PubMed · 10691504
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J P Drenth, A Livneh. 2000-02-24. Behçet's disease.. https://pubmed.ncbi.nlm.nih.gov/10691504/
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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.
OBJECTIVE: To identify a subset of clinical features of Behçet's disease (BD) that can be summated to form an overall index of disease activity appropriate for clinical and research use internationally. METHODS: Completed Behçet's Disease Current Activity Forms were collected from a total of 524 patients with BD from five countries. The data from 14 questions on the form were subjected to Rasch analysis to establish whether these items form a hierarchical and unidimensional scale of disease activity, both within and between countries. RESULTS: The data showed a good fit to the Rasch model within three countries using a dichotomous scoring function. However, when the data from these three countries were pooled, the fit to the model was poor. Cross-cultural differential item functioning (DIF) was found in seven items in the pooled data. When the items with DIF by country were separated and two items were removed, the resulting 26-item scale showed a good fit to the Rasch model. CONCLUSIONS: Within Turkey, Korea and the UK, the 14 items can be summated to give an index of disease activity. Analysis of the pooled data confirmed that the index is not suitable for comparison between countries or for pooling of data in the raw form, but after fitting the data to the Rasch model such comparisons can be made. This gives a scaling tool that is quick and easy to use in the clinical situation.
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