Handbook of Genetic Counseling/HIgA Nephropathy
< Handbook of Genetic CounselingIgA Nephropathy
What is IgA Nephropathy?
- 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 Disease
- 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
Treatment
- 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
Diagnosis
- 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
Causes
- 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
Notes
The information in this outline was last updated in 2001.
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