Granulomatosis with polyangiitis (GPA), formerly called Wegeners granulomatosis (WG), is a rare disease that causes the walls of blood vessels to become inflamed, a condition called vasculitis. This limits blood flow to tissues and can affect any organ.
Causes of Granulomatosis with Polyangiitis
GPA is a type of autoimmune disease. This means the body’s immune system attacks its own tissues. The cause is unknown.
GPA does not appear to be passed from one generation to the next. It is more common in Caucasians, and in people of middle age.
Symptoms and their severity vary from one person to another. In most cases, ear, nose, and throat symptoms appear first. These symptoms do not respond to normal treatment and worsen over time.
GPA can cause common cold- or flu-like symptoms such as:
- Achy joints and muscles
- Overall feeling of discomfort or fatigue
- Lack of appetite
- Weight loss
Common respiratory tract symptoms associated with GPA may include:
- Hearing problems
- Ear pain
- Mid-facial pain
- Persistent nasal discharge, with crusts or sores that do not heal
- Recurrent nosebleeds
- Discharge from the ear
- Recurrent ear infection
- Ulcers in the mouth and nose
Your doctor will ask about your symptoms and medical history and conduct a physical exam. The doctor may do tests to rule out other conditions, determine which organs are involved or to confirm the diagnosis.
Tests may include:
- Blood tests
- Urine tests
- Biopsy — removal of a sample of affected tissue
Images may be needed of your bodily structures. This can be done with:
- Chest X-ray
- CT scan
The outlook is good with proper treatment, but without it, GPA is fatal. Medications can treat GPA. Some induce remission, while others are used for maintenance.