Primary Systemic Vasculitides as a Cause of Group IV Pulmonary Hypertension
1Department of Rheumatology, Hacettepe University, Faculty of Medicine, Ankara, Türkiye
2Department of Rheumatology, Etlik City Hospital, Ankara, Türkiye
3Heart and Vascular Institute, University of Pittsburgh Medical Center, Pittsburgh, PA, USA
Anatol J Cardiol 2023; 27(12): 677-687 PubMed ID: 37986573 DOI: 10.14744/AnatolJCardiol.2023.3650
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Abstract

The primary systemic vasculitides are rare diseases characterized by vessel wall inflammation. Isolated pulmonary vasculitis, large-vessel vasculitis, and Behçet’s disease are mimickers of chronic thromboembolic pulmonary hypertension (CTEPH); group IV pulmonary hypertension (PH) can occur as a devastating complication in the course of these diseases. Pulmonary endarterectomy, balloon angioplasty, anticoagulation and pulmonary vasodilator agents are the main treatment options for CTEPH. There is no specific recommendation for the treatment of patients having group IV PH due to primary systemic vasculitides. We reviewed herein data about group IV PH due to primary systemic vasculitides.