IgA nephropathy is the most common cause of primary (idiopathic)
glomerulonephritis in the developed world.Although this disorder was initially
thought to follow a benign course, it is now recognized that slow progression to
end-stage renal disease occurs in up to 50 percent of affected patients, often
over 20 to 25 years of observation. The remaining patients enter a sustained
clinical remission or have persistent low grade hematuria and/or proteinuria.
The prognosis is difficult to predict with accuracy in individual patients, but
important risk factors for progressive renal disease have been identified.
There are two major clinical presentations of IgA nephropathy: the classic
presentation with gross hematuria, often recurrent, following shortly after an
upper respiratory infection; and persistent asymptomatic microscopic hematuria
with or without mild to moderate proteinuria.The diagnosis may be suspected in
patients with the classic presentation, but must be confirmed by kidney biopsy,
which, as described below, often provides prognostic information.
The renal prognosis and treatment of IgA nephropathy will be reviewed here.
The pathogenesis of IgA nephropathy and the outcomes in patients who undergo
renal transplantation are discussed separately.
Patients with IgA nephropathy who have little or no proteinuria (less than
500 to 1000 mg/day) have a low risk of progression, at least in the short term.
However, proteinuria and renal insufficiency develop in a substantial proportion
of patients over the long term.Among patients who develop overt proteinuria
and/or an elevated serum creatinine concentration, progression to end-stage
renal disease is approximately 15 to 25 percent at 10 years and 20 to 30 percent
at 20 years.
A low antigen diet consists of avoiding gluten, dairy products, eggs, and
most meats.
The rationale for this regimen is that dietary macromolecules may be
responsible for activating the mucosal IgA system. When given to 21 consecutive
patients with IgA nephropathy, protein excretion was markedly reduced or fell
into the normal range in 11 of the 12 patients whose baseline rate was more than
1 g/day. In addition, repeat renal biopsy showed significant reductions in
mesangial IgA and complement deposition and mesangial cellularity.
The benefits in the above study have not been confirmed and a report using a
gluten-free diet alone for several years did not demonstrate improvement in
either proteinuria or renal function despite a reduction in the level of
circulating IgA-containing immune complexes
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