RT Journal Article
SR Electronic
T1 Serial CT and lung function testing in pulmonary Langerhans cell histiocytosis
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP erj02107-2011
DO 10.1183/09031936.00210711
A1 Abdellatif Tazi
A1 Karima Marc
A1 Stéphane Dominique
A1 Cédric de Bazelaire
A1 Bruno Crestani
A1 Thierry Chinet
A1 Dominique Israel-Biet
A1 Jacques Cadranel
A1 Jacques Frija
A1 Gwenael Lorillon
A1 Dominique Valeyre
A1 Sylvie Chevret
YR 2012
UL http://erj.ersjournals.com/content/early/2012/03/22/09031936.00210711.abstract
AB Little is known about longitudinal lung function variation in patients with pulmonary Langerhans cell histiocytosis (LCH). The contribution of serial lung computed tomography (CT) to managing these patients has not been evaluated.This long-term retrospective study included 49 patients who were serially evaluated by lung CT and pulmonary function tests. The lung function variation was categorised as improvement or deterioration. The extent of the CT lesions was correlated with lung function.Lung function deteriorated in approximately 60% of the patients. FEV1 and DL,CO were the parameters that most frequently deteriorated. A subgroup of the patients experienced a dramatic decline in FEV1 within two years after diagnosis. Airway obstruction was the major functional pattern observed. In a multivariate analysis, FEV1% of predicted at diagnosis was the only factor associated with the incidence of airway obstruction. The increase in cystic lesions on the lung CTs was associated with impaired lung function but did not anticipate the FEV1 or the DL,CO decline.Serial lung function tests are essential for following patients with pulmonary LCH, who frequently develop airway obstruction. A lung CT at diagnosis is informative, but routine sequential CTs seem less useful. A prospective study is needed to characterise those patients with early progressive disease.