Evaluation of Outcome of Adult Pulmonary Langerhans Cell Histiocytosis Based on Lung HRCT and Lung Function (HLP)

Assistance Publique - Hôpitaux de Paris

Status: Completed

Conditions: Pulmonary Langerhans Cell Histiocytosis

Study type: Observational

Funder types: Other

Identifiers

NCT01651507

HLP

Details and patient eligibility

About

this is a multicenter retrospective study on patients with pulmonary LCH who were sequentially evaluated by concomitant lung HRCT and lung function testing. The objectives of this study were: 1) to determine the changes over time of lung function parameters in patients with pulmonary LCH; 2) to compare the variations of HRCT and lung function results during follow-up; 3) to address the respective interest of these investigations for identifying the patients who eventually will experience a progression of their disease.

Full description

Pulmonary Langerhans cell histiocytosis (pulmonary LCH) is an uncommon disorder, characterized by the accumulation of CD1a+ Langerhans cells (LCs) organized in granulomas that develop in, and destroy the wall of distal bronchioles. In adults the disease occurs predominantly in young smokers from both genders, with a peak incidence at 20-40 yrs of age, although female may be slightly older. High resolution computed tomography (HRCT) of the lung has provided a considerable input for the diagnosis of pulmonary LCH.

We conducted a multicenter retrospective study on patients with pulmonary LCH who were sequentially evaluated by concomitant lung HRCT and lung function testing. The objectives of this study were: 1) to determine the changes over time of lung function parameters in patients with pulmonary LCH; 2) to compare the variations of HRCT and lung function results during follow-up; 3) to address the respective interest of these investigations for identifying the patients who eventually will experience a progression of their disease. Results of this study may help to improve the management of patients with adult pulmonary LCH.

Enrollment: 49 patients

Sex: All

Ages: 14+ years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• pulmonary HLP diagnosed between June 1989 and February 2005

Exclusion criteria

• followed up for less than 6 months
• with less than 2 lung HRCT and lung function tests at the same time or within a 2 month period

Trial design

49 participants in 1 patient group

HLP

Description:

Patients with pulmonary LCH from eight teaching hospitals evaluated between June 1989 and February 2005 were considered for this study, if they were followed for at least 6 months and evaluated by ≥ 2 lung HRCT and lung function tests at the same time or within a 2 months period