[Autoantibodies in systemic sclerosis: clinical interest and diagnosis approach].

PURPOSE: This review describes different autoantibodies that are associated to systemic sclerosis disease, in presenting their interest for the diagnosis and prognosis, and suggests an immunologic diagnosis approach. The systemic sclerosis (SSc) is characterized by variant specific autoantibodies (autoAbs). More than 90% of SSc cases have antinuclear antibodies (ANA). Anti-centromere and anti-Th/To antibodies are often associated to the limited SSc and to the CREST syndrome. The anti-topo-isomerase I, anti-RNA polymerases and anti-fibrillarin/U3-RNP Abs are diffuse SSc markers with several organ involvements. The anti-PM/Scl and anti-U1-RNP Abs rather mark overlap shapes with polymyositis and systemic lupus erythematous, respectively. The anti-Ku, anti-B23 and anti-NOR90 Abs are a new generation of less frequent autoAbs that show a relationship with specific subsets of SSc. Another heterogeneous group of Abs, topic of research, is described in SSc as well as anti-fibrillin 1, anti-endothelial cells, anti-annexin V and anti-collagen Abs. Despite the diagnosis of scleroderma is mainly clinical, these different autoAbs constitute a diagnosis and prognosis tools by defining immuno-clinical substes of the disease. Identifying those autoAbs requires a diagnostic strategy with two steps: the indirect immunofluorescence remains the better means of ANA tracking, leading thereafter to other identification specific methods, such immunoprecipitation, ELISA or immunoblotting.