Haploinsufficiency of A20 (HA20) is a primary immune regulation disease caused by heterozygous loss-of-function variants in TNFAIP3, resulting in unchecked inflammatory signaling. HA20 is a highly heterogeneous disorder with overlapping features of autoinflammation, autoimmunity, immunodeficiency, a…
Mapping Neuro Behcet's Disease Across North Africa: a Systematic Review and Meta Analysis.
Data on neuro-Behçet's disease in North Africa remain limited and fragmented. We conducted a systematic review and meta-analysis to estimate the frequency of neurologic involvement in North African patients with Behçet's disease and to summarize the main clinical, cerebrospinal fluid, and…
Aging in Behçet's syndrome: an overlooked risk for late-life cognitive impairment.
Behçet's syndrome (BS) is a chronic multisystemic inflammatory disorder with a heterogeneous clinical course. A severe subset with central nervous system involvement (neuro-Behçet's syndrome, NBS) carries substantial morbidity. While disease activity often decreases with age, the long-term…
Adalimumab Reprograms M1 Macrophages to Attenuate Th1/Th17 Responses in Behçet's Uveitis and Vogt-Koyanagi-Harada Syndrome.
To investigate the effects and underlying mechanisms of adalimumab on monocyte-derived macrophage polarization and CD4+ T cell responses in patients with Behçet's uveitis (BU) or Vogt-Koyanagi-Harada (VKH) syndrome. Ex vivo analyses were performed on monocyte-derived macrophages and CD4+ T cell…
Behçet's disease presenting with intracardiac thrombus and pulmonary thromboembolism in a young Ethiopian male: a case report.
Behçet's disease (BD) is a rare, chronic, relapsing multisystem vasculitis with diverse manifestations, including mucocutaneous, ocular, neurological, and vascular involvement. Intracardiac thrombus is a rare manifestation of BD and can result in severe complications such as pulmonary thromboem…
Characterizing Manifestations of Optical Coherence Tomography in Pulmonary Arterial Lesions.
Effective imaging is lacking for intravascular lesions in pulmonary vascular diseases. Optical coherence tomography (OCT) offers micron-level resolution, enabling improved diagnosis and treatment guidance. This study aimed to evaluate pulmonary arterial lesions using OCT. This retrospective study re…
Clinical and Demographic Features of Behçet's Disease: A Retrospective Analysis of 4007 Patients from a Single Center in Turkey.
Behçet's disease is a multisystem inflammatory condition that can lead to severe vascular and neurological complications. To evaluate the demographic and clinical characteristics of a large single-centre Behçet's disease cohort and to investigate the associations of sex, age at onset, and…
Genetic Diagnosis and Identification of a Novel De Novo RELA Variant in Familial Behçet-like Autoinflammatory Syndrome Type 3: A Case Report.
To perform genetic diagnosis and pedigree analysis in a case of autosomal dominant Familial Behçet-like Autoinflammatory Syndrome type 3 (AIFBL3) caused by a novel RELA variant. Peripheral blood samples collected from the proband and parents underwent conventional genetic screening, next-genera…