Evaluation of Outcome of Adult Pulmonary Langerhans Cell Histiocytosis Based on Lung HRCT and Lung Function (HLP)
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ClinicalTrials.gov Identifier: NCT01651507 |
Recruitment Status :
Completed
First Posted : July 27, 2012
Last Update Posted : April 18, 2016
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Condition or disease |
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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 |
Observational Model: | Case-Only |
Time Perspective: | Retrospective |
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 |
Actual Primary Completion Date : | February 2005 |
Actual Study Completion Date : | September 2010 |

Group/Cohort |
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HLP
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
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- 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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Ages Eligible for Study: | 14 Years and older (Child, Adult, Older Adult) |
Sexes Eligible for Study: | All |
Accepts Healthy Volunteers: | No |
Sampling Method: | Non-Probability Sample |
- Patients with pulmonary LCH
- enrolled in eight teaching hospitals
- evaluated between June 1989 and February 2005
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

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.
Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT01651507
France | |
Saint Louis hospital | |
Paris, France, 75010 |
Responsible Party: | Assistance Publique - Hôpitaux de Paris |
ClinicalTrials.gov Identifier: | NCT01651507 |
Other Study ID Numbers: |
HLP |
First Posted: | July 27, 2012 Key Record Dates |
Last Update Posted: | April 18, 2016 |
Last Verified: | September 2010 |
Pulmonary Langerhans cell histiocytosis |
Histiocytosis, Langerhans-Cell Histiocytosis Lymphatic Diseases |
Lung Diseases, Interstitial Lung Diseases Respiratory Tract Diseases |