Zusammenfassung
Hintergrund
Die membranöse Glomerulonephritis (MG) ist die häufigste Ursache für ein nephrotisches Syndrom bei Erwachsenen ohne Diabetes mellitus. In den letzten Jahren wurden wichtige Erkenntnisse in der Pathophysiologie der MG gewonnen, die rasch in die Klinik umgesetzt werden konnten.
Ziel
Der Übersichtsartikel fast die aktuellen Erkenntnisse zur Pathophysiologie und Diagnostik der MG zusammen. Zudem werden die aktuellen Empfehlungen zur Therapie im Licht der neusten Studien diskutiert.
Material und Methoden
Die Arbeit beruht auf einer Literaturrecherche zum Thema in der Datenbank PubMed bis einschließlich August 2015.
Ergebnisse
Die MG ist eine Autoimmunerkrankung des Podozyten; als Hauptzielantigene bei der primären MG konnten PLA2R (Phospholipase-A2-Rezeptor) und THSD7 A („thrombospondin type‑1 domain-containing 7 A“) im Podozyten identifiziert werden. Die Bestimmung der Anti-PLA2R-Antikörper im Serum hat sich rasch in der Diagnostik der MG etabliert. Es gibt zudem gute Hinweise dafür, dass der Anti-PLA2R-Antikörper-Titer ein Aktivitätsparameter der Erkrankung ist, der bei der Indikation und der Steuerung der immunsuppressiven Therapie hilfreich sein könnte. Eine persistierende große Proteinurie, eine initial erniedrigte initiale Kreatinin-Clearance und die Veränderung der Kreatinin-Clearance im Verlauf sind wichtige Risikofaktoren für den Abfall der Nierenfunktion. In etwa 30 % der Fälle tritt eine Spontanremission auf. Die Patienten werden zunächst optimal supportiv behandelt. Nur Patienten mit einem hohen Risiko für einen Nierenfunktionsverlust werden immunsupprimiert.
Diskussion
Die wiederholte Bestimmung der Antiköper bei Patienten mit MG könnte ein wichtiger Schritt zu einer individualisierten Therapie der MG sein. Der Effekt einer Therapie mit Rituximab sollte in kontrollierten randomisierten Studien überprüft werden.
Abstract
Background
Membranous glomerulonephritis (MG) is the most common cause of nephrotic syndrome in nondiabetic adults. In recent years major insights into the pathophysiology of MG have been gained, which could be rapidly translated into clinical practice.
Aims
This overview summarizes the insights into the pathophysiology and the diagnosis of MG; moreover, the recommendations for therapy of MG are discussed in the light of the latest available studies.
Material and methods
The article is based on a literature search of this topic in PubMed up to and including August 2015.
Results
The MG is an autoimmune disease of podocytes. The phospholipase A2 receptor (PLA2-R) and recently thrombospondin type 1 domain containing 7A (THSD 7A) have been identified as the major target antigens on podocytes in idiopathic MG. The detection of anti-PLA2-R antibodies has been rapidly established in the diagnostics of MG. There is good evidence that the anti-PLA2-R antibody titer reflects the activity of the disease and it may also be helpful in the indications and monitoring of immunosuppressive therapy. Persistent nephrotic proteinuria, initially reduced basal creatinine clearance and a decline in creatinine clearance during the course of the disease are important predictors for a decline in renal function. Approximately 30 % of patients with MG develop spontaneous remission. Patients initially receive optimal supportive therapy. Only patients with a high risk of developing a loss of renal function should receive immunosuppressive therapy.
Discussion
Monitoring of autoantibody titers might be an important step towards an individualized treatment of MG. The effect of therapy with rituximab should be examined in controlled randomized studies.
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Pavenstädt, H. Membranöse Glomerulonephritis. Nephrologe 11, 96–105 (2016). https://doi.org/10.1007/s11560-015-0021-6
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DOI: https://doi.org/10.1007/s11560-015-0021-6