title: The role of superantigens in vasculitis.
abstract: Multiple risk factors are involved in susceptibility to vasculitis. Inherited determinants may increase the risk but are insufficient to induce the disease. Environmental factors, such as infections, are important modulators and probably trigger the disease in most cases. One of the possible triggers may be a bacterial superantigen (SAg). SAgs may activate autoreactive T cells that mediate autoimmune vessel wall destruction. Furthermore, SAgs may activate autoreactive B cells to produce autoantibodies that are involved in the pathophysiology of vasculitis, such as antineutrophil cytoplasmic autoantibodies or anti-endothelial cell antibodies. In patients with Kawasaki disease, Wegener's granulomatosis, and infection-related forms of vasculitis, SAg-producing microorganisms have regularly been found. Activation of circulating T cells and skewing of the T-cell repertoire have been reported in most forms of vasculitis. In the past year, for the first time, patients were described in which T-cell receptor V beta expansions were documented simultaneously with the typing of the microbial SAgs, providing evidence that the observed changes in the T-cell repertoire could be caused by these bacterial SAgs. In the future, elucidation of the immunologic mechanisms by which SAgs may play a role in the pathophysiology of vasculitis will provide more effective methods for the treatment of vasculitis.
authors.affiliation: Department of Clinical Immunology, University Hospital Groningen, The Netherlands.
authors.forename: J W
authors.initials: JW
authors.lastname: Tervaert
authors.forename: E R
authors.initials: ER
authors.lastname: Popa
authors.forename: N A
authors.initials: NA
authors.lastname: Bos
mesh_terms: D000942:Antigens, Bacterial / Q000276:immunology*; D006801:Humans; D013211:Staphylococcus aureus / Q000276:immunology; D013291:Streptococcus / Q000276:immunology; D018089:Superantigens / Q000502:physiology*; D014657:Vasculitis / Q000276:immunology*
chemical_list: D000942:Antigens, Bacterial; D018089:Superantigens