Handbook of Genetic Counseling/HIgA Nephropathy

IgA 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

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

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

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

The information in this outline was last updated in 2001.