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