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Nephrology

Ari Kostadaras, M.D.

IgA NEPHROPATHY

IgA nephropathy or Berger's disease is a clinical/pathological entity defined by the presence of macroscopic or microscopic hematuria and mesangial IgA deposits. The disease is found throughout the world but is most prevalent in Japan, Australia, Southeast Asia, and Southern Europe. In the U.S. the incidence is approximately 4% of all renal biopsies but may be as high as 45% of biopsies in Japan. It is not a benign illness as once believed, with up to 25% of patients ultimately developing renal failure. The pathogenesis of the disease remains unknown.

A number of features typify the presentation of IgA nephropathy. Hematuria is a presenting sign in >95% of cases. The hematuria may either be a single episode of gross hematuria, or recurrent episodes of gross hematuria with asymptomatic microscopic hematuria between episodes, or persistent asymptomatic microscopic hematuria. Episodes of hematuria are often associated with upper respiratory infections. The hematuria develops within 1- 2 days of the onset of the URI. By contrast, hematuria does not develop until 10- 14 days after pharyngitis in cases of classic acute glomerulonephritis. Other signs and symptoms include proteinuria, nephrotic syndrome, acute nephritis, malignant hypertension, chronic renal failure, and acute renal failure. Proteinuria is typically within the mild range. There is a 2:1 male predominance and a peak incidence in late childhood through early adult life.

Findings on renal biopsy are characteristic. In all patients, IgA is present and is deposited mainly in the mesangium of the glomerular tuft. Other immunoglobulins and complement, especially C3, are often found in a mesangial pattern. Light microscopy frequently reveals a focal lesion, present in some but not all glomeruli. Electron microscopy will show a nodular electron dense deposit in either a subepithelial or subendothelial pattern.

In large series of IgA nephropathy in children, end stage renal disease occurs in 5- 10%. Some clinical features have been correlated with a more rapid decline in renal function. These include male sex, older age at onset, absence of recurrent gross hematuria as a presenting complaint, and hypertension at the time of biopsy. In addition the histologic picture on renal biopsy correlates with outcome. Minimal glomerular abnormalities, pure mesangial proliferation, and focal or segmental change are associated with little change in renal function. Proliferative glomerulonephritis and crescents are associated with the high likelihood of progressive renal disease.

There is no known effective treatment for IgA nephropathy. Steroids and immunosuppressive agents have been tried with little success. Dilantin is known to reduce circulating serum levels of IgA but has not proven to be efficacious. At present, conservative therapy with attention to hypertension and renal insufficiency is appropriate.

Glomerulonephritis with a predominant pattern of IgA deposition occurs in other conditions including chronic hepatitis, SLE, and Henoch- Schonlein purpura. The renal manifestations of HSP are those of IgA nephropathy. This has prompted some to suggest that IgA nephropathy and HSP are illnesses along a continuum with a common etiology. All patients with IgA nephropathy should have careful followup with special attention to persistent proteinuria, hypertension, or progressive renal insufficiency.

REFERENCES

Levy, M. Idiopathic recurrent macroscopic hematuria and mesangial IgA deposits in children. Clin. Nephrol. 1973, 1: 63.

Michelk, D. Idiopathic mesangial IgA glomerulonephritis in childhood: Review of the literature. Eur. J. Ped. 13: 134, 1980.

Hogy, R. IgA nephropathy: Natural history and prognostic indices. Contrib. Nephrol. 40: 214, 1984.

Levy, M. Berger's disease in children: Natural history and outcome. Medicine 64: 157, 1985.

Friedman, A. IgA nephropathy. Contemp. Peds. Nov. 1987, p.53.

Melvin, T. IgA Nephropathy, in Holliday ed. Pediatric Nephropathy, Williams & Wilkin 1987, p. 437.

 

 

 

 

 

 


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