- 作者：Baptiste Hervier ; MD鈦?/sup> ; &dagger ; ; Laurent Arnaud鈦?/sup> ; &dagger ; ; &Dagger ; ; Frederic Charlotte ; MD&dagger ; ; § ; ; Bertrand Wechsler ; MD鈦?/sup> ; Jean Charles Piette鈦?/sup> ; Zahir Amoura鈦?/sup> ; &dagger ; ; &Dagger ; ; Julien Haroche ; MD鈦?/sup> ; &dagger ; ; julien.haroche@psl.aphp.fr
- 关键词：histiocytosis ; Erdheim-Chester disease ; interferon-伪
- 刊名：Seminars in Arthritis and Rheumatism
- 出版年：2012
- 期刊代码：283_00490172
- 类别：med
- 出版时间：June, 2012
- 卷：41
- 期：6
- 页码：907-913
- 文件大小：1473 K
Objectives
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis, characterized by a foamy CD68+, CD1a鈭?histiocyte tissue infiltration. Efficacy of standard doses of interferon-伪-2a (IFN伪) has been suggested in a small series but with variation, depending on the organs involved. Our aim was to report our single-center experience about the use of high-dose IFN伪 in ECD.Methods
Twenty-four ECD patients have received high-dose IFN伪 (IFN伪 鈮?8 mIU/wk or pegylated-IFN伪 鈮?80 渭g/wk). IFN伪 efficacy was evaluated clinically and morphologically using a standardized protocol (median follow-up 19 months).
Results
Indication for treatment was central nervous system and/or heart involvement (n = 20), exophthalmos (n = 1), and standard-dose IFN伪 inefficacy (n = 3). High-dose IFN伪 was effective in 16 patients (67%) with improvement (n = 11, 46%) and stabilization (n = 5, 21%). Late and gradual improvement was observed during prolonged follow-up in most patients. The efficacy of high-dose IFN伪 was dependent on the organs involved: central nervous system and heart improvement or stabilization occurred in 7/11 (64%) and 11/14 (79%) patients, respectively. Six patients (25%) worsened. High doses of IFN伪 were well-tolerated: 13 (54.2%) patients had side effects but treatment interruption was infrequent (n = 3, 12.5%).
Conclusions
High-dose IFN伪 may be effective in severe ECD. Improvement may be slow, and high-dose IFN伪 treatment should be prolonged.