MIF- Thyroxine Interactions in the Pathogenesis of Pulmonary Arterial Hypertension

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