median follow-up duration: 40.8 months) with biopsy-proven ECD were included in a single-center prospective cohort study, between Nov. 1981 and Jan. 2010. Five patients were excluded because of an overlap with another form of histiocytosis. Independent predictors of survival were identified using Cox proportional hazard model.

Results:

Nine patients (18.8%) died after a median follow-up of 28.1 months (13.0–155.7 months). Univariate analysis revealed no difference in survival between gender (p=0.78), patients with and without biological inflammation (p=0.64), and treatment with or without corticosteroids (p=0.54). Comparison of patients treated with (n=40) and without (n=3) interferon-alpha and/or PEGylated interferon-alpha for at least 3 months (median duration of treatment: 23.4 months [3.2–148.2 months]) revealed an almost significant improvement (p=0.059) in patients treated with interferon (5 patients were excluded from this analysis because they received interferon for <= 3 months). Multivariate survival analysis using Cox proportional hazard model revealed that treatment with interferon alpha and/or PEGylated interferon-alpha for at least 3 months was the sole independent predictor of survival in our cohort (Hazard Ratio, HR: 0.10, IC95%: 0.01–0.82; p=0.03).

Conclusions:

Although definitive confirmation would require a randomized controlled trial, our result strongly suggests that interferon-alpha improves survival in patients with ECD.

To cite this abstract, please use the following information:
Arnaud, Laurent, Haroche, Julien, Hervier, Baptiste, Wechsler, Bertrand, Costedoat-Chalumeau, Nathalie, Cacoub, Patrice, et al; Erdheim-Chester Disease: Prospective Follow-Up of a Single-Center Cohort of 48 Patients Reveals Improved Survival in Patients Treated with Interferon-alpha. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1660
DOI: 10.1002/art.29425

Abstract Supplement

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