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

Completed

• Conditions: Pulmonary Langerhans Cell Histiocytosis

• Registration Number: NCT01651507
• Lead Sponsor: Assistance Publique - Hôpitaux de Paris

Brief Summary

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.

Detailed 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.

Study Timeline

• Study Start: 1989-06
• Primary Completion: 2005-02
• Status Verified: 2010-09
• Study Completion: 2010-09
• Study First Submitted: 2012-07-25
• Study First Submitted QC: 2012-07-25
• Study First Posted: 2012-07-27
• Last Update Submitted: 2016-04-15
• Last Update Posted: 2016-04-18

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 49

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

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
lung HRCT cystic score	5 years	The extent of cystic lesions (including thick- and thin- walled cysts) was assessed for each of the 6 defined lung areas on HRCT and classified as follows: 0 (no cyst); 1 (\<25%); 2 (25-49%); 3 (50-75%) and 4 (\>75%) of the lung surface analyzed. For the whole lung, the maximal value for cystic HRCT score was of 24
Impairment of lung function	5 years	a decrease ≥10% of FEV1 or FVC, or decrease ≥15% of DLCO. Conversely, if the FEV1or FVC increased of ≥10%, or DLCO ≥15%
obstructive pattern	5 years	FEV1/FVC ratio \<70%
restrictive pattern	5 years	TLC \<80% of predicted value

Trial Locations

Locations (1)

Saint Louis hospital; 🇫🇷 Paris, France