title: The clinical relevance of autoantibodies in scleroderma.
abstract: Scleroderma (systemic sclerosis) is associated with several autoantibodies, each of which is useful in the diagnosis of affected patients and in determining their prognosis. Anti-centromere antibodies (ACA) and anti-Scl-70 antibodies are very useful in distinguishing patients with systemic sclerosis (SSc) from healthy controls, from patients with other connective tissue disease, and from unaffected family members. Whereas ACA often predict a limited skin involvement and the absence of pulmonary involvement, the presence of anti-Scl-70 antibodies increases the risk for diffuse skin involvement and scleroderma lung disease. Anti-fibrillarin autoantibodies (which share significant serologic overlap with anti-U3-ribonucleoprotein antibodies) and anti-RNA-polymerase autoantibodies occur less frequently and are also predictive of diffuse skin involvement and systemic disease. Anti-Th/To and PM-Scl, in contrast, are associated with limited skin disease, but anti-Th/To might be a marker for the development of pulmonary hypertension. Other autoantibodies against extractable nuclear antigens have less specificity for SSc, including anti-Ro, which is a risk factor for sicca symptoms in patients with SSc, and anti-U1-ribonucleoprotein, which in high titer is seen in patients with SSc/systemic lupus erythematosus/polymyositis overlap syndromes. Limited reports of other autoantibodies (anti-Ku, antiphospholipid) have not established them as being clinically useful in following patients with SSc.
authors.affiliation: Division of Rheumatology and Clinical Immunogenetics and General Medicine, The University of Texas-Houston Health Science Center (UTH-HSC), Houston, Texas, USA. john.d.reveille@uth.tmc.edu
authors.forename: Khanh T
authors.initials: KT
authors.lastname: Ho
authors.forename: John D
authors.initials: JD
authors.lastname: Reveille
mesh_terms: D000974:Antibodies, Antinuclear / Q000097:blood*; D002466:Cell Nucleolus / Q000276:immunology; D002503:Centromere / Q000276:immunology; D004264:DNA Topoisomerases, Type I / Q000276:immunology; D006801:Humans; D012595:Scleroderma, Systemic / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology
chemical_list: D000974:Antibodies, Antinuclear; D004264:DNA Topoisomerases, Type I