The natural history of adult pulmonary Langerhans cell histiocytosis: a prospective multicentre study.

ABSTRACT:

Background

The natural history of pulmonary Langerhans cell histiocytosis (PLCH) has been unclear due to the absence of prospective studies. The rate of patients who experience an early progression of their disease is unknown. Additionally, conflicting effects of smoking cessation on the outcome of PLCH have been reported.

Methods

In this prospective, multicentre study, 58 consecutive patients with newly diagnosed PLCH were comprehensively evaluated over a two-year period. Our objectives were to estimate the incidence of early progression of the disease and to evaluate the impact of smoking status on lung function outcomes. Lung function deterioration was defined as a decrease of at least 15% in FEV1 and/or FVC and/or DLCO, compared with baseline values. At each visit, smoki

SUBMITTER: Tazi A 

PROVIDER: S-EPMC4438520 | biostudies-literature | 2015 Mar

REPOSITORIES: biostudies-literature