TY - JOUR
T1 - Idiopathic membranoproliferative glomerulonephritis
T2 - Does it exist?
AU - Fervenza, Fernando C.
AU - Sethi, Sanjeev
AU - Glassock, Richard J.
N1 - Funding Information:
Conflict of interest statement. Work supported by research awards from the Fulk Family Foundation to S.S. and F.C.F.
PY - 2012/12
Y1 - 2012/12
N2 - 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.
AB - 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.
KW - C3 glomerulonephritis; membranoproliferative
KW - complement abnormalities
KW - dense deposit disease
KW - glomerular diseases
KW - glomerulonephritis
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U2 - 10.1093/ndt/gfs288
DO - 10.1093/ndt/gfs288
M3 - Review article
C2 - 22798508
AN - SCOPUS:84871193435
SN - 0931-0509
VL - 27
SP - 4288
EP - 4294
JO - Nephrology Dialysis Transplantation
JF - Nephrology Dialysis Transplantation
IS - 12
ER -