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Amyloiddiagnostik bei rheumatischen Erkrankungen

Amyloid diagnostics in rheumatic diseases

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Zusammenfassung

Amyloid ist eine pathologische Proteinablagerung im Gewebe, die im konventionell Hämatoxylin/Eosin-gefärbten Schnittpräparat homogen eosinrot erscheint und nach Kongorotfärbung im polarisierten Licht eine charakteristische apfelgrüne Polarisationsfarbe aufweist. Über 26 verschiedene, autologe physiologische Proteine sind bereits beschrieben worden, die Amyloid bilden können. In der klinischen Pathologie ist die Immunglobulin-Leichtketten-assoziierte AL-Amyloidose die häufigste generalisiert auftretende Amyloidose, gefolgt von der hereditären und nichthereditären ATTR-Amyloidose und der AA-Amyloidose. Die AA-Amyloidose tritt meistens im Gefolge einer chronisch-infektiösen oder -inflammatorischen Grunderkrankung auf und kann eine potenziell lebensbegrenzende Komplikation darstellen. Das Ursachenspektrum der AA-Amyloidose hat sich in den vergangenen Jahrzehnten gewandelt und wird jetzt von chronisch-rheumatischen Erkrankungen und hereditären Fiebersyndromen bestimmt. Die frühzeitige Diagnose einer Amyloidose und deren korrekte Klassifikation stellen unverändert eine große Herausforderung dar. Eine genaue Klassifikation des Amyloids und der Amyloidose ist für die Prognoseabschätzung und Therapieplanung unverzichtbar. Für die Behandlung der AA-Amyloidosen stehen neben entzündungshemmenden zukünftig möglicherweise auch spezifische Behandlungsstrategien zur Verfügung.

Abstract

Amyloid is a pathological protein deposit in tissue which has a red eosin color when the slice preparation is stained with traditional hematoxylin and eosin and after Congo red staining under polarized light exhibits a characteristic apple-green polarization color. Over 26 different autologous physiological proteins have been described that can form amyloid. In surgical pathology, immunoglobulin light chain-associated AL amyloidosis is the most frequent generally occurring amyloidosis, followed by hereditary and nonhereditary ATTR amyloidosis and AA amyloidosis. AA amyloidosis mostly develops subsequent to chronic infectious or inflammatory underlying disease and can represent a potentially life threatening complication. The spectrum of causes for AA amyloidosis has changed in the past few decades and is now determined by chronic rheumatic diseases and hereditary periodic fever syndromes. Early diagnosis of an amyloidosis and its correct classification continue to pose a great challenge. Precise classification of the amyloid and amyloidosis is essential for prognosis assessment and treatment planning. In addition to anti-inflammatory management of AA amyloidosis, specific treatment strategies may possibly become available in the future.

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Röcken, C., Ernst, J. Amyloiddiagnostik bei rheumatischen Erkrankungen. Pathologe 27, 422–430 (2006). https://doi.org/10.1007/s00292-006-0861-y

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