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Sklerodermie-assoziierte Autoantikörper – klinische und diagnostische Relevanz

Scleroderma associated autoantibodies – clinical and diagnostic relevance

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Zusammenfassung

Autoantikörper sind bei der systemischen Sklerose (SSc) und ihren Varianten die bislang bestbekannte immunologische Veränderung. Mehr als 95% der Patienten mit SSc haben antinukleäre oder andere Autoantikörper im Serum. Bei etwa 90% der Patienten mit antinukleären Antikörpern (ANA) finden sich Sklerodermie-assoziierte Autoantikörper, die eine hohe Spezifität für die SSc aufweisen. Diese Antikörper schließen einander gegenseitig weitgehend aus und sind früh und persistierend im Krankheitsverlauf nachweisbar. Durch die Sklerodermie-assoziierten Autoantikörper charakterisierte SSc-Patienten bilden klinisch, genetisch und prognostisch relativ homogene Subgruppen der Erkrankung. Über diese diagnostisch relevanten Autoantikörper hinaus wurden zahlreiche weitere Autoantikörper bei der SSc beschrieben, die jedoch weder SSc-spezifisch noch einander ausschließend vorkommen und deren Antigene erst teilweise charakterisiert sind. Für einige dieser Autoantikörper wird aber verstärkt die noch nicht beantwortete Frage nach einer direkten Beteiligung an der Pathogenese der Erkrankung diskutiert.

Abstract

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.

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Authors and Affiliations

  1. Rheumaklinik und Rheumaforschungsinstitut Aachen

    E. Genth

  2. Labor an der Rheumaklinik Aachen, Burtscheider Markt 24, 52066, Aachen

    R. Mierau

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  1. R. Mierau
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  2. E. Genth
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Correspondence to R. Mierau.

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Mierau, R., Genth, E. Sklerodermie-assoziierte Autoantikörper – klinische und diagnostische Relevanz. Z. Rheumatol. 65, 279–284 (2006). https://doi.org/10.1007/s00393-006-0067-y

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