Autoantibodies in the pathogenesis of mixed connective tissue disease.
Antibodies to U1-RNP are part of the clinical definition of mixed connective tissue disease (MCTD). These antibodies and other well-defined antibodies tend to arise together in affected patients. Although still speculative, hypotheses that link U1-RNP antibodies to the development of autoimmunity in MCTD and that associate U1-RNP antibodies with mechanisms of tissue injury in MCTD have emerged and are being tested. Salient features of these hypotheses include: (1) an antigen-driven response that is due to impaired clearance of potentially immunogenic self-antigens, (2) inadequate B- and T-cell tolerance to RNP autoantigens, and (3) immunogenic properties of the RNA component of targeted ribonucleoproteins. Further studies are needed to establish whether anti-RNP antibodies have prognostic importance that is relevant to practicing clinicians.
Greidinger
Eric L
EL
Division of Rheumatology and Immunology, University of Miami, Miami, FL 33136, USA.
Hoffman
Robert W
RW
D001323:Autoantibodies / Q000502:physiology*; D001402:B-Lymphocytes / Q000502:physiology; D006801:Humans; D008947:Mixed Connective Tissue Disease / Q000175:diagnosis / Q000276:immunology*; D011379:Prognosis; D017412:Ribonucleoprotein, U1 Small Nuclear / Q000276:immunology*; D013601:T-Lymphocytes / Q000502:physiology; D013997:Time Factors
D001323:Autoantibodies; D017412:Ribonucleoprotein, U1 Small Nuclear