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Rapid-progressive Glomerulonephritis

Rapid progressive glomerulonephritis

  • Schwerpunkt: Nephritiden
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Zusammenfassung

Eine rapid-progressive Glomerulonephritis ist ein internistischer Notfall, der durch einen raschen Nierenfunktionsverlust innerhalb von Wochen bis wenigen Monaten (selten Tagen) aufgrund einer histologisch extrakapillär proliferativen Glomerulonephritis mit Nekrosen und Halbmonden gekennzeichnet ist. Eine frühzeitige Diagnose verbessert die Prognose erheblich, da der Kreatininwert bei Therapiebeginn entscheidend ist. Immunhistologisch wird die Erkrankung in drei Gruppen eingeteilt; unterschieden werden dabei keine oder wenige Immunglobuline (antineutrophile-zytoplasmatische-Antikörper[ANCA]-assoziierte Vaskulitis), eine lineare Ablagerung (Anti-GBM-Erkrankung oder Goodpasture-Syndrom) oder granuläre Immunglobulinablagerungen, etwa bei systemischem Lupus erythematodes, Purpura Schönlein-Henoch oder kryoglobulinämischer Vaskulitis. Die immunsuppressiven Möglichkeiten haben sich erheblich erweitert (beispielsweise Induktion bei ANCA-assoziierter Vaskulitis mit niedrigerer Steroidgesamtdosis, Cyclophosphamidpulsen oder Rituximab), neue Therapien sind auf dem Weg und die Prognose hat sich erheblich verbessert. Die Frage der Therapiedauer und die Suche nach Rezidivmarkern stellen bis heute eine Herausforderung dar.

Abstract

The rapid progressive glomerulonephritis is an emergency case. Renal function is rapidly lost within weeks or a few months (rarely within days) due to necrotizing extracapillary proliferative crescentic glomerulonephritis. Early diagnosis and treatment improve prognosis, as the best prognostic marker is creatinine when treatment is initiated. Three classes can be distinguished by immunofluorescence in histology. Firstly, there are no or only few immunoglobulins found (anti-neutrophil cytoplasmic antibody [ANCA]-associated vasculitis). Secondly, there is linear immunofluorescence due to antibodies against the glomerular basement membrane (anti-glomerular basement membrane [GBM] disease or Goodpasture syndrome); and thirdly, there is a granular pattern of immunoglobulin deposition (for example systemic lupus erythematosus [SLE], Schoenlein-Henoch purpura or cryoglobulinaemia). The immunosuppressive repertoire has improved (such as induction therapy in ANCA-associated vasculitis with lower total steroid dose, cyclophosphamide pulses or rituximab). New treatment approaches are on their way and the prognosis regarding life expectancy and renal function has improved. There are still challenging questions to answer like treatment duration and markers of recurrence.

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Authors and Affiliations

  1. Medizinische Klinik III – Nieren- und Hochdruckerkrankungen, Klinikum Fulda gAG, Universitätsmedizin Marburg – Campus Fulda, Pacelliallee 4, 36043, Fulda, Deutschland

    Marion Haubitz

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  1. Marion Haubitz
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Correspondence to Marion Haubitz.

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M. Haubitz hat Vortragshonorare von der Firma Roche erhalten.

Dieser Beitrag beinhaltet keine vom Autor durchgeführten Studien an Menschen oder Tieren.

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H. Haller, Hannover

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Haubitz, M. Rapid-progressive Glomerulonephritis. Internist 60, 478–484 (2019). https://doi.org/10.1007/s00108-019-0575-x

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