[Scleroderma associated autoantibodies - clinical and diagnostic relevance]

In systemic sclerosis (SSc) and its variants, autoantibodies are the best known immunological aberration. In more than 95% of the patients, antinuclear antibodies or other autoantibodies can be detected. In about 90% of SSc patients with antinuclear antibodies, scleroderma associated autoantibodies highly specific for systemic sclerosis are found. These autoantibodies usually exclude each other in individual patients, and they are detectable early, persisting during the course of the disease. SSc patients characterized by scleroderma associated autoantibodies belong to disease subsets which are relatively homogeneous in clinical, genetic and prognostic terms. Besides these diagnostically relevant autoantibodies, numerous additional ones have also been described. These are neither SSc specific nor mutually exclusive, and their antigens have only been partially characterized. Some, however, are thought to be relevant to the as yet unanswered question of whether autoantibodies are directly involved in SSc pathogenesis.