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Autoimmun Rev. 2014 Feb;13(2):108-13. doi: 10.1016/j.autrev.2013.09.005. Epub 2013 Sep 26.

Differential diagnosis of membranous nephropathy with autoantibodies to phospholipase A2 receptor 1.

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Institute for Experimental Immunology, Euroimmun, Seekamp 31, 23560 Lübeck, Germany. Electronic address:


Membranous nephropathy (MN) accounts for most cases of the nephrotic syndrome in adults. Recently, studies on the underlying pathomechanisms led to the identification of the podocyte M-type receptor for secretory phospholipase A2 (PLA2R1) as a target antigen of circulating autoantibodies. Autoantibodies to PLA2R1 may not only play a role in the development of primary MN, but also serve as a marker for diagnosis, disease activity and therapy monitoring. Antibody detection is crucial to discriminate between patients with primary MN and those with a secondary form of the disease, as both forms require different diagnostic approaches and treatment strategies. Standardized test systems based on recombinant PLA2R1 allow for the sensitive and specific analysis of anti-PLA2R1 autoantibodies. Further research into pathogenic mechanisms and other disease markers can pave the way for improved patient care.


Autoantibodies; ELISA; Indirect immunofluorescence; Membranous nephropathy; Nephrotic syndrome; Phospholipase A2 receptor 1

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