MIF- Thyroxine Interactions in the Pathogenesis of Pulmonary Arterial Hypertension

MIF- Thyroxine Interactions in the Pathogenesis of Pulmonary Arterial Hypertension

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

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.

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