Factors Associated with the Development of Anti-drug Antibodies to TNFi and the Consequences for Axial Spondyloarthritis: A Two-year Follow-up Study.

Objective: To evaluate the development of anti-drug antibodies (ADAb) against tumor necrosis factor inhibitors (TNFi) therapy during a 2-year period and search the factors linked to patients with axial spondyloarthritis (axSpA).

Methods: Biologic-naive patients with axSpA were included in this observational study. Serum drug levels and ADAb were measured at weeks 12, 24, 52, and 104 of treatment by enzyme-linked immunosorbent assay (ELISA).

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.

Mimickers of nervous system involvement among patients with Behçet's syndrome.

Objectives: We aimed to identify conditions mimicking nervous system involvement among patients with Behçet's syndrome (BS) and to determine clinical, laboratory and imaging findings that may help in the differential diagnosis.

Methods: We screened the charts of 500 consecutive BS patients to identify those who were referred to neurology at any time during their follow-up. The final diagnoses, presenting signs and symptoms, laboratory and imaging results were retrieved from patient charts.

Development of Posterior Uveitis in Behçet Syndrome Patients with Vitreous Cells at Baseline.

Purpose: The prognostic importance of vitreous cells (VC) in patients with Behçet syndrome (BS) is unknown. We aimed to determine the frequency of developing posterior uveitis (PU) and any additional risk factors associated with the development of PU in BS patients with VC at diagnosis.

Methods: The charts of 572 consecutive BS patients who were registered between 2010 and 2012 were reviewed.

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.

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.

Impact of HLA-B51 on Uveitis and Retinal Vasculitis: Data from the AIDA International Network Registries on Ocular Inflammatory Disorders.

Purpose: The clinical relevance of human leukocyte antigen (HLA) subtypes such as HLA-B51 on Behçet's disease (BD)-related uveitis and non-infectious uveitis (NIU) unrelated to BD remains largely unknown.

Methods: Data were prospectively collected from the International AIDA Network Registry for BD and for NIU. We assessed differences between groups (NIU unrelated to BD and positive for HLA-B51, BD-related uveitis positive for HLA-B51 and BD-related uveitis negative for HLA-B51) in terms of long-term ocular complications, visual acuity (VA) measured by best corrected visual acuity (BCVA), anatomical pattern, occurrence of retinal vasculitis (RV) and macular edema over time.

Validation of the Behçet's Syndrome Overall Damage Index (BODI) for Retrospective Studies and a Proposal for Modification.

Objective: Assessment of damage accrual over time is important for evaluating and comparing long-term results of treatment modalities and strategies. Retrospective studies may be useful for assessing long-term damage, especially in rare diseases. We aimed to validate Behçet's Syndrome Overall Damage Index (BODI) for use in retrospective studies by evaluating its construct validity, reliability, and feasibility in retrospectively collected data.

Screening for pulmonary arterial hypertension in patients with systemic sclerosis in the era of new pulmonary arterial hypertension definitions.

Objectives: This study compares the performance of three composite pulmonary arterial hypertension (PAH) screening tools in a real-life SSc cohort, according to both the previous 2015 ESC/ERS guideline and the recent 2022 ESC/ERS guideline haemodynamic criteria.

Methods: Consecutive SSc patients without a previous diagnosis of pulmonary hypertension (PH) were screened for PAH using the European Society of Cardiology/European Respiratory Society (ESC/ERS), DETECT, and Australian Scleroderma Interest Group (ASIG) algorithms. Right heart catheterisation (RHC) referral performances for PAH were compared according to the 2022 ESC/ERS PAH criteria.

Treatment of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review and meta-analysis.

The evidence for the treatment of connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) mostly depends on subgroup or post hoc analysis of randomized controlled trials (RCTs). Thus, we performed a meta-analysis of RCTs that reported outcomes for CTD-PAH. PubMed and EMBASE were searched for CTD-PAH treatment.

Disease and Treatment-Specific Complications of Behçet Syndrome.

Purpose Of Review: We aimed to highlight disease-related and treatment-related complications of Behçet syndrome (BS) based on previous and recent studies and our own experience.

Recent Findings: The Behçet's Disease Overall Damage Index is a newly developed instrument to assess damage in BS. Validation studies showed that damage is already present in some patients at diagnosis and continues to progress during the follow-up, mainly related to treatment complications.

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.

Epidemiology of systemic vasculitis.

Purpose Of Review: Epidemiology of vasculitides exhibit geographic variation and data from some parts of the world are still scarce. Increased recognition of these rare diseases and improvement in diagnosis and patient care may lead to changes in their epidemiology. In this review, we aimed to highlight the most recent work on the epidemiology of systemic vasculitis.

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.

Heat Shock Proteins in Behçet Syndrome.

Behçet syndrome (BS) is a systemic vasculitis of unknown etiology that affects the skin, mucosa, joints, eyes, central nervous system, gastrointestinal system, arteries, and veins. It is generally believed to have a complex genetic background where both innate and adaptive immune systems are activated through environmental factors, such as infections, and auto-antigens. Heat shock proteins (HSPs) are highly conserved and immunogenic endogenous proteins that are thought to play both an enhancing and regulating role in several autoimmune and inflammatory diseases, such as rheumatoid arthritis, juvenile idiopathic arthritis, and Type I diabetes.

Treatment of systemic sclerosis-associated digital ulcers: recommendations of the Turkish Society for Rheumatology.

Objectives: Digital ulcers (DUs) are associated with a significant burden in systemic sclerosis (SSc) by leading to severe pain, physical disability, and reduced quality of life. This effort aimed to develop recommendations of the Turkish Society for Rheumatology (TRD) on the management of DUs associated with SSc.

Methods: In the first meeting held in December 2020 with the participation of a task force consisting of 23 rheumatologists the scope of the recommendations and research questions were determined.

A patient-driven registry on Behçet's disease: the AIDA for patients pilot project.

Introduction: This paper describes the creation and preliminary results of a patient-driven registry for the collection of patient-reported outcomes (PROs) and patient-reported experiences (PREs) in Behçet's disease (BD).

Methods: The project was coordinated by the University of Siena and the Italian patient advocacy organization SIMBA (Associazione Italiana Sindrome e Malattia di Behçet), in the context of the AIDA (AutoInflammatory Diseases Alliance) Network programme. Quality of life, fatigue, socioeconomic impact of the disease and therapeutic adherence were selected as core domains to include in the registry.

Infliximab for vascular involvement in Behçet's syndrome.

Objective: Vascular involvement is an important cause of morbidity and mortality in patients with Behçet's syndrome (BS). We aimed to survey the efficacy and safety of infliximab (IFX) in BS patients with vascular involvement followed in a dedicated tertiary center.

Methods: Charts of all BS patients who used IFX for vascular involvement between 2004 and 2022 were reviewed.

Behçet Syndrome.

Behçet's syndrome is a systemic vasculitis affecting arteries and veins of all sizes as well as recurrent oral, genital, and intestinal ulcers, skin lesions, predominantly posterior uveitis, and parenchymal brain lesions. These can be present in various combinations and sequences over time and diagnosis is made by recognizing the manifestations, as there are no diagnostic biomarkers or genetic tests. Treatment modalities include immunomodulatory agents, immunosuppressives and biologics, tailored according to prognostic factors, disease activity, severity, and patients' preferences.

Apremilast Long-Term Safety Up to 5 Years from 15 Pooled Randomized, Placebo-Controlled Studies of Psoriasis, Psoriatic Arthritis, and Behçet's Syndrome.

Background: Since US FDA approval in 2014, apremilast has consistently demonstrated a favorable benefit-risk profile in 706,585 patients (557,379 patient-years of exposure) worldwide across approved indications of plaque psoriasis, psoriatic arthritis, and Behçet's syndrome; however, long-term exposure across these indications has not been reported.

Objective: The aim of this study was to conduct a pooled analysis of apremilast data from 15 clinical studies with open-label extension phases, focusing on long-term safety.

Methods: We analyzed longer-term safety and tolerability of apremilast 30 mg twice daily across three indications for up to 5 years, focusing on adverse events of special interest, including thrombotic events, malignancies, major adverse cardiac events (MACE), serious infections, and depression.

Therapeutic approach to central nervous system involvement of Behçet's disease.

Background: Neurologic involvement in Behçet's disease (BD) represents a major cause of disease morbidity and mortality. Early recognition and timely treatment represent crucial aspects that aim at preventing long-term disability. The absence of robust and evidence-based studies further complicates the management of neuro-BD (NBD).

Outcome measures in Behçet syndrome.

Disease assessment has been challenging in Behçet syndrome due to the heterogeneous disease course and multiorgan involvement with variable treatment response. There have been several recent improvements regarding outcome measures including development of a Core Set of Domains for Behçet syndrome and novel instruments for assessing specific organs and overall damage. This review focuses on the current state of outcome measures in Behçet syndrome, unmet needs, and a research agenda towards the development of standardized and validated outcome measure instruments.