Abstract

Behçet's disease is a chronic, recurrent, multisystemic, inflammatory disorder affecting mainly the oral and urogenital mucosa and the uveal tract. Although the etiology and pathogenesis of Behçet's disease are unknown, numerous etiologies have been proposed, including environmental, infectious, and immunological factors; an autoimmune basis, characterized by circulating immune complexes and complement activation, has gained increasing acceptance. To test and understand immunopathogenesis of Behçet's disease, animal models were developed based on enviromental pollutants, bacterial and human heat shock protein derived peptides, and virus injections. Using these animal models separately and/or concurrently allows for a more effective investigation into Behçet's disease. Animal models developed in the last 10 years aim at the development of efficient and safe treatment options.

Highlights

  • Behcet’s disease (BD) is a chronic, multisystemic, inflammatory disorder and is characterized by mucocutaneous, ocular, arthritic, vascular, gastrointestinal, and central nervous system involvement

  • Vasculitis was common in intestinal, oral, earlobular, and genital epithelial lesions. These findings were very similar to typical morphological changes in human BD [22]. These experiments proved that it was possible to induce BD-like symptoms such as ulceration and vascular inflammation in ICR mice by inoculating them with herpes simplex virus (HSV)

  • Many researchers have used this animal model to understand the etiopathogenesis of BD and efficiency and safety of newly developed drugs in BD

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Summary

Introduction

Behcet’s disease (BD) is a chronic, multisystemic, inflammatory disorder and is characterized by mucocutaneous, ocular, arthritic, vascular, gastrointestinal, and central nervous system involvement. The disease has a chronic course with periodic exacerbations and progressive deterioration [1]. Since the dermatologist Dr Hulusi Behcet [2] comprehensively described this disease involving multisystemic organs in 1937, the etiology of BD has still remained unclear. Various hypotheses have been proposed centering on viral infection, autoimmune disease, streptococcal-related antigens, specific alleles of the human major histocompatibility complex, genetic factors, and hazardous chemicals [3,4,5,6,7]. The history and recent developments in the immunopathogenesis of BD are reviewed and discussed in this paper

Short History
Animal Models Aiming to Elucidate the Immunopathogenesis of Behcet’s Disease
Findings
Animal Models Designed to Investigate the Efficiency of Newly Developed Drugs
Full Text
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