IgA nephropathy

IgA nephropathy is the most common form of glomerulonephritis seen world-wide, having replaced poststreptococcal glomerulonephritis in this respect. The disease consists of focal proliferative glomerulonephritis with mesangial deposits of IgA.

In some cases IgG, IgM and C3 may also be seen in the glomerular mesangium. The disease may be a result of an exaggerated bone marrow and tonsillar IgA 1 immune response to viral or other antigens associated with an abnormality in O-linked glycosylation in the hinge region of the IgA 1 molecule. Such abnormal IgA 1 molecules may bind to other IgA 1 molecules or to fibronectin forming macromolecular aggregates which are cleared slowly from the circulation and become trapped passively in the glomerular mesangium.

IgA nephropathy tends to occur in children and young males. They present with asymptomatic microscopic haematuria or recurrent macroscopic haematuria sometimes following an upper respiratory or gastrointestinal viral infection. Proteinuria occurs and 5% can be nephrotic.

The prognosis is usually good especially in those with normal blood pressure, normal renal function and absence of proteinuria at presentation. Surprisingly, recurrent macroscopic haematuria is a good prognostic sign although this may be due to 'lead-time bias', patients with overt haematuria coming to medical attention at an earlier stage of their illness.

Meta-analysis has not confirmed initial evidence suggesting a beneficial effect of fish oil. Claims of benefit have been made for corticosteroid treatment with or without immunosuppressive drugs but a meta-analysis of controlled trials provides no convincing evidence that benefit exceeds the risks of treatment.

Approximately 20% of patients develop renal failure 20 years from the time of diagnosis. Mesangial IgA deposits are commonly found in the allografts of transplanted patients but loss of graft function as a result is uncommon.

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