Last modified on 18 June 2006, at 11:03

Handbook of Genetic Counseling/HIgA Nephropathy

IgA Nephropathy

What is IgA Nephropathy?Edit

  • the most common non-diabetic kidney disease
  • IgA, a normal component of the blood, collects in the kidney as damaging deposits
  • due to either increased production or reduced clearance of the immune protein IgA and associated antigen complexes
  • deposits collect in small blood vessels in the glomeruli (the filtering structure of the kidney)
  • 20%- 30% of patients will suffer eventual kidney failure within 10-20 years
  • more likely if they have high levels of creatinine in blood at time of diagnosis
  • will require life saving dialysis and/or a kidney transplant
  • a large proportion of patients who present with symptoms have a mild form of the disease
  • most people probably never know they have the disease or discover it at a late stage

Warning Signs of Kidney DiseaseEdit

  • tea colored urine (hematuria - blood in the urine)
  • very foamy urine (proteinuria - protein in the urine)
  • puffiness around the eyes, hands, or feet (edema - fluid retention)
  • high blood pressure
  • pain in the small of the back just below the ribs not aggravated by motion.
  • frequent urge to urinate especially at night, and/or reduced amount of urine produced

TreatmentEdit

  • no medical treatments except in the latest stages of the disease
  • transplant success rate is good (symptoms mild, though deposits will appear in the transplanted kidney after one year)
  • some therapies can delay the deterioration of kidney function for many years
  • ACE inhibitors, fish oil, steroid treatment, tonsillectomy
  • control of associated hypertension is important

DiagnosisEdit

  • the presence of blood and/or protein in the urine
  • some nephrologists may microscopically examination urine and red blood cell casts
  • to confirm a diagnosis, it is necessary to perform a renal biopsy and examine kidney tissue microscopically for the presence of the characteristic IgA deposits in the glomeruli
  • no genetic testing available

CausesEdit

  • unknown (recurrence risk also unknown)
  • genetic influences may play a role in the development of the disease
  • seems to cluster in certain families and in certain areas of the world
  • associated with major histocompatibility HLA and certain other genetic markers
  • HLA-DR4 is detectable in about 50% of patients

NotesEdit

The information in this outline was last updated in 2001.