Der Nephrologe

, Volume 7, Issue 3, pp 209–221 | Cite as

Kryoglobulinämische Vaskulitis

Leitthema
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Zusammenfassung

Kryoglobulinämische Vaskulitiden sind immunkomplexvermittelte Vaskulitiden kleiner und mittelgroßer Gefäße und werden v. a. im Rahmen einer Hepatitis-C-Infektion oder Kollagenose gesehen. Die typische Symptomatik umfasst eine Purpura der Haut, Arthralgien und Polyneuropathie, häufig gekoppelt mit einer membranoproliferativen Glomerulonephritis. Neuerdings existieren Klassifikationskriterien, die laborchemische und klinische Kriterien umfassen, nachdem Kryoglobuline im Serum nachgewiesen wurden. Therapeutisch steht die Behandlung der zugrunde liegenden Erkrankung im Vordergrund, d. h. in den meisten Fällen die antivirale Therapie der ursächlich auslösenden Hepatitis C, zurzeit mit pegyliertem Interferon α und Ribaverin. Glukokortikoide und Cyclophosphamid werden bei lebens- oder organbedrohenden Manifestationen eingesetzt (große Hautulzera, motorische Polyneuropathie, rapid-progressives Nierenversagen), können aber die Viruselimination verzögern und bilden bei den nicht virusvermittelten Kryoglobulinämien die Primärtherapie. B-Zell-depletierende Substanzen (Rituximab) haben hervorragende Ergebnisse sowohl bei der Hepatitis-C-assoziierten als auch bei der essenziellen kryoglobulinämischen Vaskulitis erbracht, mit Nachweis der Überlegenheit gegenüber Steroiden plus weiterer Immunsuppressiva. Seit dem Einsatz von Rituximab beschränkt sich der Einsatz der Plasmapherese auf Patienten mit Hyperviskosität durch Kryoglobuline.

Schlüsselwörter

Kryoglobulinämie Kryoglobulinämische Vaskulitis Hepatitis C Glomerulonephritis Therapie 

Cryoglobulinemic vasculitis

Abstract

Cryoglobulinemic vasculitis consists of a group of immune complex-mediated diseases of small and medium-sized vessels, which mainly occur in association with hepatitis C infections or connective tissue diseases. Typical symptoms include purpuric skin changes, arthralgia and polyneuropathy often in conjunction with membranoproliferative glomerulonephritis. Classification criteria have recently been established which encompass serological and clinical parameters after detection of cryoglobulins in serum. Therapy is based on treatment of the underlying primary disease, e.g. antiviral treatment of hepatitis C with pegylated interferon-alpha and ribavirin. Corticosteroids and cyclophosphamide are administered for life or organ function threatening manifestations (e.g. large skin ulcers, severe motor neuropathy, rapid progressive glomerulonephritis), however they can slow down virus elimination and thus constitute the primary treatment in cases of non-virus-associated cryoglobulinemia. The B-cell depleting drugs (e.g. rituximab) have proven very effective for hepatitis C-associated as well as essential cryoglobulinemia and have shown to be superior to steroids plus additional immunosuppressants. Since the introduction of rituximab plasma exchange is only used in cases with hyperviscosity due to cryoglobulins.

Keywords

Cryoglobulinemia Cryoglobulinemic vasculitis Hepatitis C Glomerulonephritis Therapy 

Notes

Interessenkonflikt

Der korrespondierende Autor gibt für sich und seine Koautoren an, dass kein Interessenkonflikt besteht.

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Copyright information

© Springer-Verlag 2012

Authors and Affiliations

  1. 1.Klinik für Innere Medizin (Allgemeine Innere Medizin, Rheumatologie, Nephrologie)Kreiskliniken Esslingen gGmbH, Akademisches Lehrkrankenhaus der Universität TübingenPlochingenDeutschland
  2. 2.Med. Klinik III (Nephrologie, Hypertensiologie, Rheumatologie)Klinikum Offenbach GmbH, KfH Nierenzentrum OffenbachOffenbachDeutschland

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