Universität zu Köln

Nagel, Christian, Ewert, Ralf, Egenlauf, Benjamin ORCID: 0000-0003-2466-1420, Lehmkuhl, Hans B., Rosenkranz, Stephan, Benjamin, Nicola, Schwenger, Vedat, Herth, Felix J. F. and Gruenig, Ekkehard (2017). Safety and Efficacy of Immunoadsorption as an Add-On to Medical Treatment in Patients with Severe Idiopathic Pulmonary Arterial Hypertension. Respiration, 94 (3). S. 263 - 272. BASEL: KARGER. ISSN 1423-0356

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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 disturbancein 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. (C) 2017 The Author(s) Published by S. Karger AG, Basel

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Nagel, Christian UNSPECIFIED UNSPECIFIED UNSPECIFIED Ewert, Ralf UNSPECIFIED UNSPECIFIED UNSPECIFIED Egenlauf, Benjamin UNSPECIFIED orcid.org/0000-0003-2466-1420 UNSPECIFIED Lehmkuhl, Hans B. UNSPECIFIED UNSPECIFIED UNSPECIFIED Rosenkranz, Stephan UNSPECIFIED UNSPECIFIED UNSPECIFIED Benjamin, Nicola UNSPECIFIED UNSPECIFIED UNSPECIFIED Schwenger, Vedat UNSPECIFIED UNSPECIFIED UNSPECIFIED Herth, Felix J. F. UNSPECIFIED UNSPECIFIED UNSPECIFIED Gruenig, Ekkehard UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-243963
DOI:	10.1159/000478744
Journal or Publication Title:	Respiration
Volume:	94
Number:	3
Page Range:	S. 263 - 272
Date:	2017
Publisher:	KARGER
Place of Publication:	BASEL
ISSN:	1423-0356
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language SUBSEQUENT IMMUNOGLOBULIN SUBSTITUTION; SYSTEMIC-LUPUS-ERYTHEMATOSUS; DILATED CARDIOMYOPATHY; HEART-TRANSPLANTATION; AUTOANTIBODIES; INFLAMMATION; ANTIBODIES; SUBCLASS; THERAPY; TARGETS Multiple languages Respiratory System Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/24396