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

Pulmonary Langerhans Cell Histiocytosis Clinical Trial

— HLP  

Official title:

Evaluation of Outcome of Adult Pulmonary Langerhans Cell Histiocytosis Based on Lung HRCT and Lung Function. A Multicenter Retrospective Study

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT01651507
• Other study ID #: HLP
• Status: Completed
• Phase: N/A
• First received: July 25, 2012
• Last updated: April 15, 2016
• Start date: June 1989
• Est. completion date: September 2010

Study information

• Verified date: September 2010
• Source: Assistance Publique - Hôpitaux de Paris
• Contact: n/a
• Is FDA regulated: No
• Health authority: France: Ministry of Health
• Study type: Observational

Clinical Trial 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.

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.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 49
• Est. completion date: September 2010
• Est. primary completion date: February 2005
• Accepts healthy volunteers: No
• Gender: Both
• Age group: 14 Years and older

Eligibility

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

Study Design

Observational Model: Case-Only, Time Perspective: Retrospective

Related Conditions & MeSH terms

• Histiocytosis
• Histiocytosis, Langerhans-Cell
• Pulmonary Langerhans Cell Histiocytosis

Locations

Country	Name	City	State
France	Saint Louis hospital	Paris

Sponsors (1)

Lead Sponsor	Collaborator
Assistance Publique - Hôpitaux de Paris

Country where clinical trial is conducted

France, 

References & Publications (1)

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 O — View Citation

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Impairment of lung function	a decrease =10% of FEV1 or FVC, or decrease =15% of DLCO. Conversely, if the FEV1or FVC increased of =10%, or DLCO =15%	5 years	No
Primary	obstructive pattern	FEV1/FVC ratio <70%	5 years	No
Primary	restrictive pattern	TLC <80% of predicted value	5 years	No
Primary	lung HRCT cystic score	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	5 years	No