IgA Nephropathy (Berger Disease)

IgA nephropathy is a type of kidney disease. The name stands for immunoglobulin A nephropathy (nuh-FROP-uh-thee). IgA is an antibody, a protein your immune system makes to protect you from bacteria and viruses.

In people with IgA nephropathy, IgA builds up in their kidneys and causes inflammation. This inflammation damages kidney tissues.

IgA nephropathy is also called Berger’s disease. Two French doctors, Jean Berger and Nicole Hinglais, discovered the condition.

What’s the difference between IgA nephropathy and selective IgA deficiency?

Both conditions have to do with the protein IgA. People with selective IgA deficiency either don’t have enough IgA or have low levels of it.

How do my kidneys work?

The kidneys are part of your urinary system. Here’s how they typically work:

• Kidneys: These bean-shaped organs are on each side of your spine. They filter your blood, removing waste and extra water. The waste and water leave your body as urine. The kidneys then reabsorb the water and chemicals that your body needs.
• Nephrons: The tiny filtering units in each kidney are called nephrons. Each kidney has about a million nephrons.
• Glomerulus and tubule: Each nephron contains these structures. The glomeruli (plural of glomerulus) are tiny blood vessels that filter blood. The tubules return what you need to your blood and remove waste.

What is the connection between IgA nephropathy and ESRD?

IgA nephropathy attacks the glomeruli. This type of glomerular disease occurs when IgA deposits build up and damage the glomeruli. The damage causes your kidneys to leak blood (hematuria) and protein (proteinuria) into your urine.

Eventually, the nephrons may scar, causing kidney disease. As the scarring progresses, you may develop end-stage kidney (renal) disease (ESRD). This process can happen quickly over the course of months or can take as long as 20 years after your initial diagnosis.

If you develop ESRD, your kidneys can’t work well enough to keep you healthy. You may need:

• Dialysis, a machine that helps filter your blood.
• Kidney transplant, surgery to remove your damaged kidney and replace it with a healthy donor kidney.

Who is at risk for IgA nephropathy?

Risk factors for IgA nephropathy include:

• Family history of IgA nephropathy.
• Family history of IgA vasculitis, (Henoch-Schönlein purpura).
• Being a young adult male (teens to late 30s).
• Asian or European ethnicity.

How common is IgA nephropathy?

IgA nephropathy is a common kidney disease. It can happen to people at any age.

Usually, the first signs of the disease occur in a person’s late teens to late 30s. In the United States, men are twice as likely as women to have IgA nephropathy.