Renale Beteiligung beim Sjögren-Syndrom

Zusammenfassung

Das Sjögren-Syndrom kann über die obligate Siccasymptomatik hinaus verschiedene Organe mitbeteiligen. Die Nierenbeteiligung ist dabei eine häufige Organmanifestation. Sie bleibt aber oftmals unerkannt, da die Symptome in den meisten Fällen eher blande sind. Die häufigste Nierenbeteiligung ist die interstitielle Nephritis, die sich klinisch beginnend von einer milden tubulären Proteinurie bis hin zu ausgeprägten tubulären Elektrolytstörungen manifestieren kann. Glomeruläre Beteiligungen sind seltener. Eine typische glomeruläre Nierenbeteiligung ist die membranoproliferative Glomerulonephritis bei Kryoglobulinen. Daneben sind aber auch andere Glomerulonephritiden beschrieben, die sich jedoch histologisch nicht von den primären Formen der entsprechenden Glomerulonephritiden unterscheiden lassen. Akute Verlaufsformen mit sich rasch verschlechternder Nierenfunktion sind nur kasuistisch beschrieben. Die Therapie ist empirisch und besteht hauptsächlich in der Gabe von Kortikosteroiden. Bei Kryoglobulinämie assoziierten Glomerulonephritiden ist auch der erfolgreiche Einsatz der Plasmapherese beschrieben.

Abstract

In addition to the obligatory sicca symptoms, the Sjögren's syndrome can affect different internal organs. The renal involvement is very common, but is undetected in the majority of cases, because of the only mild symptoms. The most frequent renal involvement is an interstitial nephritis. The clinical appearance can range from mild tubular proteinuria to severe electrolyte disorder. Glomerular involvement is more unusual. One rare but typical glomerular involvement is the finding of a membranoproliferative glomerulonephritis, associated to cryoglobulins. In addition, many other types of glomerulonephritis have been published in case series, but histological findings of these types of glomerulonephritis are identical to those of primary glomerulonephritis. Acute renal failure is a very rare finding and was reported only in sporadic cases. The treatment is empirical because of the absence of randomised studies. Case series suggest a positive effect of corticosteroids. In cryoglobulinemic glomerulonephritis also plasmapheresis might be successful.

Schlüsselwörter

renale Beteiligung - interstitielle Nephritis - renal tubuläre Azidose - Kryoglobuline - Glomerulonephritis

Key words

renal involvement - interstitial nephritis - renal tubular acidosis - cryoglobulins - glomerulonephritis

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