Wegener’s Granulomatosis

(WG; Granulomatosis with polyangiitis)

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. The outlook is good with proper treatment, but without it, WG is fatal.
Damage to Kidney Due to Decreased Blood Flow
Kidney damage
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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
Areas of Sinus Pain
Sinus Headache: Areas of Pain
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Diagnosis

Your doctor will ask 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
Imaging tests to see internal body structures include chest x-ray or CT scan .

Treatment

WG is treated with medications. Some are used to induce remission, while others are used for maintenance once remission is achieved.

Medications

Your doctor may recommend one or more of the following:
  • Corticosteroids to reduce inflammation and pain
  • Immunosuppresants
  • Medications to prevent infection or bone loss

Prevention

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

RESOURCES

National Institute of Allergy and Infectious Diseases (NIH)
http://www.niaid.nih.gov

Wegener’s Granulomatosis Association
http://www.wgassociation.org

CANADIAN RESOURCES

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

The Arthritis Society
http://www.arthritis.ca

References

Granulomatosis with polyangiitis. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed.ebscohost.com/about/about-us. Updated August 6, 2013. Accessed August 7, 2013.

Granulomatosis with polyangiitis. National Institute of Allergy and Infectious Diseases website. Available at: http://www.niaid.nih.gov/topics/gpa/pages/default.aspx. Updated July 31, 2013. Accessed August 7, 2013.

Granulomatosis with polyangiitis (GPA/Wegener's). Vasculitis Foundation website. Available at: http://www.vasculitisfoundation.org/education/forms/granulomatosis-with-polyangiitis-gpa-wegeners. Updated September 2012. Accessed August 7, 2013.

Wegener’s granulomatosis? The Cleveland Clinic website. Available at: http://www.clevelandclinic.org/health/health-info/docs/0200/0214.asp?index=4757. Accessed August 7, 2013.

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