IgA nephropathy (Berger’s disease)

Alternative names
Nephropathy - IgA; Berger’s disease

Definition
IgA nephropathy is a kidney disorder characterized by blood in the urine. It is caused by inflammation of internal kidney structures and the deposit of IgA antibodies in kidney tissue.

Causes, incidence, and risk factors

IgA nephropathy (Berger’s disease) is a form of mesangial proliferative nephritis. Structures in the kidney called glomeruli become inflamed, and a type of antibody called IgA deposits in the kidney. The disorder can appear as acute, rapidly progressive, or chronic glomerulonephritis, or as visible or microscopic blood in the urine.

IgA nephropathy usually is discovered after one or more episodes of dark or bloody urine in a person with no other symptoms of kidney disorder. Bloody urine may begin during or soon after a respiratory infection. Acute nephritic syndrome or nephrotic syndrome (groups of symptoms associated with decreased kidney functioning) may result.

Risk factors include having a personal or family history of IgA nephropathy or Henoch Schonlein purpura (a form of vasculitis that affects many parts of the body, and may cause a kidney lesion that is identical to the lesion of Berger’s disease).

IgA nephropathy can occur in persons of all ages, but most often affects males in their teens to late 30s.

Symptoms

     
  • Changes in color of urine - bloody, dark, brown, or rust colored  
  • Repeated episodes of dark or bloody urine

Signs and tests
There are no specific changes seen from a physical examination. Occasionally, blood pressure may be elevated or swelling of the body may be present.

     
  • Urinalysis shows blood and red cell casts in the urine, and may also show protein and fats.  
  • Kidney biopsy shows deposits of IgA antibodies and confirms the diagnosis.  
  • Urine immunoelectrophoresis shows antibodies to IgA.

Treatment

The goal of treatment is to relieve symptoms and prevent or delay of chronic renal failure.

Antihypertensive medications and diuretics may be given to control high blood pressure and edema. Control of blood pressure is the most important measure to delay renal damage. Sodium (salt) and fluids may be restricted to control swelling. A low- to moderate-protein diet may be recommended in some cases.

The use of ACE inhibitors is key to preventing the progression of this disease.

Support Groups

For additional information and supprt, see the IgA Nephropathy Support Network website (http://www.igansupport.org).

Expectations (prognosis)


IgA nephropathy progresses slowly. In many cases, it does not progress at all. High blood pressure, large quantities of protein in the urine, and increased BUN or creatinine levels (blood tests that reflect kidney functioning) indicate a higher risk for progression of the disorder.

About 25% of people with IgA nephropathy develop end-stage kidney failure within about 25 years.

Complications

     
  • Chronic kidney failure  
  • End-stage kidney disease

Calling your health care provider
Call your health care provider if you have bloody urine or if your urine output decreases.

Johns Hopkins patient information

Last revised: December 5, 2012
by David A. Scott, M.D.

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