Immunoglobulin a nephropathy: Pathological markers of renal survival in paediatric patients

Nephrology (Carlton). 2016 Dec;21(12):995-1002. doi: 10.1111/nep.12850.

Abstract

IgA nephropathy (IgAN) is one of the leading causes of glomerulonephritis characterized by the findings of IgA and IgG immune deposits in the mesangium of kidney biopsies from patients with persistent microscopic haematuria. IgAN is frequently detected among adolescents and young adults. IgAN presents a highly variable course that includes a spectrum from a very mild disease to end-stage renal disease (ESRD). There are several clinical and histological factors that strongly determined the final outcome of patients with IgAN. Pathological variables associated with unfavorable outcomes are mesangial hypercellularity, segmental glomerulosclerosis, endocapillary hypercellularity and interstitial fibrosis/tubular atrophy, according to the Oxford classification. Moreover, some studies also suggest a role for complement activation in the pathogenesis of IgAN. In this regard, staining for C4d may be an independent risk factor for the development of ESRD in IgAN. Despite the growing number of studies assessing IgAN risk factors, this kind of investigation in paediatric patients is still very limited. The aim of this article is to revise pathological markers related to deterioration of renal function in paediatric patients with IgAN, particularly those that can independently affect renal survival.

Keywords: C4d deposits; IgA nephropathy; Oxford classification; chronic kidney disease.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Age Factors
  • Atrophy
  • Biomarkers / analysis
  • Biopsy
  • Child
  • Complement Activation
  • Complement C4b / analysis
  • Disease Progression
  • Female
  • Fibrosis
  • Glomerulonephritis, IGA / complications
  • Glomerulonephritis, IGA / immunology
  • Glomerulonephritis, IGA / pathology*
  • Glomerulonephritis, IGA / physiopathology
  • Glomerulosclerosis, Focal Segmental / immunology
  • Glomerulosclerosis, Focal Segmental / pathology*
  • Glomerulosclerosis, Focal Segmental / physiopathology
  • Humans
  • Immunoglobulin A / analysis
  • Immunoglobulin G / analysis
  • Kidney Failure, Chronic / immunology
  • Kidney Failure, Chronic / pathology*
  • Kidney Failure, Chronic / physiopathology
  • Kidney Function Tests
  • Kidney Glomerulus / immunology
  • Kidney Glomerulus / pathology*
  • Kidney Glomerulus / physiopathology
  • Male
  • Peptide Fragments / analysis
  • Predictive Value of Tests
  • Prognosis
  • Risk Factors

Substances

  • Biomarkers
  • Immunoglobulin A
  • Immunoglobulin G
  • Peptide Fragments
  • Complement C4b
  • complement C4d

Supplementary concepts

  • Segmental glomerulosclerosis