Definition

Wegener’s 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

WG is a type of autoimmune disease. This means the body’s immune system attacks its own tissues. The cause is unknown.

Risk Factors

WG 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

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.

WG can cause common cold- or flu-like symptoms such as:

• Fever
• Achy joints and muscles
• Headache
• Overall feeling of discomfort or fatigue
• Lack of appetite
• Weight loss

Common respiratory tract symptoms associated with WG 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
• Wheezing

Diagnosis

You will be asked about your symptoms and medical history. A physical exam will be done. 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

Treatment

The outlook is good with proper treatment, but without it, WG is fatal. WG is treated with medications. Some are used to induce remission, while others are used for maintenance when remission is achieved.

Prevention

There are no current guidelines to prevent Wegener’s granulomatosis.

Vasculitis Foundation http://www.vasculitisfoundation.org

Public Health Agency of Canada http://www.phac-aspc.gc.ca

Granulomatosis with polyangiitis. National Institute of Allergy and Infectious Diseases website. Available at:
http://www.niaid.nih.gov/topics/gpa/pages/default.aspx
Updated October 29, 2013. Accessed June 30, 2015.

Granulomatosis with polyangiitis (GPA/Wegener’s). Vasculitis Foundation website. Available at:
http://www.vasculitisfoundation.org/education/forms/granulomatosis-with-polyangiitis-gpa-wegeners
Accessed June 30, 2015.