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


CONCLUSIONS
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.
Arnaud
Laurent
L
Pitié-Salpêtrière Hospital, University Paris 6, Assistance Publique Hôpitaux de Paris, Paris, France.
Pierre
Isabelle
I
Beigelman-Aubry
Catherine
C
Capron
Frédérique
F
Brun
Anne-Laure
AL
Rigolet
Aude
A
Girerd
Xavier
X
Weber
Nina
N
Piette
Jean-Charles
JC
Grenier
Philippe A
PA
Amoura
Zahir
Z
Haroche
Julien
J
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D031249:Erdheim-Chester Disease / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D005260:Female; D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011859:Radiography; D012189:Retrospective Studies; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D016898:Interferon-alpha