Der Nephrologe

, Volume 8, Issue 3, pp 265–275 | Cite as

Therapie der fokal segmentalen Glomerulosklerose

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Zusammenfassung

Die fokal segmentale Glomerulosklerose (FSGS) ist eine häufige Ursache für das nephrotische Syndrom. Sie ist eine histologische Diagnose, die durch eine Schädigung der Podozyten charakterisiert ist, und keine singuläre Krankheitsentität. Es wird eine primäre von einer sekundären Form unterschieden. Es ist wichtig, eine sekundäre FSGS zu erkennen, denn diese Patienten haben oft keine nephrotische Proteinurie und werden in der Regel nicht immunsuppressiv behandelt. Unbehandelte Patienten mit einer primären FSGS mit nephrotischem Syndrom haben eine schlechte renale Prognose. Wichtige Prognosefaktoren sind das Ausmaß der Proteinurie, eine eingeschränkte Nierenfunktion, histologische Charakteristika und das Ansprechen auf eine Steroidtherapie. Es gibt keine randomisiert placebokontrollierten Studien zur Initialtherapie der primären FSGS. In der Regel werden Patienten mit einer primären FSGS mit hochdosierten Steroiden behandelt. Von Steroidresistenz spricht man, wenn die Proteinurie nach mehr als 4 Monaten hochdosierter Steroidtherapie persistiert. Als Alternativen zur Therapie der primären FSGS stehen Calcineurininhibitoren, Mycophenolat-Mofetil, Cyclophosphamid und Rituximab zur Verfügung.

Schlüsselwörter

Nephrotisches Syndrom Proteinurie Immunsuppressive Therapie Steroidtherapie Steroidresistenz 

Therapy of focal segmental glomerulosclerosis

Abstract

Focal segmental glomerulosclerosis (FSGS) is one of the leading courses of idiopathic nephrotic syndrome in adults. It is a histological diagnosis rather than a disease and characterized by damage to podocytes. It occurs as a primary form or as a secondary response to glomerular injury. Distinguishing between primary and secondary forms is important because immunosuppressive therapy is not indicated in patients with secondary FSGS. Patients with secondary FSGS often present with non-nephrotic proteinuria and untreated patients with nephrotic syndrome have a poor renal prognosis. Prognostic factors that influence renal survival of patients with FSGS include the degree of proteinuria, renal dysfunction, histological findings and the response to steroid therapy. There are no randomized controlled trials comparing steroids or other agents to placebo for initial therapy of primary FSGS; however, first line treatment of nephrotic patients with primary FSGS is high dose steroids. Steroid resistance is defined by persistence of the nephrotic syndrome after a more prolonged course of steroid therapy over more than 4 months. Alternative therapies include treatment with calcineurin inhibitors, mycophenolate mofetil, cyclophosphamide and rituximab.

Keywords

Nephrotic syndrome Proteinuria Immunosuppressive therapy Steroid therapy Steroid resistance 

Notes

Interessenkonflikt

Der korrespondierende Autor gibt an, dass kein Interessenkonflikt besteht.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  1. 1.Medizinische Klinik DUniversitätsklinikum MünsterMünsterDeutschland

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