Primary Systemic Vasculitides as a Cause of Group IV Pulmonary Hypertension

Creative Commons License

AKDOĞAN A., Sarı A., Sade L. E.

Anatolian Journal of Cardiology, vol.27, pp.677-687, 2023 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Review
  • Volume: 27
  • Publication Date: 2023
  • Doi Number: 10.14744/anatoljcardiol.2023.3650
  • Journal Name: Anatolian Journal of Cardiology
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, CINAHL, EMBASE, MEDLINE, Directory of Open Access Journals, TR DİZİN (ULAKBİM)
  • Page Numbers: pp.677-687
  • Keywords: Behçet’s disease, group IV PH, primary systemic vasculitides, Takayasu arteritis
  • Hacettepe University Affiliated: Yes


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.