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

Overview

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.

Full Title of Study: “Evaluation of Outcome of Adult Pulmonary Langerhans Cell Histiocytosis Based on Lung HRCT and Lung Function. A Multicenter Retrospective Study”

Study Type

  • Study Type: Observational
  • Study Design
    • Time Perspective: Retrospective
  • Study Primary Completion Date: February 2005

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.

Arms, Groups and 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

Clinical Trial Outcome Measures

Primary Measures

  • 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

Participating in This Clinical Trial

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

Gender Eligibility: All

Minimum Age: 14 Years

Maximum Age: N/A

Are Healthy Volunteers Accepted: No

Investigator Details

  • Lead Sponsor
    • Assistance Publique – Hôpitaux de Paris
  • Provider of Information About this Clinical Study
    • Sponsor

Citations Reporting on 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. doi: 10.1183/09031936.00210711. Epub 2012 Mar 22.

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