Pilot Study of the Safety and Efficacy of Anakinra (Recombinant Human Interleukin-1 Receptor Antagonist) in Pulmonary Hypertension

Inclusion Criteria:

- Age >18 years

- Diagnosis of PAH (from prior right heart catheterization), with functional
class III symptoms of right ventricle failure despite optimal PAH therapy

- Mean pulmonary artery pressure >25 mmHg

- Pulmonary capillary wedge pressure <15 mmHg

- Pulmonary vascular resistance >3 wood units

Exclusion Criteria:

- PAH due to connective tissue disease (including scleroderma), interstitial lung
disease (total lung capacity <65% predicted value), or cirrhosis (portopulmonary
hypertension)

- Angina or electrocardiograph (ECG) changes that limit maximum exertion during
cardiopulmonary exercise testing or baseline ECG changes that limit the ability to
detect ischemia (i.e. left bundle-branch block).

- Recent (<14 days) use of anti-inflammatory drugs (not including NSAIDs), chronic
inflammatory disorder (including but not limited to rheumatoid arthritis, systemic
lupus erythematosus), malignancy, active infection, or any comorbidity limiting
survival or ability to complete the study

- Severe kidney dysfunction (eGFR <30 mL/min)

- Thrombocytopenia (<50,000/mm3), or neutropenia (absolute neutrophil count <1,500/mm3)

- Refusal by a woman of childbearing potential (not post-menopausal or surgically
sterile) to use a medically acceptable form of birth control (including, but not
limited to, a diaphragm, an intrauterine device (IUD), progesterone implants or
injections, oral contraceptives, the double-barrier method, or a condom) throughout
the duration of the study

- History of hypersensitivity to anakinra or E. coli products

- Latex or rubber allergy

- Inability to give informed consent