Definition

Granulomatosis with polyangiitis (GPA) is a rare disease of the blood vessels. The walls of blood vessels become inflamed. This slows the flow of blood to tissue. It can lead to damage of connected organs. Left untreated it can be fatal.

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

Causes

The immune system begins to attack healthy blood vessels. This causes inflammation. It’s not clear what causes this change in the immune system.

Risk Factors

GPA is more common in people over 65 years old.

Symptoms

Symptoms will vary. They can range from mild to serious. Ear, nose, and throat symptoms often appear first. They are common but don’t respond to normal care. 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

The doctor will ask about your symptoms and past health. Tests may be needed to rule out other conditions. The doctor may suspect GPA based on your symptoms. A blood test will help to confirm it. A sample of the tissue may also be taken. It will be looked at in a lab for more details. Other tests may be done to look for any changes in kidneys or lungs.

Treatment

Treatment can help to reduce inflammation. It can create a period of time without disease called remission.

Medicines will:

• Ease inflammation in the arteries
• Decrease the reaction of the immune system

The choice of medicine will depend on what organs are affected. Treatment may last several months or years.

Prevention

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