TY  - JOUR
U1  - Zeitschriftenartikel, wissenschaftlich - begutachtet (reviewed)
A1  - Nagel, Christian
A1  - Ewert, Ralf
A1  - Egenlauf, Benjamin
A1  - Lehmkuhl, Hans B.
A1  - Rosenkranz, Stephan
A1  - Benjamin, Nicola
A1  - Schwenger, Vedat
A1  - Herth, Felix J.F.
A1  - Grünig, Ekkehard
T1  - Safety and Efficacy of Immunoadsorption as an Add-On to Medical Treatment in Patients with Severe Idiopathic Pulmonary Arterial Hypertension
JF  - Respiration
N2  - 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.
KW  - -
KW  - Immunoadsorption
KW  - Plasmapheresis
KW  - Pulmonary arterial hypertension
KW  - Autoantibodies
UN  - https://nbn-resolving.org/urn:nbn:de:gbv:9-opus-30879
SN  - 0025-7931
SS  - 0025-7931
SN  - 1423-0356
SS  - 1423-0356
U6  - https://doi.org/10.1159/000478744
DO  - https://doi.org/10.1159/000478744
PM  - 28787715
VL  - 94
IS  - 3
SP  - 263
EP  - 271
PB  - S. Karger AG
CY  - Basel, Switzerland
ER  -