Behçet's syndrome

Emmi, Giacomo; Bettiol, Alessandra; Hatemi, Gülen; Prisco, Domenico

doi:10.1016/s0140-6736(23)02629-6

Cited by 8 publications

(2 citation statements)

References 129 publications

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“…Ongoing research is focused on elucidating triggers of immunopathology, identifying reliable biomarkers, and developing targeted immunomodulatory therapies [12]. Given the systemic nature of Behçet's disease, the treatment approach is tailored to the specific organs affected and guided by disease severity [106]. Mild presentations may be managed with topical measures and nonsteroidal anti-inflammatory drugs.…”

Section: Discussionmentioning

confidence: 99%

Behçet’s Disease, Pathogenesis, Clinical Features, and Treatment Approaches: A Comprehensive Review

Lavalle,

Caruso,

Foti

et al. 2024

Medicina

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Behçet’s disease is a systemic inflammatory disorder of unknown etiology. The disease manifests with diverse clinical symptoms, most commonly recurrent oral and genital ulcers, skin lesions, and uveitis, though it can affect multiple organ systems. Diagnosis is primarily clinical due to the lack of a definitive diagnostic test, and management involves a multidisciplinary approach to control inflammation and manage symptoms. Current treatment strategies involve corticosteroids, immunosuppressive agents, and, increasingly, biological therapies. Behçet’s disease exhibits a higher prevalence along the Silk Road, suggesting a role of environmental and genetic factors. Despite significant progress in understanding its clinical characteristics and treatment approaches, gaps remain in our understanding of its pathogenesis. Future research is needed to elucidate the disease’s pathophysiology and optimize treatment strategies.

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Section: Discussionmentioning

confidence: 99%

Behçet’s Disease, Pathogenesis, Clinical Features, and Treatment Approaches: A Comprehensive Review

Lavalle,

Caruso,

Foti

et al. 2024

Medicina

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“…Autoimmune uveitis (AU) is an ocular autoimmune-inflammatory disease that imposes a substantial risk on visual acuity [ 1 ]. Patients suffering from AU encounter significant physical and economic burdens due to the recurrence and prolonged duration of the disease [ 2 , 3 ]. The primary therapeutic approach for AU involves corticosteroids and immunosuppressants; nevertheless, prolonged use of these medicines can lead to significant adverse effects both locally and systemically [ 4 , 5 ].…”

Section: Introductionmentioning

confidence: 99%

Beneficial mechanisms of dimethyl fumarate in autoimmune uveitis: insights from single-cell RNA sequencing

Zhu,

Li,

Peng

et al. 2024

J Neuroinflammation

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Background Dimethyl fumarate (DMF) is a fumaric acid ester that exhibits immunoregulatory and anti-inflammatory properties. However, the function of DMF in autoimmune uveitis (AU) is incompletely understood, and studies comprehensively exploring the impact of DMF on immune cells are still lacking. Methods To explore the function of DMF in uveitis and its underlying mechanisms, we conducted single-cell RNA sequencing (scRNA-seq) on the cervical draining lymph node (CDLN) cells of normal, experimental autoimmune uveitis (EAU), and DMF-treated EAU mice. Additionally, we integrated scRNA-seq data of the retina and CDLNs to identify the potential impact of DMF on ocular immune cell infiltration. Flow cytometry was conducted to verify the potential target molecules of DMF. Results Our study showed that DMF treatment effectively ameliorated EAU symptoms. The proportional and transcriptional alterations in each immune cell type during EAU were reversed by DMF treatment. Bioinformatics analysis in our study indicated that the enhanced expression of Pim1 and Cxcr4 in EAU was reversed by DMF treatment. Further experiments demonstrated that DMF restored the balance between effector T (Teff) /regulatory T (Treg) cells through inhibiting the pathway of PIM1-protein kinase B (AKT)-Forkhead box O1 (FOXO1). By incorporating the scRNA-seq data of the retina from EAU mice into analysis, our study identified that T cells highly expressing Pim1 and Cxcr4 were enriched in the retina. DMF repressed the ocular infiltration of Teff cells, and this effect might depend on its inhibition of PIM1 and CXCR4 expression. Additionally, our study indicated that DMF might reduce the proportion of plasma cells by inhibiting PIM1 expression in B cells. Conclusions DMF effectively attenuated EAU symptoms. During EAU, DMF reversed the Teff/Treg cell imbalance and suppressed the ocular infiltration of Teff cells by inhibiting PIM1 and CXCR4 expression. Thus, DMF may act as a new drug option for the treatment of AU.

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Clinical phenotypes of adult-onset Behçet’s syndrome: a comprehensive cross-sectional study in China

She,

Hu,

Zou

et al. 2024

Clin Rheumatol

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Behçet's syndrome

Cited by 8 publications

References 129 publications

Behçet’s Disease, Pathogenesis, Clinical Features, and Treatment Approaches: A Comprehensive Review

Behçet’s Disease, Pathogenesis, Clinical Features, and Treatment Approaches: A Comprehensive Review

Beneficial mechanisms of dimethyl fumarate in autoimmune uveitis: insights from single-cell RNA sequencing

Clinical phenotypes of adult-onset Behçet’s syndrome: a comprehensive cross-sectional study in China

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