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 IIIsymptoms 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 lungdisease (total lung capacity <65% predicted value), or cirrhosis (portopulmonaryhypertension)

• Angina or electrocardiograph (ECG) changes that limit maximum exertion duringcardiopulmonary exercise testing or baseline ECG changes that limit the ability todetect ischemia (i.e. left bundle-branch block).

• Recent (<14 days) use of anti-inflammatory drugs (not including NSAIDs), chronicinflammatory disorder (including but not limited to rheumatoid arthritis, systemiclupus erythematosus), malignancy, active infection, or any comorbidity limitingsurvival 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 surgicallysterile) to use a medically acceptable form of birth control (including, but notlimited to, a diaphragm, an intrauterine device (IUD), progesterone implants orinjections, oral contraceptives, the double-barrier method, or a condom) throughoutthe duration of the study

• History of hypersensitivity to anakinra or E. coli products

• Latex or rubber allergy

• Inability to give informed consent