• Vol. 29 No. 3, 364–369
  • 15 May 2000

Genetics of Immunoglobulin A Nephropathy

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ABSTRACT

Introduction: Immunoglobulin (Ig) A nephropathy is the most common primary glomerulonephritis in the world and about 20% to 50% of patients with it develop progressive renal failure. There is considerable evidence to show that IgA nephropathy is influenced by genetic factors. The purpose of this review is to provide useful information concerning genetics of IgA nephropathy.

Methods and Results: Epidemiological, familial clustering, human leukocyte antigen and IgA immune system (immunoglobulin class switch gene, Iα1 germ-line transcript regulatory region gene) studies have led to the hypothesis of genetic susceptibility to IgA nephropathy. Moreover, research on renin angiotensin system, platelet activating factor acetylhydrolase, neuropeptide Y Y1 receptor and others genes has demonstrated that genetic factors influence the pathological severity and natural course of IgA nephropathy.

Conclusions: The evidence presented in this review strongly supports the role of genetic factors in IgA nephropathy. Detection of genetic risk factors for IgA nephropathy will allow us to study further the pathogenesis of IgA nephropathy and devise effective therapy.


Immunoglobulin (Ig) A nephropathy is the most common primary glomerulonephritis in various parts of the world, and it was detected in 25% of biopsy specimens taken from children in Kobe University Hospital. IgA nephropathy was initially considered to be a benign disease with a favourable prognosis, but as data from a long-term follow up study became available, it was recognised that the disease progressed to renal failure in 20% to 50% of adult patients.

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