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
• Sponsor: Assistance Publique - Hôpitaux de Paris
• Information provided by (Responsible Party): Assistance Publique - Hôpitaux de Paris

Study Description

Brief Summary:

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 or disease
Pulmonary Langerhans Cell Histiocytosis

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.

Study Design

• 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

Groups and Cohorts

Group/Cohort
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

Outcome Measures

Primary Outcome Measures :

1. 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%
2. obstructive pattern [ Time Frame: 5 years ]
   FEV1/FVC ratio <70%
3. restrictive pattern [ Time Frame: 5 years ]
   TLC <80% of predicted value
4. 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

Eligibility Criteria

• 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

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

Locations

France

Saint Louis hospital

Paris, France, 75010

Sponsors and Collaborators

Assistance Publique - Hôpitaux de Paris

More Information

Publications of Results:

Tazi A, Marc K, Dominique S, de Bazelaire C, Crestani B, Chinet T, Israel-Biet D, Cadranel J, Frija J, Lorillon G, Valeyre D, Chevret S. Serial computed tomography and lung function testing in pulmonary Langerhans' cell histiocytosis. Eur Respir J. 2012 Oct;40(4):905-12. Epub 2012 Mar 22.

• Responsible Party: Assistance Publique - Hôpitaux de Paris
• ClinicalTrials.gov Identifier: NCT01651507
• Other Study ID Numbers: HLP
• First Posted: July 27, 2012
• Last Update Posted: April 18, 2016
• Last Verified: September 2010

Keywords provided by Assistance Publique - Hôpitaux de Paris:

Pulmonary Langerhans cell histiocytosis

Additional relevant MeSH terms:

Histiocytosis, Langerhans-Cell; Histiocytosis; Lymphatic Diseases; Lung Diseases, Interstitial; Lung Diseases; Respiratory Tract Diseases