Granulomatosis with polyangiitis

Overview

Granulomatosis with polyangiitis (GPA) is a rare disease that causes swelling, also called inflammation, of small blood vessels. This condition mainly affects blood vessels in the nose, sinuses, throat, lungs and kidneys. But it can affect any organ.

GPA used to be called Wegener granulomatosis. It's one of a group of blood vessel diseases called vasculitis. GPA slows blood flow to some organs. The affected tissues can develop inflamed areas called granulomas. Granulomas can damage these organs and affect how they work.

Symptoms of GPA vary. At first, the symptoms can seem like those of a cold. Other symptoms depend on which organs the disease affects. The cause of GPA isn't clear, but the immune system may play a role.

It's important for healthcare professionals to find and treat GPA as soon as possible. Early treatment can help people live full lives. Without treatment, the condition can lead to organ damage that sometimes is fatal.

Causes

The exact cause of granulomatosis with polyangiitis (GPA) isn't clear. The immune system may play a role. Some white blood cells may not function as they should, and they may be involved in the disease. The immune system makes proteins called antibodies that protect the body from germs such as viruses and bacteria. GPA has been linked with the presence of certain antibodies that attack healthy cells by mistake.

The condition can lead to inflamed, narrowed blood vessels and harmful, inflamed tissue masses called granulomas. Granulomas can destroy healthy tissue. And narrowed blood vessels lower the amount of blood and oxygen that reaches tissues and organs.

GPA doesn't spread from person to person. And it likely doesn't pass from parents to children through genes.

Risk factors

Age is a risk factor for granulomatosis with polyangiitis. Most often, the disease affects people between the ages of 40 and 65. But it can happen at any age.

Complications

Granulomatosis with polyangiitis (GPA) can lead to other health conditions called complications. The complications depend on which organs or body parts the disease affects. They can include:

• Hearing loss.
• A loss of height in the bridge of the nose caused by weakened cartilage.
• Skin sores or scarring.
• Kidney damage or kidney failure.
• Bleeding and scarring of the lungs.
• A blood clot that forms in one or more deep veins, usually in the leg.
• Heart conditions such as swelling of the sac that surrounds the heart.
• Rarely, stroke or vision loss.

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