Adult Pulmonary Langerhans Cell Histiocytosis
Conditions
Brief summary
Study objectives: * To estimate the incidence of pulmonary deterioration in adult pulmonary Langerhans cell histiocytosis * To assess the impact of tobacco discontinuation * Study Design Multicentric prospective cohort study * Main endpoint: Pulmonary deterioration * Sample size : 40 patients
Detailed description
Study objectives To estimate the incidence of pulmonary deterioration in adult pulmonary Langerhans cell histiocytosis To assess the impact of tobacco discontinuation Study Design Multicentric prospective cohort study Main endpoint: Pulmonary deterioration Sample size : 40 patients
Interventions
BEHAVIORALTabacco discontinuation
Tabacco discontinuation
Sponsors
Assistance Publique - Hôpitaux de Paris
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Pulmonary Langerhans cell histiocytosis diagnosed within the past 48 months
Exclusion criteria
* Age \< 18 * Deep pulmonary dysfunction (CPT\< 60%, FEV1\< 30%, DLCO\< 30%, partial pressure of oxygen in arterial blood (PaO2)\< 60mmHg) * No informed consent or consent withdrawal
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Pulmonary deterioration | 2 years | decrease in Forced expiratory volume in 1 second (FEV1), vital capacity (VC), or Diffusing capacity of the lung for carbon monoxide (DLCO) of at least 15% as compared to baseline values and/or progression of clinical symptoms (general symptoms) and/or occurrence of pneumothorax |
Secondary
| Measure | Time frame |
|---|---|
| Evolution of pulmonary volumes (FEV1) | 6 months |
| Evolution of pulmonary lesions in High Resolution Computed Tomography (HRCT) | 6 months |
Countries
France
Outcome results
None listed