PubMed · 10691503
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T O Cheng. 2000-02-24. Behçet's disease.. https://pubmed.ncbi.nlm.nih.gov/10691503/
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PURPOSE OF REVIEW: In this review, we aimed to highlight recent findings from "-omics" studies in Behçet's disease. RECENT FINDINGS: Recent genomic studies in Behçet's disease identified possible risk loci associated with Behçet's disease related uveitis, neurologic involvement and gastrointestinal involvement. Additionally, sex-specific genetic effects were determined in Behçet's disease. Transcriptomic analyses of immune cells in Behçet's disease revealed that key inflammatory pathways such as NF-κB and MAPK have roles in Behçet's disease pathogenesis. Proteomic studies have highlighted the role of immune cell derived extracellular vesicles and identified potential biomarkers for vascular involvement and examined HLA I-bound immunopeptidomes. Metabolomics studies are still limited, but recent research has pointed to alterations in fatty acid metabolism and lipid profiles in Behçet's disease patient. SUMMARY: Omics studies have gained importance in the field of Behçet's disease through the generation of large data sets and efforts to extend their application are intensifying. These studies can provide opportunities for understanding Behçet's disease pathogenesis when they lead to testable hypotheses. Current challenges include the choice of appropriately homogeneous patient and control groups, effective data management and sharing, high cost and a rapidly increasing gap between the wealth of observational data generated and the relative paucity of controlled experimental efforts that could lead to mechanistic understanding.
Central nervous system involvement in Behçet disease (BD) may be either parenchymal or nonparenchymal. Abnormal cerebrospinal fluid findings and parenchymal involvement are associated with a poorer prognosis. A self-antigenic role for HLA B51 has been postulated in the pathogenesis of BD. The sibling occurrence rate is 3.6%. Familial aggregation may be more pronounced among pediatric cases compared with adult cases. The importance of inherited coagulation abnormalities in the pathogenesis of BD is not clear. Vasculitis of the vasa vasorum seems to be the major site of pathology in large vessel disease. Even among experts, no consensus exists regarding the optimal approach to treatment. Low-dose thalidomide is effective for the mucocutaneous lesions, but polyneuropathy complicates its prolonged use. Neurologic side effects of cyclosporine A should preclude its use in patients with central nervous system involvement except in unusual circumstances. The heightened inflammatory response of BD patients to simple trauma may lead to postoperative complications, but should not be regarded as a contraindication to surgery.
Although the classification of small-and medium-vessel vasculitides involving the skin remains complex and imperfect, a substantial body of knowledge related to cutaneous vasculitis exists. Some components of this knowledge, though beyond the usual purview of rheumatologists, are essential to understanding the vasculitides. The correct interpretation of cutaneous findings combined with a properly performed skin biopsy yields important insights not only into underlying diagnoses, but also into the pathophysiological mechanisms of individual cases. In this review, we discuss a classification scheme for the cutaneous vasculitides, outline the work-up for evaluating patients with these disorders, and describe the essential features of the major categories of skin vasculitis.