Treatment And Prognosis Of IgA Nephropathy

Introduction
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.
Renal Prognsis
Patients with IgA nephropathy who have little or no proteinuria (less than 500 to 1000mg/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.
The rate of progression is typically slow with the GFR often falling by as little as 1 to 3 mL/min per year, a change not associated with an elevation in the serum creatinine concentration in the short term. Thus, a stable and normal serum creatinine concentration does not necessarily indicate stable disease. The frequency with which this occurs has been evaluated in studies in which repeat renal biopsy was used to assess the frequency of progressive disease. In one report, repeat renal biopsies were performed at five years in 73 patients with persistent proteinuria and a normal or near-normal initial serum creatinine. Histologic improvement occurred in only 4 percent, with 41 percent remaining stable and 55 percent showing progressive glomerular and secondary vascular and tubulointerstitial injury. An increase in serum creatinine to more than 1.5 mg/dL (133 micromol/L) was associated with major pathologic lesions.
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