IgA nephropathy (Berger's disease)

IgA nephropathy is a kidney disorder that occurs when IgA-a protein that helps the body
fight infections-settles in the kidneys. After many years, the IgA deposits may cause the kidneys
to leak blood and sometimes protein in the urine.
Many people with IgA nephropathy leak blood in the urine, but this leakage does not
mean they will have long-term problems. Others leak both blood and protein in the urine. If too
much protein leaks into the urine, the hands and feet can swell. After 10 to 20 years with IgA
nephropathy, the kidneys may show signs of damage. About 25 percent of adults with IgA
nephropathy develop total kidney failure. Only 5 to 10 percent of children develop total kidney
failure. Symptoms of kidney failure include swelling in the hands and feet, nausea, fatigue,
headaches, and sleep problems. By the time these symptoms occur, total kidney failure is near.
Total kidney failure means the kidney damage is permanent. People with kidney failure need
dialysis or a kidney transplant.

Pathophysiology
The pathogenesis of IgA nephropathy remains incompletely understood. The
characteristic pathologic findings by immunofluorescence microscopy of granular deposits of
IgA and complement 3 (C3) in the glomerular mesangium suggest that this disease is the result
of the deposition of circulating immune complexes leading to the activation of the complement
cascade.
Deposited IgA is predominantly polymeric IgA1, which is mainly derived from the
mucosal immune system. The association of some cases of IgA nephropathy with syndromes
that affect the respiratory tract or gastrointestinal tract, such as celiac disease, led to the
suggestion that IgA nephropathy is a disease of the mucosal immune system. This concept is
also supported by the clinical observation that hematuria worsens during or after upper
respiratory tract or gastrointestinal tract infections.
The role of the complement system in the pathogenesis of IgA nephropathy is
controversial. While IgA antibodies cannot activate complement through the classic pathway,
studies have shown that complement can be activated by the alternate pathway.
IgA in the mesangium is likely to be deposited from the circulation as IgA-containing immune
complexes. This hypothesis is supported by the high recurrence rate of IgA nephropathy in renal
transplant recipients who have IgA nephropathy and, conversely, by the disappearance of the
deposits from donor kidneys with IgA nephropathy when transplanted into donors without the
disease. Furthermore, the mesangial pattern of IgA deposits suggests that circulating IgA
complexes are responsible for the disease.
Serum IgA levels are elevated in approximately half of patients with IgA nephropathy,
but that increase is unlikely to play a role in the pathogenesis of the disease, as markedly
elevated IgA levels are observed in patients with AIDS who do not have IgA nephropathy.
However, IgA is probably accumulated and deposited because of a systemic abnormality rather
than a defect intrinsic to the kidney.

Symptoms
There may be no symptoms for many years.
Symptoms include:
Bloody urine that starts during or soon after a respiratory infection
Repeated episodes of dark or bloody urine
Swelling of the hands and feet
Symptoms of chronic kidney disease

How to diagnose
A urine test called urinalysis usually provides the first clues. In a urinalysis, the doctor or
nurse dips a special strip with chemicals into the urine sample. The strip changes color when
blood or protein is present in the urine. If the test strip is positive, the urine will then be
examined with a microscope to look for red blood cells. The red blood cells may be clumped
together to form little tubes. These tubes are called casts because they are formed or molded
inside the kidneys tiny draining structures. If casts are found, it usually means the kidney filters
are damaged.
Blood tests measure the waste products in the blood that the kidneys usually get rid of.
Two examples are creatinine and blood urea nitrogen (BUN). If the BUN and creatinine levels
are high, it means the kidneys are not working well. If the creatinine level is high at the time of
diagnosis, the patient is more likely to develop kidney failure.
If there is kidney damage, the doctor will probably recommend a kidney biopsy. In this
procedure, a needle is used to retrieve a small piece of kidney tissue for examination with
different microscopes. Only a biopsy can show the IgA deposits in the kidney filters. The biopsy
can also tell how much kidney damage has already occurred. The biopsy results can help the
doctor determine the best treatment. Once a diagnosis of IgA nephropathy is established, a
person should have regular blood tests to monitor kidney function.

Treatment
The goal of treatment is to relieve symptoms and prevent or delay chronic renal failure.
You may get medicines to control high blood pressure and swelling (edema), such as
angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs).
Controlling blood pressure is the most important way to delay kidney damage.
Corticosteroids, other drugs that suppress the immune system, and fish oil have also been used
to treat this disorder.
Salt and fluids may be restricted to control swelling. A low to moderate protein diet may be
recommended in some cases.
Some people will need to take medicines to lower their cholesterol.
Eventually, many patients must be treated for chronic kidney disease and may need dialysis