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

NCT01651507 | Completed

• 1 other identifier: HLP
• Study Type: observational
• Target: 49
• Locations: 1 country
• Sites: 1

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.

Conditions

Pulmonary Langerhans Cell Histiocytosis

Keywords

Pulmonary Langerhans cell histiocytosis

Outcome Measures

Primary Outcomes (4)

• 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

• obstructive pattern

  FEV1/FVC ratio \<70%

  5 years

• restrictive pattern

  TLC \<80% of predicted value

  5 years

• 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

Study Arms (1)

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

Eligibility Criteria

• Age: 14 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

* Patients with pulmonary LCH * enrolled in eight teaching hospitals * evaluated between June 1989 and February 2005

You may qualify if:

• pulmonary HLP diagnosed between June 1989 and February 2005

You may not qualify if:

• 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

Sponsors & Collaborators

• Assistance Publique - Hôpitaux de Paris: lead

Study Sites (1)

Saint Louis hospital

Paris, Paris, 75010, France

Location

Related 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 Oct;40(4):905-12. doi: 10.1183/09031936.00210711. Epub 2012 Mar 22.

  PMID: 22441752 RESULT

MeSH Terms

Conditions

Histiocytosis, Langerhans-Cell

Condition Hierarchy (Ancestors)

Lung Diseases, Interstitial; Lung Diseases; Respiratory Tract Diseases; Histiocytosis; Lymphatic Diseases; Hemic and Lymphatic Diseases

Study Design

• Study Type: observational
• Observational Model: CASE ONLY
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: July 25, 2012
• First Posted: July 27, 2012
• Study Start: June 1, 1989
• Primary Completion: February 1, 2005
• Study Completion: September 1, 2010
• Last Updated: April 18, 2016

Record last verified: 2010-09

Locations

FR (1)