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Systemische Sklerose – Diagnose und Klassifikation

Systemic sclerosis – diagnosis and classification

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Zusammenfasssung

Die systemische Sklerose (SSc) ist eine klinisch vielgestaltige und heterogene Systemkrankheit mit Entzündung, vermehrter Bindegewebsbildung und Schäden an den Blutgefäßen. Die frühe Diagnose und Klassifikation kann vor allem bei oligosymptomatischer (undifferenzierter) Krankheitsausprägung mit Raynaud-Phänomen oder gering ausgeprägter Sklerodermie schwierig sein. Sklerodermie-spezifische antinukleäre Antikörper, die bei etwa 90% der Patienten mit systemischer Sklerose früh und persistierend vorkommen, sind von wesentlicher taxonomischer Bedeutung. Die Kapillarmikroskopie der Nagelfalz ist bei frühen Verläufen sensitiv und prädiktiv für die SSc. Es wird ein Algorithmus zu Diagnose und Klassifikation der SSc dargestellt, der klinische, kapillarmikroskopische und serologische Kriterien verwendet und die verschiedenen Misch- und Sonderformen berücksichtigt. Die 6. OMERACT-Konferenz hat verschiedene Outcome-Maße für klinische Studien zusammengestellt. Bisher gibt es noch keine für die tägliche klinische Praxis geeignete Statusindices für die Krankheitsaktivität und für krankheitsbedingte Schäden oder geeignete prognostische Kriterien.

Abstract

Systemic sclerosis (SSc) is a polymorphic and heterogenic systemic disorder with inflammation, fibrosis and vascular damage. Early diagnosis and classification may be difficult if disease expression is oligosymptomatic (undifferentiated), presenting with only Raynaud’s phenomenon or limited scleroderma. Scleroderma specific antinuclear autoantibodies, which are present early and persistently in about 90% of the patients with SSc, play an important taxonomic role. Scleroderma specific findings in nailfold capillary microscopy are sensitive and predictive for evolving SSc. An algorithm will be presented for the diagnosis and classification of SSc using clinical, capillaroscopic and serologic criteria, which are also useful for mixed or special forms of SSc. The 6th Outcome Meaures in Rheumatoloty Clinical Trials (OMERACT) conference proposed different outcome measurements for clinical studies, however, for daily clinical practice there is as yet no consensus on status indices for disease activity, disease related damage or suitable prognostic criteria.

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Authors and Affiliations

  1. Klinik und Poliklinik für Dermatologie und Venerologie der Universität zu Köln

    T. Krieg

  2. Rheumaklinik und Rheumaforschungsinstitut Aachen, Burtscheider Markt 24, 52066, Aachen

    E. Genth

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  2. T. Krieg
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Correspondence to E. Genth.

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Genth, E., Krieg, T. Systemische Sklerose – Diagnose und Klassifikation. Z. Rheumatol. 65, 268–274 (2006). https://doi.org/10.1007/s00393-006-0065-0

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