IgA Nephropathy

Background

  • Also known as Berger’s disease
  • Most common form of idiopathic glomerulonephritis resulting in CKD
    • Around 30-40% of patients go on to develop end-stage CKD within 20 years.
    • Usually presents in young adults and there is a slight male predominance (males tend to have a poorer prognosis)
    • Can be associated with a number of other conditions
      • e.g. Henoch Schonlein purpura; SLE; autoimmune hepatitis; ankylosing spondylitis

Pathophysiology

  • Characterised by IgA- and C3- complex deposition in the glomerular mesangium
  • It is uncertain as to the exact mechanism by which this occurs but patients with IgAN seem to have an raised level of circulating IgA
    • It is thought that a specific type of IgA (galactose-deficient IgA) is responsible
  • As the disease progresses, several features may be seen (all contribute towards a poorer prognosis- collectively known as Oxford classification)
    • Increased mesangial cellularity
    • Segmental glomerulosclerosis
    • Endocapillary hypercellularity
    • Tubular atrophy/interstitial fibrosis

Presentation

  • Usually presents with frank haematuria following an upper respiratory tract infection
    • May also present with microscopic haematuria (more common in older adults) or an AKI
  • Usually this resolves in several days
    • If haematuria persists, there is usually progression to renal failure

Investigation

  • Urinalysis (dipstick)
    • Usually shows protein and blood
  • Urine microscopy (red cells, leukocytes and casts)
  • Urinary protein and creatinine (24 hour-collection)
  • Plasma IgA may be raised in around 50% of cases
  • Renal biopsy will give a definitive diagnosis

Management

  • Manage any hypertension – ACE inhibitors have shown to be beneficial
    • ACEIs plus Angiotensin receptor blockers may have additional benefit
  • Steroids (a 6 months course of prednisolone) is thought to be protective against progression to ESKD
  • Regular monitoring of renal function is important
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