ISSN 2149-2263 | E-ISSN 2149-2271
The Anatolian Journal of Cardiology
Primary Systemic Vasculitides as a Cause of Group IV Pulmonary Hypertension [Anatol J Cardiol]
Anatol J Cardiol. 2023; 27(12): 677-687 | DOI: 10.14744/AnatolJCardiol.2023.3650

Primary Systemic Vasculitides as a Cause of Group IV Pulmonary Hypertension

Ali Akdoğan1, Alper Sarı2, Leyla Elif Sade3
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

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.

Keywords: Behçet’s disease, group IV PH, primary systemic vasculitides, Takayasu arteritis

Ali Akdoğan, Alper Sarı, Leyla Elif Sade. Primary Systemic Vasculitides as a Cause of Group IV Pulmonary Hypertension. Anatol J Cardiol. 2023; 27(12): 677-687

Corresponding Author: Alper Sarı
Manuscript Language: English


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