Behçet's Syndrome With Multiple Bilateral Pulmonary Aneurysms Associated With Endomyocardial Fibrosis Presented With Pulmonary Emboli: A Case Report

Abstract

Behçet's syndrome (BS) is a rare chronic multisystemic inflammatory disorder of unknown etiopathogenesis. BS is classified as a vasculitis of variable vessel size, which can manifest in both arterial and venous blood vessels. BS commonly presents with mucocutaneous and ocular manifestations. Superficial and deep vein thrombosis is present in 50% of patients, with atypical venous thrombosis affecting the inferior vena cava, superior vena cava, hepatic veins with Budd-Chiari syndrome, portal vein, cerebral sinuses, and right atrium and ventricle. Arterial manifestations include in situ thrombosis, pulmonary artery aneurysms, aneurysms of the abdominal aorta, and aneurysms of visceral and peripheral arteries. This article reports a new case of BS in a 28-year-old female patient who presented with severe dyspnea and hemoptysis. Echocardiography and cardiovascular magnetic resonance imaging led to the diagnosis of endomyocardial fibrosis and a large right ventricular thrombus with pulmonary embolism. Computed tomography angiography revealed multiple pulmonary aneurysms and emboli. Rare findings such as endomyocardial fibrosis and Budd-Chiari syndrome were noted. This case highlights the role of medical imaging modalities in diagnosing rare syndromes such as BS, as demonstrated in the current case.

Keywords: behçet's syndrome; budd-chiari syndrome; endomyocardial fibrosis; hughes-stovin syndrome; multiple pulmonary artery aneurysms; pulmonary artery thrombosis.

Figure 1. Cardiac magnetic resonance (CMR): (a, b) Localized and cine four-chamber views and (c, d) short-axis and two-chamber views of the right side show a dilated right atrium (RA) (red arrow), small right ventricle (RV) cavity with a thrombus filling the small lumen (yellow arrow), and a small pericardial effusion. (e, f) Axial views noted bilateral pulmonary artery dilatation with a filling defect. (g, h, i) Late gadolinium enhancement images reveal V-shaped subendocardial enhancement of the RV wall extending into the inflow tract (arrows).

Figure 2. Computed tomography pulmonary angiography (CTPA): (a) Arterial phase shows right pulmonary artery aneurysmal dilatation with a filling defect representing thrombosis (red arrow) and left pulmonary artery dilatation with wall thickening (yellow arrow). (b) The late venous phase shows a filling defect in the superior vena cava (SVC), indicating SVC thrombosis (yellow arrow). (c) Arterial phase and (d) late venous phase show inferior vena cava (IVC) filling defects (yellow arrows) with hepatomegaly and heterogeneous enhancement (nutmeg liver), indicating an association with Budd-Chiari syndrome.