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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
  Purpose

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.


Condition
Pulmonary Langerhans Cell Histiocytosis

Study Type: Observational
Study Design: 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

Resource links provided by NLM:

U.S. FDA Resources

Further study details as provided by Assistance Publique - Hôpitaux de Paris:

Primary Outcome Measures:
  • 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


Enrollment: 49
Study Start Date: June 1989
Study Completion Date: September 2010
Primary Completion Date: February 2005 (Final data collection date for primary outcome measure)
Groups/Cohorts
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

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.

  Eligibility

Ages Eligible for Study:   14 Years and older
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
Study Population
  • Patients with pulmonary LCH
  • enrolled in eight teaching hospitals
  • evaluated between June 1989 and February 2005
Criteria

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
  Contacts and Locations
Please refer to this study by its ClinicalTrials.gov identifier: NCT01651507

Locations
France
Saint Louis hospital
Paris, France, 75010
Sponsors and Collaborators
Assistance Publique - Hôpitaux de Paris
  More Information

No publications provided

Responsible Party: Assistance Publique - Hôpitaux de Paris
ClinicalTrials.gov Identifier: NCT01651507     History of Changes
Other Study ID Numbers: HLP
Study First Received: July 25, 2012
Last Updated: July 25, 2012
Health Authority: France: Ministry of Health

Keywords provided by Assistance Publique - Hôpitaux de Paris:
Pulmonary Langerhans cell histiocytosis

Additional relevant MeSH terms:
Histiocytosis
Histiocytosis, Langerhans-Cell
Lymphatic Diseases
 Lung Diseases, Interstitial
Lung Diseases
Respiratory Tract Diseases

ClinicalTrials.gov processed this record on May 22, 2013