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

This study has been completed.
Sponsor:
Information provided by (Responsible Party):
Assistance Publique - Hôpitaux de Paris
ClinicalTrials.gov Identifier:
NCT01651507
First received: July 25, 2012
Last updated: NA
Last verified: September 2010
History: No changes posted

July 25, 2012
July 25, 2012
June 1989
February 2005   (final data collection date for primary outcome measure)
  • Impairment of lung function [ Time Frame: 5 years ] [ Designated as safety issue: No ]
    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 ] [ Designated as safety issue: No ]
    FEV1/FVC ratio <70%
  • restrictive pattern [ Time Frame: 5 years ] [ Designated as safety issue: No ]
    TLC <80% of predicted value
  • lung HRCT cystic score [ Time Frame: 5 years ] [ Designated as safety issue: No ]
    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
Same as current
No Changes Posted
Not Provided
Not Provided
Not Provided
Not Provided
 
Evaluation of Outcome of Adult Pulmonary Langerhans Cell Histiocytosis Based on Lung HRCT and Lung Function
Evaluation of Outcome of Adult Pulmonary Langerhans Cell Histiocytosis Based on Lung HRCT and Lung Function. A Multicenter Retrospective Study

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.

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.

Observational
Observational Model: Case-Only
Time Perspective: Retrospective
Not Provided
Not Provided
Non-Probability Sample
  • Patients with pulmonary LCH
  • enrolled in eight teaching hospitals
  • evaluated between June 1989 and February 2005
Pulmonary Langerhans Cell Histiocytosis
Not Provided
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
Not Provided

*   Includes publications given by the data provider as well as publications identified by ClinicalTrials.gov Identifier (NCT Number) in Medline.
 
Completed
49
September 2010
February 2005   (final data collection date for primary outcome measure)

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
Both
14 Years and older
No
Contact information is only displayed when the study is recruiting subjects
France
 
NCT01651507
HLP
No
Assistance Publique - Hôpitaux de Paris
Assistance Publique - Hôpitaux de Paris
Not Provided
Not Provided
Assistance Publique - Hôpitaux de Paris
September 2010

ICMJE     Data element required by the International Committee of Medical Journal Editors and the World Health Organization ICTRP