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Berger disease

ORPHA34145
Synonym(s) IgA nephropathy
Prevalence -
Inheritance Multigenic/multifactorial
Age of onset All ages
ICD-10
  • N02
OMIM
UMLS -
MeSH -
MedDRA -

Summary

Berger disease, also called IgA nephropathy (IgAN), is characterized by prominent mesangial IgA deposition, with variable degrees of mesangial proliferation. IgAN affects up to 1 in 79 individuals in the general population worldwide and is therefore not a rare disease. Typical clinical features include hematuria with or without proteinuria and episodes of macroscopic hematuria. Approximately 30% of the patients develop chronic renal failure. The pathogenesis of IgAN is unknown. It has been suggested that glycosylation abnormalities of the IgA hinge region play a key role in mesangial deposition. A genetic study performed on 30 multiple IgAN families recently confirmed that genetic factors are important in disease susceptibility. This study provided evidence that a gene on chromosome 6 is involved in the disease. Only a subset of IgAN patients with adverse prognostic features (such as hypertension, proteinuria > 1 g/24 hour, slowly progressive renal failure, and severe histologic changes) may be candidates for therapy. Treatment options include angiotensin-converting enzyme (ACE) inhibitors, steroids, immunosuppressive drugs and fish oil supplements. For patients who progress to end-stage renal failure, treatment consists of dialysis or a kidney transplant. The success rate of transplantation is good, although de novo IgA deposits appear in the transplanted kidney in about half the patients.

Expert reviewer(s)

  • Dr Silvana SAVOLDI

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Detailed information

Article for general public
  • FR (2006,pdf)
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