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

Chronic Obstructive Pulmonary Disease (COPD) is a major cause of morbidity and mortality throughout the world.COPD, a common preventable and treatable disease, is characterized by persistent airflow limitation that is usually progressive and associated with an enhanced chronic inflammatory response in the airways and the lung to noxious particles or gases. Exacerbations and comorbidities contribute to the overall severity in individual patients.Spirometry is required to make a clinical diagnosis of COPD; the presence of a post-bronchodilator FEV1/FVC < 0.70 confirms the presence of persistent airflow limitation and thus of COPD .

Interstitial lung disease is a general category that includes many different lung conditions. All interstitial lung diseases affect the interstitium, a part of the lungs' anatomic structure.

Obstructive sleep apnea (OSA) is a sleep disorder that involves cessation or significant decrease in airflow in the presence of breathing effort. It is the most common type of sleep-disordered breathing and is characterized by recurrent episodes of upper airway collapse during sleep.
These episodes are associated with recurrent oxyhemoglobin desaturations and arousals from sleep.
OSA that is associated with excessive daytime sleepiness is commonly called obstructive sleep apnea syndrome—also referred to as obstructive sleep apnea-hypopnea syndrome.

Pulmonary Hypertension

Kasr Alaini

Clinical Profile of Patients With Pulmonary Hypertension Due to Lung Diseases ( Single Center Experience)

Mostafa Elshazly

Professor of pulmonary Medicine

Cairo University

All patients underwent extensive evaluation:
- Body composition, Diabetes; dyslipidemia, hemoconcentration, hypertension, exercise capacity: 6-Minute Walk test procedure, renal function, inflammatory markers, cause and place of death, time tracking concentrator, quality of life and pulmonary Function.

Chronic Obstructive Pulmonary Disease and Allied Conditions

The Global Initiative for Chronic Obstructive Lung Disease (GOLD) works with health care professionals and public health officials to raise awareness of Chronic Obstructive Pulmonary Disease (COPD) and to improve prevention and treatment of this lung di

Predictors of Mortality in Patients With Advanced Lung Disease in Home Oxygen Therapy

Evaluate the results of evaluations carried out in patients who died and had advanced lung disease diagnosis in use of home oxygen, compared to patients who did not die and the normal population

Assess the amount of oxygen that was used by patients during treatment indication and measurement of variable

Measure the quality of life of these patients and compared with the normal population and patients who died and had survival for longer period

Search the causes of death of these patients and the place to compare with the current epidemiology and relate to the main disease

Vinícius Pafume de Oliveira

Physiotherapist

Federal University of Uberlandia

Eligible participants received 5 mg ambrisentan once daily for the first 24 weeks. One dose reduction to 2.5 mg was permitted during the 24-week fixed-dose treatment period if the participant did not tolerate the study drug. After the initial 24-week treatment period, investigators could adjust the study drug dose as clinically indicated (available doses were 2.5, 5, and 10 mg).

Ambrisentan

Sex: Female, Male

Change From Baseline to Week 24 in 6 Minute Walk Distance (6MWD)

Change from Baseline to Week 24 in Borg Dyspnea Index. The Borg Dyspnea Index of Perceived Exertion Scores range from 0 to 10. Best and Worst values are: 0 (Best) to 10 (Worst). Scales are described as rating of breathlessness and its description: 0= none; 0.5= very,very slight (just noticeable); 1= very slight; 2=slight; 3= moderate; 4= somewhat severe; 5= severe; 6 (in between severe and very severe); 7= very severe; 8 (in between very, very severe and maximum); 9= very, very severe; and 10= maximum.

Change From Baseline to Week 24 in Borg Dyspnea Index

Change from Baseline to Week 48 in Borg Dyspnea Index. The Borg Dyspnea Index of Perceived Exertion Scores range from 0 to 10. Best and Worst values are: 0 (Best) to 10 (Worst). Scales are described as rating of breathlessness and its description: 0= none; 0.5= very,very slight (just noticeable); 1= very slight; 2=slight; 3= moderate; 4= somewhat severe; 5= severe; 6 (in between severe and very severe); 7= very severe; 8 (in between very, very severe and maximum); 9= very, very severe; and 10= maximum.

Change From Baseline to Week 48 in Borg Dyspnea Index

Percent Change From Baseline to Week 24 in B-type Natriuretic Peptide (BNP)

Percent Change From Baseline to Week 48 in BNP

Change from baseline in World Health Organization functional class (WHO) at Week 24 is the incidence of participants that improved, had no change, or worsened. WHO categories are 1 to 4 with the worse category at 4. Improvement = a category change from baseline of <= -1: change of -3 (eg, WHO from 4 to 1), change of -2 (eg, WHO from 3 to 1), change of -1 (eg, WHO from 2 to 1). Inversely, participants worsening are those with a category change from baseline of at least +1. No change in WHO functional class represents the percentage of participants with a change in category from baseline of 0.

Change From Baseline to Week 24 in WHO Functional Class

Change from baseline in WHO at Week 48 is expressed as the incidence of participants that improved, had no change or worsened. WHO categories range from 1 to 4 with the worse category at 4. Improvement = a category change from baseline of <= -1: change of -3 (eg, WHO from 4 to 1), change of -2 (eg, WHO from 3 to 1), change of -1 (eg, WHO from 2 to 1). Inversely, participants worsening are those with a category change from baseline of at least +1. No change in WHO functional class represents the percentage of participants with a change in category from baseline of 0.

Change From Baseline to Week 48 in WHO Functional Class

Change from baseline to Week 24 in the SF-36 health survey physical functioning scale. 10 activities rated by health limitations using 3 categories (1= Yes, limited a lot; 2= Yes, limited a little; and 3= No, not limited at all). The best score is 3 and the worst score is 1. Scores are transformed by subtracting the unit by the lowest raw score and dividing by the raw score range. The scores are then standardized with the 1998 General United States (US) population mean and standard deviation (SD). Finally, the scores are transformed to the norm-based scoring with a mean of 50 and SD of 10.

Change From Baseline to Week 24 in SF-36 Health Survey Physical Functioning Scale

Change from baseline to Week 48 in the SF-36 health survey physical functioning scale. 10 activities are rated by health limitations using 3 categories (1= Yes, limited a lot; 2= Yes, limited a little; and 3= No, not limited at all). The best score is 3 and the worst score is 1. Scores are transformed by subtracting the unit by the lowest raw score and dividing by the raw score range. The scores are then standardized with the 1998 General US population mean and standard deviation. Finally, the scores are transformed to the norm-based scoring with a mean of 50 and standard deviation of 10.

Change From Baseline to Week 48 in SF-36 Health Survey Physical Functioning Scale

Clinical worsening: occurrence of death, lung transplantation, hospitalization for PH, atrial septostomy, a change to chronic prostanoid or sildenafil treatment due to protocol-defined worsening criteria, or study withdrawal due to the addition of other clinically approved PH therapeutic agents

Percent of Participants With no Clinical Worsening of Pulmonary Hypertension (PH) at Week 24

Percent of Participants With no Clinical Worsening of PH at Week 48

Defined by occurrence of death, lung transplantation, or study withdrawal due to the addition of other clinically approved PAH therapeutic agents

Failure-free Treatment Status

Defined by no addition of sildenafil, iloprost, treprostinil, or epoprostenol to ongoing ambrisentan treatment

Monotherapy Treatment Status

Defined as not dying during study participation

sarcoidosis/hypoxia

Immune Response in Hypersensitivity Pneumonitis

Clinical Profile of Patients With Pulmonary Hypertension Due to Lung Diseases ( Single Center Experience)

Predictors of Mortality in Patients With Advanced Lung Disease in Home Oxygen Therapy

Safety and Efficacy Study of Ambrisentan in Subjects With Pulmonary Hypertension

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