Evaluation of Outcome of Adult Pulmonary Langerhans Cell Histiocytosis Based on Lung HRCT and Lung Function (HLP)
|ClinicalTrials.gov Identifier: NCT01651507|
Recruitment Status : Completed
First Posted : July 27, 2012
Last Update Posted : April 18, 2016
|Condition or disease|
|Pulmonary Langerhans Cell Histiocytosis|
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 Type :||Observational|
|Actual Enrollment :||49 participants|
|Official Title:||Evaluation of Outcome of Adult Pulmonary Langerhans Cell Histiocytosis Based on Lung HRCT and Lung Function. A Multicenter Retrospective Study|
|Study Start Date :||June 1989|
|Primary Completion Date :||February 2005|
|Study Completion Date :||September 2010|
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
- Impairment of lung function [ Time Frame: 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 [ Time Frame: 5 years ]FEV1/FVC ratio <70%
- restrictive pattern [ Time Frame: 5 years ]TLC <80% of predicted value
- lung HRCT cystic score [ Time Frame: 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
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Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT01651507
|Saint Louis hospital|
|Paris, France, 75010|