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Systemische Amyloidosen

Systemic amyloidoses

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Zusammenfassung

Amyloidosen sind seltene Proteinfaltungskrankheiten, bei denen sich Proteine infolge einer Konformationsänderung als unlösliche fibrilläre Aggregate ablagern. Dies kann systemisch oder lokalisiert erfolgen. Systemische Amyloidosen sind lebensbedrohliche Komplikationen monoklonaler Gammopathien oder chronischer Entzündungen oder treten im Rahmen einer familiären Erkrankung auf. Die kausale Behandlung der Amyloidosen besteht in der Reduktion der amyloidbildenden Proteine durch Chemotherapie, antientzündliche Behandlung oder Lebertransplantation. Die Frühdiagnose der Erkrankung ist essenziell, um die Patienten effektiv behandeln zu können und eine weitere Funktionsverschlechterung der Organe zu verhindern.

Abstract

Amyloidoses are rare protein folding disorders, in which proteins are deposited as insoluble fibrillar aggregates due to a conformational change. This can occur in a local or systemic form. Systemic amyloidoses are life-threatening complications of monoclonal gammopathy, chronic inflammatory diseases or within hereditary diseases. The causative treatment of amyloidosis is the reduction of the amyloid precursor protein by chemotherapy, anti-inflammatory treatment, or liver transplantation. Early diagnosis of the disease is essential in order to effectively treat patients and avoid further deterioration of organ functions.

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Interessenkonflikt

Der korrespondierende Autor weist auf folgende Beziehungen hin: Forschungsunterstützung durch Celgene GmbH, Honorare für Vorträge und Finanzierung von Kongressteilnahmen von Janssen und Celgene GmbH.

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Authors and Affiliations

  1. Amyloidose-Zentrum, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Deutschland

    S. Schönland, N. Blank, A.V. Kristen, J. Beimler, T. Ganten & U. Hegenbart

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  1. S. Schönland
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  2. N. Blank
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  3. A.V. Kristen
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  4. J. Beimler
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  5. T. Ganten
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  6. U. Hegenbart
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Corresponding author

Correspondence to S. Schönland.

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Schönland, S., Blank, N., Kristen, A. et al. Systemische Amyloidosen. Internist 53, 51–64 (2012). https://doi.org/10.1007/s00108-011-2952-y

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  • DOI: https://doi.org/10.1007/s00108-011-2952-y

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