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Review
. 2023 Dec 1;27(12):677-687.
doi: 10.14744/AnatolJCardiol.2023.3650. Epub 2023 Nov 21.

Primary Systemic Vasculitides as a Cause of Group IV Pulmonary Hypertension

Ali Akdoğan  1 , Alper Sarı  2 , Leyla Elif Sade  3
Affiliations
Review

Primary Systemic Vasculitides as a Cause of Group IV Pulmonary Hypertension

Ali Akdoğan et al. Anatol J Cardiol. .

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.

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Figures

Figure 1.
Figure 1.
Imaging findings of patients with Takayasu arteritis and group IV pulmonary hypertension: (A) cardiomegaly; (B) systemic arterial involvement and collateral vessels; (C) dilatation of the main pulmonary artery, wall thickening and luminal narrowing in right and left main pulmonary arteries; and (D) wall thickening in both the left upper lobe and left lower lobe pulmonary arteries; subtotal occlusion in the left upper lobe pulmonary artery.
Figure 2.
Figure 2.
Treatment algorithm for Takayasu arteritis patients with group IV Pulmonary hypertension. *According to 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. PH, pulmonary hypertension; TTE, transthoracic echocardiography; PAI, pulmonary arterial involvement; RHC, right heart catheterization; TA, Takayasu arteritis; PEA, pulmonary endarterectomy.
Figure 3.
Figure 3.
Imaging findings of pulmonary involvement in patients with Behçet’s disease: A) pulmonary artery aneurysm; B) image of aneurysm after bleeding in the same patient; C) occlusion of basal segmental branch of left lower lobe pulmonary artery (arrow); D) coronal maximum intensity projection (MIP) reformat image shows no filling of left lower lobe basal segment arteries, calibration of right basal segment arteries are decreased and distal branches are depleted; E) multivessel involvement in pulmonary angiography.
Figure 4.
Figure 4.
Treatment algorithm for patients with Behçet’s disease and group IV Pulmonary hypertension. *According to 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. PAI, pulmonary arterial involvement; TTE, transthoracic echocardiography; RHC, right heart catheterization; PH, pulmonary hypertension; PAA, pulmonary artery aneurysm; PEA, pulmonary endarterectomy.
See this image and copyright information in PMC

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