MIF- Thyroxine Interactions in the Pathogenesis of Pulmonary Arterial Hypertension

Study Overview
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Brief Summary
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The investigators will investigate the interrelationship of macrophage migration inhibitory factor (MIF) and free T4 in patients with PAH.

Detailed Description
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The investigators will test our hypothesis and accomplish our objectives by pursuing three Specific Aims: To investigate the interrelationship between macrophage migration inhibitory factor (MIF) and free T4 in patients with PAH with a working hypothesis that an increased release of MIF into the pulmonary vasculature during cardiopulmonary exercise changes the normal balance between plasma MIF and T4.

Official Title
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MIF- Thyroxine Interactions in the Pathogenesis of Pulmonary Arterial Hypertension

Conditions
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Pulmonary Hypertension, Pulmonary Arterial Hypertension, Interstitial Lung Disease

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Subject must be at least 18 years of age and have confirmed pulmonary hypertension (PH) and interstitial lung disease (ILD). ILD was identified on high resolution computed tomography along with a total lung capacity (TLC) <70% of predicted value on pulmonary function tests (PFTs) with no evidence of pulmonary hypertension on echocardiogram. PAH group patients were defined as patients with a mean pulmonary artery pressure >25 mmHg at rest and a wedge pressure of <15 mmHg (millimeters of mercury), measured by right heart catheterization (WHO group I PAH). PH+ILD group, subjects with evidence of ILD on high resolution CT Scan and mean pulmonary artery pressure >25 mmHg on right heart catheterization. Patient with evidence of Chronic thromboembolic disease on CT angiogram and mean pulmonary artery pressure >25mmHg on right heart catheterization. (WHO group IV) Patient with Pulmonary Hypertension due to other conditions, such as Sarcoidosis, Gauchers disease, Hemolytic Anemia and Myeloproliferative disorders. Normal control group: Subjects without cardiopulmonary disease, with a normal PFT and ECG. Exclusion Criteria: Failure to obtain informed consent; Pregnancy Subjects with cardiopulmonary disease other than PH and ILD; Unexplained abnormal PFT, ECG, Routine blood/chemistries test History or existing other sever organ/system diseases Defined Acute pulmonary embolism Age < 18 years

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Healthy Controls<br>Subjects are made up of healthy adults Interventions: Venous blood drawn before and after cardiopulmonary exercise test Will have a resting 12 lead EKG Undergo pulmonary function test | |
| Affected<br>Subjects have Pulmonary Hypertension Subjects have Pulmonary hypertension with Interstitial lung disease Subjects have Interstitial lung disease Interventions: Venous blood drawn before and after cardiopulmonary exercise test Will have a resting 12 lead EKG Undergo pulmonary function test | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| To examine the MIF-T4 relationship in patients before/after a standardized exercise challenge | Assess levels of the proinflammatory cytokine macrophage migration inhibitory factor (MIF) before/after standard exercise challenge | up to 30 mins |