HLA class II restriction of autoreactive T cell responses in pemphigus vulgaris: review of the literature and potential applications for the development of a specific immunotherapy.
Pemphigus vulgaris (PV) is a life-threatening autoimmune bullous disease of the skin and mucous membranes which requires immunosuppressive therapy, most commonly a combination of glucocorticoids and additional immunosuppressive agents. Since the side effects of long-term immunosuppressive therapy contribute to the poor prognosis of this disorder, there is considerable interest in a more specific treatment of this severe skin disease. PV may serve as a model disease for the development of a specific immunotherapy, because its pathogenesis as well as involved immunogenetic factors are well-characterized. This review focuses on the characterization of autoreactive T cell responses to desmoglein 3 (Dsg3), the autoantigen of PV, that presumably regulate the production of autoantibodies by providing help to the autoreactive B cells. Current knowledge on T cell epitopes of Dsg3 and the HLA class II alleles that restrict Dsg3-specific autoreactive T cell responses, as well as potential applications for a specific immunotherapy of PV, are described.
Riechers
R
R
Department of Dermatology, RWTH Aachen, Germany.
Grötzinger
J
J
Hertl
M
M
D000595:Amino Acid Sequence; D001324:Autoantigens / Q000737:chemistry / Q000276:immunology*; D015551:Autoimmunity; D015820:Cadherins / Q000737:chemistry / Q000276:immunology*; D051184:Desmoglein 3; D000949:Histocompatibility Antigens Class II / Q000378:metabolism*; D006801:Humans; D007167:Immunotherapy; D008969:Molecular Sequence Data; D010392:Pemphigus / Q000276:immunology* / Q000628:therapy; D013601:T-Lymphocytes / Q000276:immunology*
D001324:Autoantigens; D015820:Cadherins; C071894:DSG3 protein, human; D051184:Desmoglein 3; D000949:Histocompatibility Antigens Class II