Search

Introduction: Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by widespread venous/arterial thrombosis and pulmonary artery aneurysms (PAAs), which is associated with serious morbidity and mortality. All fatalities reported in HSS resulted from unpredictable fatal suffocating hemoptysis. Therefore, it is necessary to recognize pulmonary complications at an early stage of the disease., Objectives: The aims of this study are to develop a reference atlas of images depicting the characteristic features of HSS by computed tomography pulmonary angiography (CTPA). To make a guide for physicians by developing a classification of PAAs according to the severity and risk of complications associated with each distinct lesion type., Methods: The Members of the HSS International Study Group (HSSISG) collected 42 cases, with high-quality CTPA images in one radiology station and made reconstructions from the source images. These detailed CTPA studies were reviewed for final image selection and approved by HSSISG board members. We classified these findings according to the clinical course of the patients., Results: This atlas describes the CTPA images that best define the wide spectrum of pulmonary vasculitis observed in HSS. Pulmonary aneurysms were classified into six radiographic patterns: from true stable PAA with adherent in-situ thrombosis to unstable leaking PAA, BAA and/or PAP with loss of aneurysmal wall definition (most prone to rupture), also CTPA images demonstrating right ventricular strain and intracardiac thrombosis., Conclusion: The HSSISG reference atlas is a guide for physicians regarding the CTPA radiological findings, essential for early diagnosis and management of HSS-related pulmonary vasculitis. Key Points • The Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by extensive vascular thrombosis and pulmonary artery aneurysms (PAAs) that can lead to significant morbidity and mortality. • All fatalities reported in HSS were related to unpredictable massive hemoptysis; therefore, it is critical to recognize pulmonary complications at an early stage of the disease. • The HSS International Study Group reference atlas  classifies pulmonary vasculitis in HSS at 6 different stages of the disease process and defines the different radiological patterns of pulmonary vasculitis notably pulmonary artery aneurysms, as detected by computed tomography pulmonary angiography (CTPA). • The main aim of the classification is to make a guide for physicians about this rare syndrome. Such a scheme has never been reached before since the first description of the syndrome by Hughes and Stovin since 1959. This classification will form the basis for future recommendations regarding diagnosis and treatment of this syndrome., (© 2021. The Author(s).)

Background: Cardiac and vascular involvement in Behçet disease (BD), also referred as vasculo BD, is frequent. We aimed to describe clinical characteristics, predictive factors and management of vasculo BD in the Tunisian context., Methods: We retrospectively studied 213 records of all BD patients followed between January 2004 and May 2016 in the Internal Medicine Department and who fulfilled the ISGBD criteria. We described first clinical features of BD with cardiac and vascular involvement then predictive factors were studied in univariate then multivariate analysis., Results: Among the 213 patients, 64 (30%) were diagnosed as having vasculo BD. The mean age at diagnosis was 31.5 years. About 81.25% of them were males and 18.75% females. Vascular involvement associated or not with cardiac involvement was found in 64 patients (30%). Deep venous thromboses are most common (62.5%) compared with superficial ones (23.4%), pulmonary arterial thrombosis (14.1%) or aneurysms (9.4%). Cardiac involvement is ranging from pericarditis (1.6%) to intra cardiac thrombosis (3.1%) and myocardial infarction (1.6%). Predictive factors associated with cardiac and vascular involvement in BD are male gender (OR = 3.043, 95% CI = 1.436-6.447, p = 0.004), erythema nodosum (OR = 4.134, 95% CI = 1.541-11.091, p = 0.005) and neurologic involvement (OR = 2.46, 95% CI = 1.02-5.89, p = 0.043)., Conclusion: Cardiac and vascular involvement in BD is frequent in the Tunisian context with a broad spectrum of manifestations ranging from vascular involvement to cardiac one. Male gender, patients with erythema nodosum or neurologic involvement are prone to develop cardiac or vascular features of BD needing therefore a close monitoring.