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Wegener’s Granulomatosis

(WG; Granulomatosis with polyangiitis)

Pronounced: VEGG-eh-nerz GRAN-u-luh-mah-TOE-sis
En Español (Spanish Version)

Definition | Causes | Risk Factors | Symptoms | Diagnosis | Treatment | Prevention

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.

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.

Last reviewed August 2013 by Michael J. Fucci, DO; Brian Randall, MD

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.


 
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