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Wegener's Granulomatosis

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(WG)

Definition

Wegener’s granulomatosis (WG) is a rare disease that causes the walls of blood vessels to become inflamed (called vasculitis). This limits blood flow to tissues and can affect any organ. If left untreated, WG can be fatal. The outlook, though, is good with proper treatment. If you suspect you have WG, see your doctor right away.
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. Its cause is unknown. An infection may trigger it, but none has yet been found.

Risk Factors

A risk factor is something that increases your chance of getting a disease or condition. The risk factors for WG are not clear. Males and females are equally affected. It can occur at any age, although it more often begins in middle age. It does not appear to be passed from one generation to the next. Compared with Caucasians, however, African-Americans rarely get the disease.

Symptoms

Symptoms may vary from mild to severe. They may also progress at a different rate from one person to another. Often, WG first causes chronic cold, sinus, or allergy-like symptoms. If you have any of the following symptoms, do not assume they are due to WG. See your doctor. They may be caused by other, less serious health conditions. Symptoms of WG may include:
  • Upper respiratory tract problems
    • Recurring middle ear infections that get better very slowly and cause ear problems and hearing loss
    • Chronic sinus inflammation, causing congestion and pain
    • Runny nose and other “cold” symptoms that don’t respond well to treatment
    • Nasal crusting or ulcers, and frequent nosebleeds
    • Erosion of the septum between the nasal passages, which can cause the bridge of the nose to collapse
    Areas of Sinus Pain
    Sinus Headache: Areas of Pain
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  • Chest pain, shortness of breath, cough, or coughing up blood; hoarseness or voice changes
  • Muscle or joint pain or swelling
  • Red, burning, or painful eyes; double vision or decrease in vision
  • Skin problems
    • Rash; small red or purple raised areas
    • Blister-like lesions, ulcers, or nodules
    • Extreme sensitivity to cold in fingers or toes
  • Other possible symptoms include:
    • Fever or night sweats
    • Fatigue or weakness
    • Weight loss or loss of appetite
    • Changes in urine color
    • Numbness, tingling, shooting pain, or weakness in arms or legs

Diagnosis

Your doctor will ask about your symptoms and medical history, and perform 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—taking blood to check blood counts, sedimentation rate, kidney function, and antineutrophil cytoplasmic antibodies (ANCA)
  • Urinalysis—taking a sample of urine to check for signs that kidneys are involved
  • Chest x-ray —a test that uses radiation to take a picture of lungs
  • CT scan —a type of x-ray that uses a computer to make pictures of structures, such as sinuses
  • Biopsy —removal of a sample of tissue (eg, sinus, nose, skin, lung, or kidney) to check for signs of WG

Treatment

Talk with your doctor about the best treatment plan for you. Treatment usually includes a combination of medication types.

Medications

  • Oral corticosteroids, such as prednisone—to reduce inflammation and pain
  • Chemotherapy —to control the overactive immune system; these may include cyclophosphamide, methotrexate, or azathioprine.
  • Reduced dosages of these drugs for at least a year, if all signs of disease go away
  • Medications to prevent infection or bone loss, which are possible side effects of treatment

Exercise

Although it can’t cure WG, exercise may help you manage its symptoms.

Prevention

Because the cause of this condition is unknown, it is not yet clear what you can do to prevent it.

RESOURCES

American Autoimmune Related Diseases Association http://www.aarda.org

National Institute of Allergy and Infectious Diseases (NIH) http://www3.niaid.nih.gov

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

CANADIAN RESOURCES

BC Health Guide, British Columbia Ministry of Health http://www.bchealthguide.org

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

References

Types of vasculitis: Wegener’s granulomatosis. The Johns Hopkins Vasculitis Center website. Available at: http://vasculitis.med.jhu.edu/typesof/wegeners.html . Accessed September 21, 2005.

Wegener’s granulomatosis. National Institute of Allergy and Infectious Diseases website. Available at: http://www.niaid.nih.gov/topics/wegeners/Pages/default.aspx . Accessed September 21, 2005.

What is Wegener’s granulomatosis? The Cleveland Clinic website. Available at: http://www.clevelandclinic.org/health/health-info/docs/0200/0214.asp?index=4757. Accessed September 21, 2005.

What is Wegener’s granulomatosis? Wegener’s Granulomatosis Association website. Available at: http://www.wgassociation.org/aboutwg/whatis%5Fwg.shtml. Accessed September 21, 2005.

Revision Information

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.

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