Proof of Concept Study of IMMUNOadsorption Therapy in Patients With Idiopathic Pulmonary Arterial Hypertension

Inclusion Criteria

• •Able to understand and willing to sign the Informed Consent Form
• •age \> 18 and \< 80 years at time of informed consent
• •Idiopathic pulmonary arterial hypertension diagnosed by right heart catheter
• •WHO functional classification III
• •Application of conventional IPAH therapy, which has been stable for the prior 8 weeks
• •Able to perform a 6-minute-walk test (MWT)
• •Negative pregnancy test (β-HCG) at the start of the trial and appropriate contraception throughout the trial for women with child-bearing potential.
• •Contraindication for right heart catheterization
• •Any change in disease-targeted therapy within 8 weeks prior to inclusion
• •Walking impairment due to causes other than IPAH that would preclude performance of a 6MWT
• •Any patient who has received any investigational medication within 8 weeks prior to the start of this trial or who is scheduled to receive another investigational drug during the course of the trial between visits V1 and V9
• •Known intolerance to immunoadsorption in general or to one of the excipients (e.g. sheep antibodies or agarose) or other supporting agents
• •Hemoglobin concentration of \< 75% below the lower limit of normal
• •Systolic blood pressure \< 85 mmHg
• •Lack of compliance or other similar reason that, in the judgment of the Investigator, precludes satisfactory participation in the trial
• •Concurrent severe or uncontrolled medical disease (i.e. uncontrolled diabetes, congestive heart failure, myocardial infarction within 6 months prior to the study, unstable and uncontrolled hypertension, chronic renal disease, or active uncontrolled generalized viral, bacterial and/ or mycotic infection) which by assessment of the treating Investigator could compromise patients safety or participation in the study
• •Drug or alcohol abuse within the last 5 years
• •Hypercoagulability
• •Known severe immunodeficiency (e.g. AIDS)
• •Severe lung disease: FEV1/FVC \< 50%, total capacity \< 60% of normal value
• •Indications that prohibit transient anticoagulation using Heparin and/or ACD-A-solutions