IgA nephropathy (also known as IgA nephritis or Berger disease) is a form of glomerulonephritis.
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Epidemiology
IgA nephropathy is considered the most common glomerulonephritis worldwide and is a leading cause of chronic kidney disease and renal failure.
Pathology
Primary IgA nephropathy is characterized by deposition of IgA antibodies in the glomeruli. This is thought to start with defective glycosylation of IgA subclass IgA1 that results in overproduction of galactose-deficient IgA1 (Gd-IgA1), occurrence of anti-Gd-IgA1 autoantibodies, and mesangial deposition of nephritogenic immune complexes. This may eventually lead to increased mesangial cell proliferation, inflammatory responses, and complement activation.
Associations
The most common association is IgA vasculitis (which is often considered a broader systemic form of IgA nephropathy).
Clinical presentation
- macroscopic hematuria in conjunction with respiratory or gastrointestinal infection (common presentation in younger patients)
- microscopic hematuria
- proteinuria
- hypertension
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