@article{NagelEwertEgenlaufetal.2017,
  author    = {Christian Nagel and Ralf Ewert and Benjamin Egenlauf and Hans B. Lehmkuhl and Stephan Rosenkranz and Nicola Benjamin and Vedat Schwenger and Felix J.F. Herth and Ekkehard Gr{\"u}nig},
  title     = {Safety and Efficacy of Immunoadsorption as an Add-On to Medical Treatment in Patients with Severe Idiopathic Pulmonary Arterial Hypertension},
  series   = {Respiration},
  volume    = {94},
  number    = {3},
  publisher = {S. Karger AG},
  address   = {Basel, Switzerland},
  issn      = {0025-7931},
  doi       = {10.1159/000478744},
  url       = {https://nbn-resolving.org/urn:nbn:de:gbv:9-opus-30879},
  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.},
  language  = {en}
}