Procedures that each subject will undergo include:
Chest CT scan to determine extent of disease.
Pulmonary function testing to determine severity of disease.
Bronchoscopy with lavage.
Venipuncture.

Hypersensitivity Pneumonitis

Bronchoscopy will be performed according to standard clinical procedures. Bronchoscopy will be performed under conscious sedation with continuous cardiopulmonary monitoring and the subject's upper airway will be anesthetized with topical lidocaine. The bronchoscope will be passed through the naris or mouth to the upper airway and then into the lower airway. 360cc of sterile saline will be instilled into 2 sub-segments by instilling and aspirating 3 aliquots of 60cc sequentially into each sub-segment.
Venipuncture to obtain peripheral blood mononuclear cells (PBMCs) for the proposed assays.
Cells collected will be analyzed for various T-cell subsets and gene expression to help determine the type of immune response.

Bronchoscopy with lavage

University of Iowa

Immune Response in Hypersensitivity Pneumonitis

CD4 T-cell Immunephenotype in Hypersensitivity Pneumonitis

T-cell subtypes will be determined by flow cytometry.

Nabeel Hamzeh

Associate Professor of Medicine

National Jewish Health

Patients with diagnosis of LCH based on histopathological or clinical and radiological findings.

Current or ex smokers matched with cases for age, gender and smoking history.

Langerhans Cell Histiocytosis

InCor Heart Institute

Evaluation of Exercise Capacity and Exercise Limitation in Patients With Pulmonary Langerhans Cell Histiocytosis

Evaluation of Exercise Capacity and Mechanisms of Exercise Limitation in Patients With Pulmonary Langerhans Cell Histiocytosis

Maximal O2 uptake capacity (VO2 max) during a cardiopulmonary exercise test (mL/kg/min)

Distance during a six-minute walk test in meters

SF-36 questionnaire to evaluate quality of life

Diffusing capacity for carbon monoxide (mL/min/mmHg)

Serial measurement of inspiratory capacity during incremental cardiopulmonary exercise test (L)

FEV1 (Pulmonary lung function - spirometry) - L

Velocity measurement of tricuspid regurgitant jet evaluated by transthoracic echocardiography (m/s)

Residual volume/total lung capacity ratio evaluated in pulmonary lung function - plethysmography

Residual volume evaluated in pulmonary lung function - plethysmography (L)

Desaturation during a six-minute walk test (%)

Ejection fraction evaluated by transthoracic echocardiography (%)

Diameter of the cardiac chambers evaluated by transthoracic echocardiography (cm)

granuloma/hypoxia

Immune Response in Hypersensitivity Pneumonitis

Evaluation of Exercise Capacity and Exercise Limitation in Patients With Pulmonary Langerhans Cell Histiocytosis

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