Labordiagnostik bei Kollagenosen und Vaskulitiden

Zusammenfassung

Die Kollagenosen und Vaskulitiden sind chronisch-entzündliche Systemerkrankungen mit Beteiligung des Immunsystems. Bei allen Kollagenosen und einigen wichtigen primären Vaskulitiden zeigt sich die Immun-Komponente vor allem in der Assoziation mit charakteristischen Autoantikörpern. Der Beitrag des Labors in der Diagnostik dieser Erkrankungen besteht daher – neben entzündungsdiagnostischen, hämatologischen, urinanalytischen, klinisch-chemischen, cytologischen und histologischen Untersuchungen – vor allem in der Bestimmung diagnostisch richtungsweisender Autoantikörper. Grundstein der Labordiagnostik ist dabei der Nachweis von antinukleären Antikörpern (ANA) bzw. Anti-Neutrophilen Cytoplasmatischen Antikörpern (ANCA). In der Regel wird stufenweise ein globaler Immunfluoreszenztest auf zellulärem Substrat (HEp-2-Zellen bzw. humane neutrophile Granulocyten) kombiniert mit spezifischen Bindungstests zur Identifikation der für die jeweiligen Erkrankungen typischen Antikörper-Spezifitäten. Der Nachweis dieser Autoantikörper unterstützt Diagnose-Sicherung oder -Ausschluss, Sub-Spezifizierung der Erkrankungs-Entitäten, Prognose und – seltener – die Verlaufsbeobachtung.

Abstract

Connective tissue diseases and vasculitides are chronic inflammatory systemic diseases in which the immune system is involved. In all connective tissue diseases and some important primary vas­culitides the immunological component becomes manifest in characteristic autoantibodies. Besides analyses of inflammatory, haematological, urine analytical, chemical, cytological and histological parameters, the laboratory therefore mainly contributes to diagnostics of these diseases by measuring diagnostically useful autoantibodies, based on antinuclear antibodies (ANA) or antineutrophil cytoplasmic antibodies (ANCA). Usually this is accomplished by stepwise combination of a global immunofluorescence assay with cellular substrates (HEp-2 cells or human neutrophils) and specific binding assays to identify the antibody specificities typical for the disease in question. Detection of these autoantibodies supports confirmation or exclusion of the diagnosis, sub-typing of disease entities, prognosis and – less often – longitudinal observation.

Schlüsselwörter

Kollagenosen - Vaskulitis - Autoantikörper - antinukleäre Antikörper - ANCA

Key words

connective tissue disease - vasculitis - autoantibodies - antinuclear antibodies - ANCA

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