@article{NagelEwertEgenlaufetal.2017, author = {Nagel, Christian and Ewert, Ralf and Egenlauf, Benjamin and Lehmkuhl, Hans B. and Rosenkranz, Stephan and Benjamin, Nicola and Schwenger, Vedat and Herth, Felix J.F. and Gr{\"u}nig, Ekkehard}, title = {Safety and Efficacy of Immunoadsorption as an Add-On to Medical Treatment in Patients with Severe Idiopathic Pulmonary Arterial Hypertension}, journal = {Respiration}, volume = {94}, number = {3}, issn = {0025-7931}, doi = {10.1159/000478744}, institution = {Kliniken und Polikliniken f{\"u}r Innere Medizin}, pages = {263 -- 271}, year = {2017}, abstract = {Background: Despite optimized medical therapy, severe idiopathic pulmonary arterial hypertension (IPAH) is a devastating disease with a poor outcome. Autoantibodies have been detected in IPAH that can contribute to worsening of the disease. Objectives: The objective of this prospective, open-label, single-arm, multicenter trial was to evaluate the safety and efficacy of immunoadsorption (IA) as an add-on to optimized medical treatment for patients with IPAH. Methods: A total of 10 IPAH patients received IA over 5 days. Their clinical parameters, including hemodynamics measured by right heart catheter, were assessed at baseline and after 3 and 6 months. The primary endpoint was the change in pulmonary vascular resistance (PVR). Secondary endpoints included the change in 6-min walking distance, quality of life, safety, and plasma levels of IgG and autoantibodies. Results: The evaluation of the 10 IPAH patients (75\% female; 51 ± 12 years; 166 ± 10 cm; WHO functional class III; 53\% on combination therapy) revealed that IA was a safe procedure that efficiently removed IgG and autoantibodies from the circulation. After 3 months, the mean PVR improved significantly by 13.2\% (p = 0.03) and the cardiac index improved by 13.1\%, but no significant changes were found in 6-min walking distance. The quality of life physical functioning subscale score significantly improved after 6 months. The serious adverse events in 3 patients were possibly related to IA and included pneumonia, temporary disturbance in attention, and thrombocytopenia. Conclusions: IA as an add-on to targeted medical treatment for IPAH is a safe procedure with beneficial effects on hemodynamics, especially in patients with high levels of autoantibodies. Larger-scale controlled studies are needed to assess its efficacy in IPAH and to identify responders.}, subject = {-}, language = {en} }