The clinical relevance of autoantibodies in scleroderma

被引:242
作者
Ho, KT [1 ]
Reveille, JD [1 ]
机构
[1] Univ Texas, Hlth Sci Ctr, Div Rheumatol & Clin Immunogenet & Gen Med, Houston, TX 77030 USA
关键词
anti-centromere; anti-Scl-70; autoantibodies; scleroderma; systemic sclerosis;
D O I
10.1186/ar628
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:80 / 93
页数:14
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