Zusammenfassung
Die Immunglobulin-A-Nephropathie (IgAN) ist die häufigste primäre Glomerulonephritisform in der westlichen Welt. Die pathogenetische Bedeutung von autoimmunen Mechanismen, Genetik und Umwelt- bzw. Ernährungsfaktoren ist nicht abschließend geklärt. Ein maßgeblicher Anteil der Patienten präsentiert sich mit milden Symptomen, jedoch ist die individuelle Prognose einer IgAN oftmals schwer vorhersagbar. Bei etwa einem Drittel der Patienten nimmt die Erkrankung einen stabilen, benignen Verlauf, wohingegen sich bei etwa 30 % eine chronische Niereninsuffizienz mit drohender Dialysepflicht entwickelt. Als Risikofaktoren für die Progression der Erkrankung gelten eine persistierende Mikrohämaturie und Proteinurie über 1 g/Tag, das Vorliegen einer arteriellen Hypertonie und das Ausmaß der tubulointerstitiellen Fibrose bei Diagnosestellung. In neueren genomweiten Assoziationsstudien konnten zahlreiche Risikoallele identifiziert werden, die zur Pathophysiologie der IgAN beitragen. Dabei rückt die sogenannte Darm-Nieren-Achse, aber auch das Komplementsystem zunehmend in den Fokus. Neben intensivierten supportiven Therapiemaßnahmen spielt die Steroidtherapie in seltenen Fällen weiterhin eine Rolle, wohingegen andere immunsuppressive Strategien überwiegend verlassen wurden. Zudem werden neue Therapieansätze verfolgt, von verkapseltem Budesonid bis hin zur Hemmung der Lymphozytenaktivierung.
Abstract
Immunoglobulin A nephropathy (IgAN) is the most prevalent primary form of glomerulopathy in the western world. The pathogenetic relevance of autoimmune mechanisms, genetics and environmental or nutritional factors is not fully established. The majority of IgAN patients present with mild symptoms; however, the exact prognosis of the individual IgAN course is often difficult to predict. In approximately one third of the patients the disease remains on a stable benign course, whereas approximately 30% may develop end-stage renal disease. Risk factors for disease progression are a persistent microhematuria and proteinuria >1 g/day, arterial hypertension and the extent of tubulointerstitial fibrosis at the time of diagnosis. Recent genome-wide association studies (GWAS) identified numerous risk alleles, which can contribute to the pathophysiology of IgAN. The so-called gut-kidney axis as well as the complement system and genes that are linked to mucosal immunity appear to be important for the manifestation of the disease. Intensive supportive care should be initiated as first-line treatment and only rare cases with progressive features require treatment with corticosteroids. Other immunosuppressive treatment strategies have currently no indications for IgAN. Future approaches might be the use of local budesonide or the inhibition of lymphocyte activation.
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J. Floege war in den letzten 4 Jahren beratend für Pharmalink (Nefecon-Studie) tätig. C. Seikrit und T. Rauen geben an, dass kein Interessenkonflikt besteht.
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Seikrit, C., Rauen, T. & Floege, J. Immunglobulin-A-Nephropathie. Internist 60, 432–439 (2019). https://doi.org/10.1007/s00108-019-0588-5
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DOI: https://doi.org/10.1007/s00108-019-0588-5