title: CD4+CD25+ regulatory T cells in systemic sclerosis and other rheumatic diseases.
abstract: Systemic sclerosis (SSc) is a generalized connective tissue disorder, characterized by a wide spectrum of microvascular and immunological abnormalities, leading to a progressive thickening and fibrosis of the skin and other organs, such as the lungs, GI tract, heart and kidneys. SSc is thought to be an autoimmune disease owing to the presence of high affinity antibodies and possible clinical overlap with other autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis. Autoimmune diseases arise because of a breakdown in immunological self tolerance. Self tolerance is maintained via multiple regulatory mechanisms within the immune system, including the thymic deletion of self-reactive T cells and mechanisms of peripheral tolerance. In recent years, the presence of CD4(+)CD25(+)FOXP3(+) Tregs has been identified as a major mechanism of peripheral tolerance, and accumulating evidence indicates that alterations in Treg frequencies and/or function may contribute to autoimmune diseases. Here, we will review recent data on the percentage, function and phenotype of CD4(+)CD25(+) Tregs in rheumatic disease, and discuss how recent developments may guide research in this area in SSc.
authors.affiliation: Department of Rheumatology & Clinical Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands.
authors.forename: Jocea M R
authors.initials: JM
authors.lastname: Michels-van Amelsfort
authors.forename: Gina J
authors.initials: GJ
authors.lastname: Walter
authors.forename: Leonie S
authors.initials: LS
authors.lastname: Taams
mesh_terms: D000818:Animals; D001323:Autoantibodies / Q000276:immunology*; D015704:CD4 Antigens / Q000096:biosynthesis; D002450:Cell Communication; D005355:Fibrosis; D051858:Forkhead Transcription Factors / Q000276:immunology; D006801:Humans; D053645:Interleukin-2 Receptor alpha Subunit / Q000096:biosynthesis; D012216:Rheumatic Diseases / Q000276:immunology*; D012595:Scleroderma, Systemic / Q000276:immunology*; D017634:Self Tolerance; D012867:Skin / Q000276:immunology / Q000473:pathology; D050378:T-Lymphocytes, Regulatory / Q000276:immunology; D058504:Th17 Cells; D016212:Transforming Growth Factor beta / Q000276:immunology*
chemical_list: D001323:Autoantibodies; D015704:CD4 Antigens; C418974:FOXP3 protein, human; D051858:Forkhead Transcription Factors; D053645:Interleukin-2 Receptor alpha Subunit; D016212:Transforming Growth Factor beta