Systemic Sclerosis (SS) is a chronic idiopathic inflammatory disease whose main hint towards autoimmunity is given by the presence of high serum levels of autoantibodies in the majority of the patients. In indirect immunofluorescence in HEp-2 cells, there is predominant reactivity against the nucleolus and the nucleus. Some of the autoantibodies depict high specificity for SS, to the point of being considered as diagnostic markers for the disease. Examples are anti-Scl-70, antifibrillarin, and anti-RNA polymerase I antibodies. Some other autoantibodies present an interesting association with particular features of ES, such as anticentromere antibodies associated with the limited forms of disease, anti-RNA polymerase III antibodies associated with extensive skin involvement, and anti-To/Th antibodies associated with limited forms with propensity to the development of pulmonary hypertension. Some of these associations have been well established through several studies by distinct groups of investigators. Others, however, should be regarded with caution, since the exiguity of patients available for study may induce preliminary and inaccurate conclusions. The identification of anticentromere and anti-Scl-70 antibodies is available in most clinical laboratories with specific interest in autoimmunity. The same is not true for most of the autoantibody specificities associated with SS. The possible implementation of these rarer autoantibodies in routine laboratories might be decisive in providing more definitive evidence of their clinical associations.
Systemic Sclerosis (SE); autoantibody; scleroderma; antinuclear antibody
