Behçet's is a rare autoimmune vasculitis causing recurring oral ulcers, genital ulcers, and uveitis.
✦ AI summary
Behçet's disease (BD) is described in these sources as a chronic, multisystem inflammatory disorder (also called Behçet's syndrome) that can cause recurrent mouth and genital ulcers, skin lesions, eye inflammation, gut problems, and, less commonly, airway or vascular complications [8][11][9]. It seems to affect immune signaling pathways such as Th17/IL-17, JAK-STAT, and interferon pathways, and involves biological markers like anti-HSP27 antibodies (found in 57% of BD patients in one review) and HLA-B51 (positive in about half of pediatric cases) [1][10][11]. In children, mucocutaneous symptoms are almost universal and often the first sign, while other organ involvement (joints, eyes, gut, nervous system, blood vessels) varies widely between patients [11][12]. Research on treatment includes biologic and targeted therapies: adalimumab showed strong improvement in eye inflammation and reduced steroid needs in refractory Behçet's uveitis [8], ustekinumab showed good response rates for intestinal BD [5], and a review of JAK inhibitors (like upadacitinib) found promising results, especially for gut involv
This is an AI-generated summary of the sources below — not medical advice. Always talk to a doctor or pharmacist about your own situation.
HSP27 in autoimmune diseases: Context-dependent regulation and clinical potential.
Heat shock protein 27 (HSP27) is a crucial member of the small heat shock protein family with a molecular weight of approximately 27 kDa, playing key roles in cellular stress response, protein homeostasis, and cell survival regulation. Recent studies have revealed that HSP27 plays a complex dual rol…
Type III interferons attenuates Th1/Th17 cell pathogenicity and regulates retinal pigment epithelium cells via NLRP1/NLRP3 signaling axis in autoimmune uveitis.
Accumulating data implicate Type III interferons (IFN-λs) in autoimmune disorders, prompting our exploration of their role in uveitis pathogenesis. Serum and peripheral blood mononuclear cells (PBMCs) from patients with active Vogt-Koyanagi-Harada (VKH) and active Behçet's disease (BD) were analyzed…
Precision management of autoimmune ocular complications: Th17 mechanisms and therapeutic innovations.
Precision management of ocular complications in systemic autoimmune diseases, such as Sjögren's syndrome (SS), systemic lupus erythematosus (SLE), Behçet's disease, and thyroid-associated ophthalmopathy (TAO), requires integration of immune mechanisms with clinical translation. These disorders manif…
Reduction of monocyte activation and oxidative stress by phosphodiesterase 4 blockade in Behçet's syndrome.
Behçet's syndrome (BS) is a systemic vasculitis characterized by multisystemic inflammatory manifestations driven by innate and adaptive immunity. Apremilast, an oral small-molecule selective inhibitor of phosphodiesterase 4 (PDE4), has shown effectiveness in treating oral ulcers of BS in both rando…
Single-cell analysis identifies monocyte signatures of disease activity and clinical subtypes in Behçet's disease.
Behçet's disease (BD) is a multisystem inflammatory disorder with diverse phenotypes and incompletely defined immune mechanisms. This study aimed to map immune dysregulation in BD at high resolution, comparing active vs remission states and identifying pathways linked to clinical phenotypes. We perf…
Assessment of long-term organ damage and predictors of damage accrual in Behçet's syndrome: a 10-year longitudinal study using the Behçet's Syndrome Overall Damage Index.
To assess long-term organ damage and its accrual in a real-life cohort of Behçet's syndrome (BS) patients using the Behçet's Syndrome Overall Damage Index (BODI), identifying predictors of damage at diagnosis and factors associated with its accrual during follow-up. This single-centre retrospective…
Massive haemoptysis secondary to pulmonary artery aneurysms in Hughes-Stovin syndrome: a variant of Behçet's disease managed with immunosuppression and endovascular embolisation.
We report a rare and potentially life-threatening case of a young male with Behçet's disease complicated by Hughes-Stovin syndrome who presented with massive haemoptysis secondary to multiple pulmonary artery aneurysms with associated thrombosis. The patient presented to the emergency department wit…
Unraveling the psychological burden of Behçet disease: the impact of anxiety and depression on health-related quality of life outcomes.
Behçet's disease (BD) is a chronic, inflammatory multisystem disease that has impact both physical health and mental well-being. However, the psychological impact of BD remains underexplored, particularly in Arabic-speaking populations. The aim of this study was to assess the prevalence of anxiety a…