What Is IgA Nephropathy (Berger's Disease)?

What is IgA nephropathy (Berger's disease)?

Berger's disease or immunoglobulin A (IgA) nephropathy is a kidney disorder
Berger's disease or immunoglobulin A (IgA) nephropathy is a kidney disorder.

Berger's disease or immunoglobulin A (IgA) nephropathy is a kidney disorder characterized by the passing of blood in the urine (hematuria). This happens due to swelling of the glomeruli (filtering units of the kidney that produce urine).

  • The swelling is usually caused due to a buildup of a substance called IgA in the kidneys. This hampers the filtration process of the kidneys.
  • IgA is a type of antibody (protein) manufactured by the immune system. When the body is unable to filter the urine, it can't excrete body wastes or eliminate excess fluid from the bloodstream.
  • IgA nephropathy leads to kidney failure and other related complications. It may be acute (attacking quickly and suddenly) or chronic.
  • IgA nephropathy often strikes in the teen or early adult years. 
  • It affects more men than women.

What causes IgA nephropathy (Berger's disease)?

The causes of IgA nephropathy (Berger’s disease) include the following:

  • Anyone at any age can get IgA nephropathy, although it's more common in men. Caucasians and Asians also have a higher incidence of IgA nephropathy than other ethnic groups. The predisposing factors for IgA nephropathy are as follows:
    • Genetic: IgA nephropathy is more common in some families and certain ethnic groups such as Europeans and Asians.
    • Liver diseases: Liver cirrhosis is a condition where inelastic scar tissue replaces normal tissue within the liver, and infections are caused by hepatitis B and hepatitis C virus.
    • Celiac disease: A reaction to eating gluten, a protein found in most grains, triggers this digestive condition.
    • Infections: These include human immunodeficiency virus (HIV) and some bacterial infections.
  • No one knows why IgA nephropathy happens, but it can happen more than once and for years. The frequency and duration of the condition do not indicate much about the eventual outcome, and 85% of cases result in no long-term kidney damage, with kidney function remaining normal all the while.
  • In some cases, it can develop after a child or young adult has a viral infection of the upper respiratory or gastrointestinal tracts. For some people, a genetic defect may be linked to the development of IgA nephropathy.

What are the signs and symptoms of IgA nephropathy (Berger's disease)?

Signs and symptoms of IgA nephropathy (Berger’s disease) include the following:

What tests diagnose IgA nephropathy (Berger's disease)?

Diagnosing IgA nephropathy: Blood in the urine is the most common sign of IgA nephropathy. If blood or protein is found in your urine, a series of tests may be ordered by your doctor. These tests can usually determine if the kidneys are injured and how well they are working.

  • Blood urea nitrogen (BUN) test to measure kidney function
  • A creatinine blood test to measure kidney function
  • Kidney biopsy to confirm the diagnosis
  • Urinalysis
  • Urine immunoelectrophoresis

What is the treatment for IgA nephropathy (Berger's disease)? Is it possible to prevent IgA nephropathy?

Treatment options:

IgA nephropathy cannot be prevented and has no cure. Some people can live with the disease without any complications. However, half of the people who do get IgA nephropathy may progress to end-stage renal disease. Doctors may recommend the following:

  • High blood pressure medications (such as an angiotensin-converting enzyme or ACE inhibitors prescribed by your doctor).
  • Immunosuppressants (such as corticosteroids and methylprednisolone).
  • Omega-3 fatty acids (reduces inflammation in the kidneys)
  • A kidney diet (as planned by the dietitians) where salt and fluids may be restricted to control swelling. A low to moderate protein diet may be recommended in some cases.
  • Medicines to lower the cholesterol in the body
  • Eventually, many people may need dialysis and kidney transplants, as well.

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References
Tashakkorinia, N., Muco, E., and Tudor, M.E. "Berger Disease (Thromboangiitis Obliterans)." In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing, January 2020. <https://www.ncbi.nlm.nih.gov/books/NBK499998/>.