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Behçet's Disease: A Comprehensive Overview of Symptoms, Pathology, Genetics, and Treatment
Arbnora Batalli1, Thomas Liehr2, Gazmend Temaj3
1Paediatric Department, University Clinical Center of Kosovo, Prishtina, Kosovo.
Insights
Behçet's disease (BD) is a chronic inflammatory disorder causing ulcers and systemic issues. Research explores its genetic links, pathology, and new treatments like biologics and microRNAs for better patient outcomes.
Area of Science:
- Rheumatology
- Immunology
- Genetics
Background:
- Behçet's disease (BD) is a multisystemic inflammatory disorder characterized by recurrent oral/genital ulcers, skin lesions, and vasculitis.
- It predominantly affects populations along the Mediterranean, Middle Eastern, and East Asian regions.
- Pathology involves neutrophil infiltration and endothelial damage, leading to complications like thrombosis and aneurysms.
Purpose of the Study:
- To review the current understanding of Behçet's disease (BD).
- To examine symptoms, pathology, genetic factors, and treatment strategies for BD.
- To highlight emerging research, including the role of microRNAs in BD.
Main Methods:
- Literature review of existing studies on Behçet's disease.
- Analysis of pathological findings, genetic predispositions (HLA-B*51), and environmental factors.
- Evaluation of current and novel therapeutic approaches, including biologics and microRNAs.
Main Results:
- BD exhibits significant genetic influence, particularly the HLA-B*51 allele.
- Treatment involves corticosteroids, TNFα inhibitors, and biologics to manage inflammation.
- MicroRNAs show potential as diagnostic biomarkers and therapeutic targets in BD.
Conclusions:
- Behçet's disease requires comprehensive management due to its complex multisystemic nature.
- Optimizing treatment strategies and understanding genetic/environmental factors are crucial for improving outcomes.
- Further research into microRNAs offers promising avenues for BD diagnosis and therapy.
Abstract:
Behçet's disease (BD, also called Behçet's syndrome) is a complex, chronic, and multisystemic disorder characterised by recurrent oral and genital ulcers, skin lesions, and various other systemic manifestations due to underlying vasculitis. This review examines the symptoms, pathology, genetic factors, and treatment approaches associated with BD. The syndrome mainly affects populations in the Mediterranean region, the Middle East, and East Asia, with varying prevalence rates in different countries. Pathologically, BD is characterized by neutrophil infiltration and endothelial cell damage, which can lead to complications such as thrombosis and aneurysms. Genetic predisposition plays an important role - particularly via the HLA-B*51 allele - while additional non-HLA-related genetic and environmental influences further increase susceptibility to the disease. Treatment strategies have focussed on reducing inflammation and managing symptoms through a range of medications, including corticosteroids, TNFα inhibitors and emerging biologics. Recent research has highlighted the potential of microRNAs in regulating inflammatory pathways, as well as their role as biomarkers for diagnosis and treatment. Ongoing studies aim to optimize therapeutic approaches and improve treatment outcomes for this challenging disease.
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