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:


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
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

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
Lung Diseases
Lung Diseases, Interstitial
Lymphatic Diseases
Respiratory Tract Diseases

ClinicalTrials.gov processed this record on October 23, 2014