Pronounced: VEGG-eh-nerz GRAN-u-luh-mah-TOE-sis
by Annie Stuart
Wegeners 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.
WG is a type of autoimmune disease. This means the bodys immune system attacks its own tissues. Its cause is unknown. An infection may trigger it, but none has yet been found.
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 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 dont 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
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 teststaking blood to check blood counts, sedimentation rate, kidney function, and antineutrophil cytoplasmic antibodies (ANCA)
Urinalysistaking 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 prednisoneto 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 cant 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
Wegeners 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: Wegeners granulomatosis. The Johns Hopkins Vasculitis Center website. Available at: http://vasculitis.med.jhu.edu/typesof/wegeners.html . Accessed September 21, 2005.
Wegeners granulomatosis. National Institute of Allergy and Infectious Diseases website. Available at: http://www.niaid.nih.gov/factsheets/wegeners.htm . Accessed September 21, 2005.
What is Wegeners 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 Wegeners granulomatosis? Wegeners Granulomatosis Association website. Available at: http://www.wgassociation.org/aboutwg/whatis_wg.shtml . Accessed September 21, 2005.
Last reviewed Janaury 2008 by Jill D. Landis, 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.
