Cardiac Manifestations in Behçet's Syndrome.

Purpose Of The Review: Behçet's Syndrome (BS) is a multisystemic vasculitis that can affect the heart, leading to pericarditis, myocarditis, intracardiac thrombosis, endomyocardial fibrosis, valvular dysfunction, and coronary artery disease. This review summarizes the clinical presentation, diagnostic challenges, and therapeutic strategies for cardiac involvement in BS.

Recent Findings: Advanced imaging techniques have revealed subclinical cardiac involvement in BS.

Primary versus systemic sclerosis-associated Raynaud's phenomenon: relationship with clinical and environmental factors.

Objectives: Raynaud's phenomenon (RP) can be induced by stress and environmental factors, occurring as a primary disease (pRP) or associated with connective tissue disease. RP is seen in more than 95% of patients with systemic sclerosis (SSc) and may precede its diagnosis by several years. Accordingly, there is a clear need to identify those patients with RP who will eventually develop connective tissue disease, including SSc.

Epigenetics in autoimmune diseases: Unraveling the hidden regulators of immune dysregulation.

Autoimmune diseases result from complex interactions between genetic and environmental factors. Recent advances in epigenetic research shed light on the intricate regulatory mechanisms that contribute to the development and progression of such conditions. The present review aims to explore the role of epigenetic modifications, including DNA methylation, histone modifications, and non-coding RNAs, in the context of autoimmune diseases.

Efficacy and safety of infliximab or adalimumab in severe mucocutaneous Behçet's syndrome refractory to traditional immunosuppressants: a 6-month, multicentre, randomised controlled, prospective, parallel group, single-blind trial.

Introduction: Evidence from randomised controlled trials on anti-tumour necrosis factor (TNF) agents in patients with Behçet's syndrome (BS) is low.

Method: We conducted a phase 3, multicentre, prospective, randomised, active-controlled, parallel-group study to evaluate the efficacy and safety of either infliximab (IFX) or adalimumab (ADA) in patients with BS. Adults patients with BS presenting with active mucocutaneous manifestations, occurring while on therapy with either azathioprine or cyclosporine for at least 3 months prior to study entry, were eligible.

Exploring relief for Behçet's disease refractory oral ulcers: a comparison of TNF inhibitors versus apremilast.

Objectives: Oral and genital ulcers are the hallmark manifestation of Behçet's disease (BD), significantly impacting patients' quality of life. Our study focuses on comparing the effectiveness and safety of TNF inhibitors (TNFis) and apremilast in controlling oral ulcers of BD, aiming to provide evidence-based guidance for physicians in selecting appropriate treatment modalities.

Methods: A retrospective analysis was performed on BD patients treated between December 2016 and December 2021 with TNFis or apremilast for refractory oral ulcers.

Autoimmune hepatitis in genetic syndromes: A literature review.

Genetic syndromes represent relevant and rare diseases. These conditions include a large amount of epidemiological, pathogenetic and clinical features. However, a systematic approach to genetic syndromes is often prevented by the rareness of these diseases.