Pulmonary Langerhans Cell Histiocytosis
Conditions
Keywords
Pulmonary Langerhans cell histiocytosis
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.
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.
Interventions
None listed
Sponsors
Assistance Publique - Hôpitaux de Paris
Study design
Observational model
CASE_ONLY
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
14 Years to No maximum
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Impairment of lung function | 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 | 5 years | FEV1/FVC ratio \<70% |
| restrictive pattern | 5 years | TLC \<80% of predicted value |
| lung HRCT cystic score | 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 |
Countries
France
Outcome results
None listed