Pulmonary involvement in Erdheim-Chester disease: a single-center study of thirty-four patients and a review of the literature.

Details

Serval ID
serval:BIB_864A1401475B
Type
Article: article from journal or magazin.
Collection
Publications
Title
Pulmonary involvement in Erdheim-Chester disease: a single-center study of thirty-four patients and a review of the literature.
Journal
Arthritis and Rheumatism
Author(s)
Arnaud L., Pierre I., Beigelman-Aubry C., Capron F., Brun A.L., Rigolet A., Girerd X., Weber N., Piette J.C., Grenier P.A., Amoura Z., Haroche J.
ISSN
1529-0131 (Electronic)
ISSN-L
0004-3591
Publication state
Published
Issued date
2010
Volume
62
Number
11
Pages
3504-3512
Language
english
Abstract
OBJECTIVE: Erdheim-Chester disease (ECD) is a rare form of non-Langerhans' cell histiocytosis that may present with pulmonary involvement. We undertook the current study to evaluate the characteristic features of pulmonary involvement in ECD, in the largest single-center series of patients reported to date. METHODS: We performed a retrospective study of the characteristics of 34 consecutive patients with biopsy-proven ECD who were referred to the internal medicine department of Pitie-Salpetriere Hospital between 1981 and November 2008. RESULTS: Data were obtained from 23 men and 11 women. The median age at the time of diagnosis was 53.7 years (range 16-73 years), and the median followup was 3.5 years (1.4-5.3 years). Eight patients (24%) had pulmonary symptoms. High-resolution computed tomography (HRCT) scans of the chest revealed involvement of lung parenchyma in 18 patients (53%) and of the pleura in 14 patients (41%). Bronchoalveolar lavage fluid analysis revealed the presence of an opalescent aspirate in all the patients studied. Treatment with corticosteroids and/or interferon-alpha (IFNalpha) resulted in a marked improvement of the pulmonary lesions in only a single patient. Comparison of the survival between patients with and those without pulmonary involvement yielded no significant difference between the groups (P = 0.82). CONCLUSION: Pulmonary involvement in ECD has been overlooked in previous studies. HRCT reveals typical lesions in most patients. There is no clear response of these lesions to corticosteroids and IFNalpha. The overall prognosis of the disease is poor, but pulmonary involvement does not appear to be a major prognostic factor in ECD.
Keywords
Adolescent, Adrenal Cortex Hormones/therapeutic use, Adult, Aged, Erdheim-Chester Disease/pathology, Erdheim-Chester Disease/radiography, Female, Humans, Interferon-alpha/therapeutic use, Lung/pathology, Lung/radiography, Male, Middle Aged, Retrospective Studies, Treatment Outcome
Pubmed
Web of science
Open Access
Yes
Create date
31/08/2011 12:46
Last modification date
20/08/2019 14:45
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