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.

Turkish Society for Rheumatology (Turkish Takayasu Arteritis Study Group) recommendations for the diagnosis, follow-up and the treatment of Takayasu's arteritis.

Objectives: To develop evidence-based and expert opinion guided recommendations for Takayasu's arteritis (TAK) management.

Methods: A systematic literature review was conducted following the principles outlined in the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. To structure the key clinical questions, the task force employed the Population, Intervention, Comparison, and Outcome (PICO) format.

Central nervous system involvement and mimickers in ANCA associated vasculitis.

Objective: Central nervous system (CNS) involvement is rare in ANCA-associated vasculitis (AAV). On the other hand, AAV patients may develop complications or other conditions that mimic CNS involvement. We aimed to present the clinical, laboratory and imaging features of our AAV patients with CNS involvement and conditions other than CNS involvement that caused neurologic symptoms.

Arterial and venous thrombosis in systemic and monogenic vasculitis.

Systemic vasculitis, common forms of which include anti-neutrophil cytoplasmic antibody-associated small-vessel vasculitis, large-vessel vasculitis and Behçet syndrome, are frequently complicated by arterial or venous thrombotic events (AVTEs). Newly identified entities such as DADA2 (deficiency of adenosine deaminase 2) and VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome, which are driven by genetic mutations, also exhibit vasculitic features and are associated with a high risk of AVTEs. AVTEs in systemic vasculitis, including monogenic forms of vasculitis, are due to the complex interaction of inflammation and coagulation.

Venous ulcers in Behçet syndrome.

Introduction: Post-thrombotic syndrome (PTS) and stasis ulcers are late complications of deep vein thrombosis (DVT) in Behçet's syndrome (BS). We aimed to determine the clinical and histopathological characteristics, treatment modalities, and outcomes in BS patients with stasis ulcers.

Method: We included 63 BS patients with stasis ulcers from a total of 310 with vascular involvement, seen at a multidisciplinary center between January 2021 and July 2022.

Behçet's syndrome: one year in review 2024.

The aim of this review is to provide a critical summary of studies published during 2023 that contribute to our understanding of Behçet's syndrome. An increase in the incidence of BS was reported in Northern Spain after 2014.Studies on patient perspectives showed the impact of Behçet's syndrome on quality of life, daily activities, education, work, and relationships.

Sexual dysfunction among female patients with rheumatic diseases.

To demonstrate the burden of sexual dysfunction (SD) among females with rheumatic diseases, we conducted a cross-sectional comparative study in patients with systemic sclerosis (SSc), systemic lupus erythematosus (SLE), and Behçet's syndrome (BS) along with suitable healthy controls (HCs). Age-matched female patients with SSc (n = 50), SLE (n = 49), and BS (n = 54), along with 52 female HCs were included in this study between April and October, 2021. Sociodemographic features were recorded, and psychometric tests, i.

Targeting complement dysregulation: eculizumab in scleroderma renal crisis management-a case-based review.

Systemic sclerosis (SSc) poses significant challenges in clinical management, especially when complicated by scleroderma renal crisis (SRC), a rare but life-threatening manifestation. Here, we report a 41-year-old female patient with SSc who presented with SRC and concurrent thrombotic microangiopathy. Her condition persisted despite conventional treatments such as plasma exchange and renin-angiotensin-aldosterone system blockade.

Atrial myxoma as a mimicker of intracardiac thrombus in Behçet's syndrome: a case study with histopathological confirmation.

Cardiomyopathies cause most intracardiac thrombosis (ICT), and Behçet's syndrome (BS) is a rare inflammatory disease that can be responsible for a proportion of ICT. Other inflammatory disorders involved in the aetiology of ICT include antiphospholipid syndrome, Henoch-Schonlein purpura, COVID-19, and Loeffler endocarditis. ICT usually occur during the active phase of BS, and they have a close relationship with vascular involvement.

Brain white matter microstructural alterations in Behcet's syndrome correlate with cognitive impairment and disease severity: A diffusion tensor imaging study.

Objectives: To evaluate the microstructural integrity of brain white matter tracts in patients with Neuro-Behcet's syndrome (NBS) and Behcet's syndrome (BS) without neurological manifestations using diffusion tensor imaging (DTI) and to investigate potential utility of DTI as a surrogate biomarker of neurocognitive functioning and disease severity.

Methods: This cross-sectional study comprised 34 NBS patients and 32 BS patients without neurological involvement, identified based on the International Study Group of the Behcet's disease (ISGBD) and the International Consensus Recommendation (ICR) criteria, as well as 33 healthy controls. Cognitive functions, including attention, memory, language, abstraction, executive control, visuospatial skills, and sensorimotor performance were assessed using standardized questionnaires.

Tocilizumab may not be a good option for vascular involvement due to Behçet's syndrome.

Objectives: Tocilizumab has been increasingly reported as an alternative therapeutic agent in the management of Behçet's syndrome (BS) and it has been mostly tried in BS patients with neurological and eye involvement. As therapeutic responses to each drug may vary across different types of BS involvement, we aimed to report seven patients with large vessel involvement treated with tocilizumab.

Methods: We enrolled seven BS patients with vascular involvement who were given tocilizumab at the Behçet's Disease Research Centre in Istanbul University-Cerrahpaşa between 2000 and 2022.

Carotid artery ultrasonography and shear wave elastography in Takayasu's arteritis: a comparative analysis with diabetes mellitus.

Objectives: Takayasu's arteritis (TAK) is a chronic inflammatory large vessel vasculitis with a grim prognosis due to the excessive risk for cardiovascular (CV) diseases. Its diagnosis relies on radiographic imaging and its differentiation particularly from atherosclerosis could be challenging. Hypothesising that vascular morphology observed in TAK would be comparable to that found in type 2 diabetes mellitus (T2DM), a prototype for advanced atherosclerosis, we compared two disease groups using carotid artery B mode US and shear wave elastography (SWE).

Role of Optical Coherence Tomography in Vasculitis-Associated Pulmonary Hypertension and Chronic Thromboembolic Pulmonary Hypertension.

Background: Identifying and understanding the microstructural changes within the wall of the pulmonary artery (PA) is crucial for elucidating disease mechanisms and guiding treatment strategies. We assessed the utility of optical coherence tomography (OCT) in identifying such changes within segmental/subsegmental PAs and compared the morphological variations in WHO group 4 pulmonary hypertension associated with Behcet Disease (BD), Takayasu arteritis (TA) and chronic thromboembolic pulmonary hypertension (CTEPH). Idiopathic pulmonary arterial hypertension (IPAH) patients served as controls.

A Proposal of a New Tool for the Assessment of Damage in Behçet Syndrome Uveitis: Cerrahpasa Ocular Damage Grading System.

Introduction/objective: There is currently no tool available to assess the severity of damage in uveitis due to Behçet's syndrome (BS). In this preliminary study, we developed a new grading system to evaluate ocular damage and assessed it in a prospective cohort.

Methods: A specialist in BS uveitis (YO) developed a grading system for ocular damage with five grades based on the extent of damage in the posterior segment.

Folliculitis might be associated with pathergy-positivity in patients with Behçet syndrome.

Pathergy test indicates nonspecific hyper-reactivity of the skin to aseptic trauma in Behçet syndrome (BS) and is considered as an adjunctive diagnostic test with a good specificity albeit with low sensitivity. We tested the hypothesis that a relationship exists between active clinical manifestations of BS and the pathergy-positivity when performed simultaneously. Pathergy test and detailed dermatologic examination were done in 105 BS patients (60M/45F); who were seen consecutively at the multi-disciplinary BS outpatient clinic in a single tertiary center.

Hearing loss in Takayasu's arteritis.

Objectives: Neurosensory hearing loss is well-documented in chronic autoimmune conditions such as systemic lupus erythematosus (SLE). However, the literature lacks data on the prevalence and characteristics of hearing impairment in Takayasu's arteritis (TAK). In this cross-sectional study, our principal objective was to systematically assess the auditory function of individuals diagnosed with TAK, against SLE patients and healthy controls (HC).

HLA-B gene methylation and expression in Behçet's syndrome: a potential role of epigenetics in the pathogenesis.

Objectives: The HLA-B51 locus has the strongest association with Behçet's syndrome (BS). The presence of a CpG island in the HLA-B gene led us to examine the role of epigenetic regulation in BS.

Methods: HLA-B51 genotyping was performed via sequence-specific PCR in 15 index familial BS cases, 17 affected relatives, 26 unaffected relatives, 46 sporadic BS cases, and 41 healthy controls.

Pulmonary artery involvement due to Behçet's syndrome and Hughes Stovin syndrome: a comparative study.

Objectives: Hughes-Stovin syndrome (HSS) is a rare inflammatory condition defined as pulmonary artery aneurysms (PAA) associated with deep vein thrombosis. It is similar to vascular involvement of Behçet's syndrome (BS), but differs in the absence of typical skin-mucosal findings. Whether HSS is a distinct entity or a form fruste of BS is debated.

Behçet's syndrome: one year in review 2023.

This critical review of studies on Behçet's syndrome published during 2022 includes studies on epidemiology, patients' perspective, pathogenesis, diagnosis, clinical features and management. Studies on pathogenesis included potential biomarkers mostly related to macrophages, neutrophil and cytokine balance, new GWAS and polymorphism studies, and studies on miRNAs and long non-coding RNAs. Clinical studies showed that application of pneumococcal vaccine to the prick site increased the sensitivity and specificity of the pathergy test and the prevalence of AA amyloidosis had decreased over the years.

Mycophenolate mofetil may be an alternative for maintenance therapy of Behçet syndrome uveitis: a single-center retrospective analysis.

Experience with mycophenolate in uveitis due to Behçet syndrome (BS) is limited. Twelve patients with panuveitis or posterior uveitis who were started mycophenolate were included. Data on demographic characteristics, therapies, ocular attacks, and adverse events were extracted from patient charts.

Behçet disease, familial Mediterranean fever and MEFV variations: More than just an association.

Behçet disease (BD) and familial Mediterranean fever (FMF) are two inflammatory disorders that share many features including historical background, ethnical distribution and inflammatory characteristics. Several studies suggested that BD and FMF might occur in the same individual more commonly than expected. Additionally, the pathogenic MEFV gene variants, especially p.

Increased arterial stiffness and accelerated atherosclerosis in Takayasu arteritis.

Introduction: Cardiovascular diseases are the leading causes of morbidity and mortality in patients with Takayasu arteritis (TAK). Arterial stiffness and accelerated atherosclerosis have been reported in TAK, however, morphological changes in the arterial wall have not been adequately addressed. Shear wave elastography (SWE) is a new, non-invasive, direct and quantitative method of ultrasonography (US) that evaluates elasticity of biological tissues.