Journal Article

Idiopathic membranoproliferative glomerulonephritis: does it exist?

Fernando C. Fervenza, Sanjeev Sethi and Richard J. Glassock

in Nephrology Dialysis Transplantation

Published on behalf of European Renal Association - European Dialysis and Transplant Assoc

Volume 27, issue 12, pages 4288-4294
Published in print December 2012 | ISSN: 0931-0509
Published online July 2012 | e-ISSN: 1460-2385 | DOI: http://dx.doi.org/10.1093/ndt/gfs288
Idiopathic membranoproliferative glomerulonephritis: does it exist?

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When membranoproliferative glomerulonephritis (MPGN) was first delineated as a discrete clinico-pathological entity more than a half-century ago, most cases were regarded as idiopathic (or primary) in nature. Advances in analysis of pathogenetic mechanisms and etiologies underlying the lesion of MPGN have radically altered the prevalence of the truly idiopathic form of MPGN. In addition, MPGN as a category among renal biopsies showing glomerulonephritis has diminished over time. In the modern era, MPGN is mainly classified morphologically on the basis of immunoglobulin (Ig; monoclonal or polyclonal) and complement (C3 only or combined with Ig) deposition and secondarily on the basis of its appearance on ultra-structural examination. Idiopathic MPGN is a diagnosis of exclusion, at least in many adults and a portion of children, and a systematic approach to evaluation will often uncover a secondary cause, such as an infection, autoimmune disease, monoclonal gammopathy, neoplasia, complement dysregulation or a chronic thrombotic microangiopathy. Idiopathic MPGN remains an ‘endangered species’ after its separation from these known causes.

Keywords: complement abnormalities; dense deposit disease; glomerular diseases; glomerulonephritis; C3 glomerulonephritis; membranoproliferative

Journal Article.  4461 words.  Illustrated.

Subjects: Nephrology

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