Idiopathic membranoproliferative glomerulonephritis: Does it exist?

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

Research output: Contribution to journalReview article

34 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)4288-4294
Number of pages7
JournalNephrology Dialysis Transplantation
Volume27
Issue number12
DOIs
StatePublished - Dec 2012

Keywords

  • C3 glomerulonephritis; membranoproliferative
  • complement abnormalities
  • dense deposit disease
  • glomerular diseases
  • glomerulonephritis

ASJC Scopus subject areas

  • Nephrology
  • Transplantation

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