Pneumologie 2018; 72(S 01): S76
DOI: 10.1055/s-0037-1619321
Sektion 6 – Kardiorespiratorische Interaktion
Posterbegehung – Titel: Kardiorespiratorische Interaktion in Ruhe, im Schlaf und unter Belastung
Georg Thieme Verlag KG Stuttgart · New York

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

C Nagel
1   Lungenzentrum, Zentrum für Pulmonale Hypertonie, Thoraxklinik am Universitätsklinikum Heidelberg
,
R Ewert
2   Zentrum für Innere Medizin, Klinik für Innere B, Bereich Pneumologie, Universitätsmedizin Greifswald Körperschaft des Öffentlichen Rechts
,
B Egenlauf
3   Pneumologie und Beatmungsmedizin, Zentrum für Pulmonale Hypertonie, Thoraxklinik am Universitätsklinikum Heidelberg
,
S Rosenkranz
4   Department of Cardiology, University of Cologne
,
N Benjamin
5   Zentrum für Pulmonale Hypertonie, Thoraxklinik am Universitätsklinikum Heidelberg; Translational Lung Research Centre Heidelberg (TLRC), German Centre for Lung Research (DZL)
,
V Schwenger
6   Transplantationszentrum, Klinik für Nieren- u. Hochdruckkrankheiten, Klinikum Stuttgart – Katharinenhospital
,
FJF Herth
7   Pneumology, Institute of Internal Medicin III, Thoraxklinik, University Hospital Heidelberg
,
E Grünig
5   Zentrum für Pulmonale Hypertonie, Thoraxklinik am Universitätsklinikum Heidelberg; Translational Lung Research Centre Heidelberg (TLRC), German Centre for Lung Research (DZL)
› Author Affiliations
Further Information

Publication History

Publication Date:
21 February 2018 (online)

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Background:

Despite optimized medical therapy, severe idiopathic pulmonary arterial hypertension (IPAH) is a devastating disease with poor outcome. Autoantibodies have been detected in IPAH that can contribute to the worsening of the disease.

Objectives:

The objective of this prospective, open-label, single-arm, multicenter trial was to evaluate safety and efficacy of immunoadsorption (IA) as an add-on to optimized medical treatment in patients with idiopathic pulmonary arterial hypertension (IPAH).

Methods:

10 IPAH-patients received IA over 5 days. Clinical parameters including hemodynamics measured by right heart catheter were assessed at baseline and after three and six months. Primary end point was the change in pulmonary vascular resistance (PVR). Secondary endpoints included the change of six-minute walking distance (6MWD), quality of life, safety and plasma levels of IgG and autoantibodies.

Results:

Evaluation of 10 IPAH-patients (75% female, 51 ± 12 years, 166 ± 10 cm, WHO-FC III, 53% on combination therapy) revealed that IA was a safe procedure that efficiently removed IgG and autoantibodies from the circulation. After three months, mean PVR significantly improved by 13,2% (p = 0,03), cardiac index improved by 13,1%, no significant changes were found in 6MWD. The quality of life subscale physical functioning significantly improved after 6 months. Serious adverse events in 3 patients were possibly related to IA and included pneumonia, temporary disturbance in attention and thrombocytopenia.

Conclusions:

IA as add-on to targeted medical treatment in IPAH is a safe procedure with beneficial effects on hemodynamics, especially in patients with high levels of autoantibodies. Larger scaled controlled studies are needed to assess efficacy in IPAH and to identify responders.