Safety and efficacy of immunoadsorption as an add-on to medical treatment in patients with severe idiopathic pulmonary arterial hypertension

• Verfasst von: Nagel, Christian [VerfasserIn]
• Verfasst von: Ewert, Ralf [VerfasserIn]
• Verfasst von: Egenlauf, Benjamin [VerfasserIn]
• Verfasst von: Lehmkuhl, Hans Brendan [VerfasserIn]
• Verfasst von: Rosenkranz, Stephan [VerfasserIn]
• Verfasst von: Benjamin, Nicola [VerfasserIn]
• Verfasst von: Schwenger, Vedat [VerfasserIn]
• Verfasst von: Herth, Felix [VerfasserIn]
• Verfasst von: Grünig, Ekkehard [VerfasserIn]
• Titel: Safety and efficacy of immunoadsorption as an add-on to medical treatment in patients with severe idiopathic pulmonary arterial hypertension
• Verf.angabe: Christian Nagel, Ralf Ewert, Benjamin Egenlauf, Hans B. Lehmkuhl, Stephan Rosenkranz, Nicola Benjamin, Vedat Schwenger, Felix J. F. Herth, Ekkehard Grünig
• E-Jahr: 2017
• Jahr: August 5, 2017
• Umfang: 9 S.
• Teil: volume:94
• Teil: year:2017
• Teil: number:3
• Teil: pages:263-271
• Teil: extent:9
• Fussnoten: Gesehen am 04.09.2018
• Titel Quelle: Enthalten in: Respiration
• Ort Quelle: Basel : Karger, 1944
• Jahr Quelle: 2017
• Band/Heft Quelle: 94(2017), 3, Seite 263-271
• ISSN Quelle: 1423-0356
• DOI: doi:10.1159/000478744
• Datenträger: Online-Ressource
• Sprache: eng
• K10plus-PPN: 1580642845

Abstract

<b><i>Background:</i></b> 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. <b><i>Objectives:</i></b> 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. <b><i>Methods:</i></b> 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. <b><i>Results:</i></b> 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% (<i>p</i> = 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. <b><i>Conclusions:</i></b> 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.