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 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.