Pilot Study of the Safety and Efficacy of Anakinra in Pulmonary Arterial Hypertension

Patients with pulmonary arterial hypertension will undergo cardiopulmonary exercise testing
(CPET) at baseline and at 2 weeks. After the initial CPET, all patients will receive anakinra
as a daily injection for 2 weeks. Patients will be instructed in the use of anakinra during
their initial visit. Our primary outcome will be the difference in the exercise capacity of
patients with PAH, as measured by maximal uptake of oxygen on CPET.

Inclusion Criteria:

- age over 18

- functional class II or III symptoms of right ventricular failure despite optimal PAH
therapy

- mean pulmonary artery pressure >25mmHg on previous right heart catheterization

- pulmonary arterial wedge pressure <15mmHg on previous right heart catheterization

- pulmonary vascular resistance >3 wood units on previous right heart catheterization

Exclusion Criteria:

- PAH due to connective tissue disease

- angina or electrocardiograph changes that limit maximum exertion during
cardiopulmonary exercise testing or baseline EKG changes that limit the ability to
detect ischemia

- recent (<14 days) use of anti-inflammatory drugs (not including NSAIDs), chronic
inflammatory disorder, malignancy, active infection, or any comorbidity limiting
survival or ability to complete the study

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

- thrombocytopenia (<50,000/mm3), or neutropenia (absolute neutrophil count <1500/mm3)

- refusal by a woman of childbearing potential to use a medically acceptable form of
birth control

- history of hypersensitivity to anakinra or E. coli products

- latex or rubber allergy

- inability to give informed consent

- non-English speaking