Wegnerer's Granulomatosis Disease


Fact: Between 1 in 20,000 and 1 in 30,000 people are diagnosed with the disease

What is Wegnerer's Granulomatosis Disease?

Wegener's granulomatosis is a very rare, autoimmune disease that affects many different organs and systems of the body. It mainly attacks the respiratory system (sinuses, nose, windpipe, and the lungs) and the kidneys. One of the main features of the disease is an inflammation of the blood vessels (vasculitis). The inflammation narrows the blood vessels and reduces the blood flow to the affected organs. This destroys tissues and damages vital organs.

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What causes Wegnerer's Granulomatosis?

It is not known what causes this disease. While it appears similar to an infection, no bacteria or virus has been identified as a cause. Changes in the cells caused by this disorder suggest that it may be caused a type of allergic reaction. It can affect persons of any age. It is twice as likely to affect men as women.

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Are there complications with Wegnerer's Granulomatosis?


Besides affecting your nose, throat and lungs, granulomatosis with polyangiitis may affect your skin, eyes, ears, kidneys, heart and other organs. Complications may include:
  • Hearing loss
  • Skin scarring
  • Heart disease
  • Kidney damage
  • A loss of height in the bridge of the nose (saddling) caused by weakened cartilage
  • Deep vein thrombosis
How is it diagnosed?

Abnormal lab findings in patients with granulomatosis with polyangiitis (GPA) include urine tests that detect protein and red blood cells in the urine (not visible to the naked eye) and x-ray tests of the chest and sinuses which detect abnormalities resulting from lung and sinus inflammation. Blood tests that detect the abnormal inflammation include the sedimentation rate (sed rate) and C-reactive protein. A more specific blood test used to diagnose and monitor Wegener's granulomatosis is the antineutrophil cytoplasmic antibody (ANCA test), which is commonly elevated when the disease is active.

The diagnosis of GPA is confirmed by detecting both abnormal cellular formations, called granulomas, and vasculitis in a biopsy of tissue involved with the inflammatory process. For examples, an open lung biopsy or a kidney biopsy are commonly used in making a diagnosis of granulomatosis with polyangiitis.

Is there treatment for Wegnerer Granulomatosis Disease?

Granulomatosis with polyangiitis (GPA) is a serious disease and without treatment can be fatal within months. Treatment is directed toward stopping the inflammation process by suppressing the immune system.

Medications used to treat GPA include high-dose cortisone (prednisone) and the immunosuppressive drug cyclophosphamide (Cytoxan). Recent reports also suggest that trimethoprim/sulfamethoxazole (Bactrim) can also be helpful to prevent relapse of disease activity in patients with GPA.

Cytoxan that is taken by mouth with prednisone until the disease is in remission and then switched to methotrexate (Rheumatrex, Trexall) for two years and tapered off has been reported to be effective and less toxic than the traditional long-term Cytoxan treatment.

Methotrexate has recently been introduced as a drug for Cytoxan treatment failures. Moreover, it now appears that Cytoxan will not be necessary in order to maintain long-term remission and that doctors can convert to the less toxic methotrexate for maintenance. The reports also demonstrate that methotrexate can eventually be tapered off entirely after two years. Azathioprine (Imuran) has also been used as a maintenance medication after Cytoxan. Recently, intravenous immunoglobulin therapy (IVIG) has been shown to be helpful in treating relapses of granulomatosis with polyangiitis. Studies suggest that rituximab (Rituxan) may be helpful to maintain remission once the initial inflammatory disease has been controlled using medications mentioned above. These new regimens are welcome news for patients with GPA as medical researchers are searching for better treatments.
Symptoms: 

​Wegener's granulomatosis may either begin suddenly and severely or come on more mildly and gradually. It can take years for the full range of the disease to develop.

A person usually goes to the doctor with symptoms of a chest condition, including:

A general feeling of not being well
Fever
Joint pain that comes and goes
Loss of appetite
Congestion of the nose and sinuses
Sores on the lining of the nose
Bloody runny nose
Cough, which causes blood to come up
Inflammation of the linings of the lungs which causes difficulty and pain when breathing
Inflammation of the middle ear, which is painful, can cause hearing loss or potential rupture the eardrum
Weight loss
Inflammation of the nose is what usually brings patients to a doctor first. At this stage, Wegener's granulomatosis can often be mistaken for chronic sinusitis. The mucous membrane that lines the nose looks red, has a raised grainy appearance and bleeds easily. Other symptoms may also appear including rough, bumpy skin rashes, eye problems including redness of the eye and blockages of the ducts between the eyes and the nose and inflammation of the cartilage of the outer ear.

Serious complications may also develop as a result of Wegener's granulomatosis, including heart attack, meningitis or inflamed masses of the central nervous system that don't heal. Eventually, the condition spreads to the blood vessels and causes complications such as fluid leaking into the lungs and inflammation of the tiny capillaries of the kidneys. This latter condition can cause rising blood pressure and blood leaking into the urine.

Want to learn more?

The following are organizations and/or websites dedicated to providing information and education surrounding Wegnerer's Granulomatosis Disease. These organizations are dedicated to research, education, awareness, and/or support. They are listed in Alphabetical order without any preference or prejudice. Listing these organizations is not a recommendation or referral in any regard for seeking treatment or consultation or support for treatment.

Cedars-Sinai
Cleveland Clinic
John Hopkins Vasculitus Center
Mayo Clinic
Medline Plus
National Institute of Allergy and Infectious Diseases




​Information on this page taken from:
Mayo Clinic
Cedars-Sinai
Medicine Net