Definition

Granulomatosis with polyangiitis (GPA) is a rare disease. It causes the walls of blood vessels to become inflamed. This is called vasculitis. It limits blood flow to tissues and can affect any organ.

Damage to Kidney Due to Decreased Blood Flow Copyright © Nucleus Medical Media, Inc.

Causes    TOP

The exact cause of GPA is unknown. The immune system attacks healthy blood vessels. This causes inflammation. It’s not known what causes the immune system to be overactive.

Risk Factors    TOP

GPA is more common in people over 65 years old. It’s also seen more in people who are White.

Symptoms    TOP

Symptoms are different in everyone. They range from mild to serious. In most cases, ear, nose, and throat symptoms appear first. They don’t respond to normal care and get worse over time.

GPA can cause:

• Fever
• Achy joints and muscles
• Headache
• Overall feeling of discomfort or tiredness
• Lack of hunger
• Weight loss

Other common problems:

• Hearing problems
• Ear pain
• Mid-facial pain
• Persistent nasal discharge, with crusts or sores that don't heal
• Frequent nosebleeds
• Ear discharge
• Frequent ear infections
• Ulcers in the mouth and nose
• Wheezing

Areas of Sinus Pain Copyright © Nucleus Medical Media, Inc.

Diagnosis    TOP

The doctor will ask about your symptoms and health history. The doctor may do tests to rule out other conditions, determine which organs are involved, or to confirm the diagnosis. You may have:

• A physical exam
• Blood tests
• Urine tests
• Biopsy—removal of a sample of affected tissue
• Imaging tests such as:
  • Chest x-ray
  • CT scan

Treatment    TOP

The outlook is good with proper care. The goal is to reduce inflammation of the blood vessels to bring on a remission. Without care, the condition can lead to death.

Medicines will:

• Ease inflammation in the arteries
• Change how the immune system works
• Help manage infections

Prevention    TOP

There is no way to prevent GPA since the cause is unknown.