Document Detail

Pulmonary involvement in Erdheim-Chester disease: a single-center study of thirty-four patients and a review of the literature.
MedLine Citation:
PMID:  20662053     Owner:  NLM     Status:  MEDLINE    
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 Pitié-Salpêtrière 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-α (IFNα) 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 IFNα. The overall prognosis of the disease is poor, but pulmonary involvement does not appear to be a major prognostic factor in ECD.
Laurent Arnaud; Isabelle Pierre; Catherine Beigelman-Aubry; Frédérique Capron; Anne-Laure Brun; Aude Rigolet; Xavier Girerd; Nina Weber; Jean-Charles Piette; Philippe A Grenier; Zahir Amoura; Julien Haroche
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Arthritis and rheumatism     Volume:  62     ISSN:  1529-0131     ISO Abbreviation:  Arthritis Rheum.     Publication Date:  2010 Nov 
Date Detail:
Created Date:  2010-11-02     Completed Date:  2010-11-30     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0370605     Medline TA:  Arthritis Rheum     Country:  United States    
Other Details:
Languages:  eng     Pagination:  3504-12     Citation Subset:  AIM; IM    
Copyright Information:
Copyright © 2010 by the American College of Rheumatology.
Pitié-Salpêtrière Hospital, University Paris 6, Assistance Publique Hôpitaux de Paris, Paris, France.
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MeSH Terms
Adrenal Cortex Hormones / therapeutic use
Erdheim-Chester Disease / pathology*,  radiography,  therapy
Interferon-alpha / therapeutic use
Lung / pathology*,  radiography
Middle Aged
Retrospective Studies
Treatment Outcome
Reg. No./Substance:
0/Adrenal Cortex Hormones; 0/Interferon-alpha

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