Wegener's Granulomatosis

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Wegener's Granulomatosis (WG) is an uncommon disease that affects about 1 in 20,000 to 1 in 30,000 people. Symptoms are due to inflammation that can affect many tissues in the body, including blood vessels (vasculitis). It is also considered a disease of abnormal immune function.

There is no known cause of WG; but it is not contagious, and there is no evidence it is hereditary. It is systemic, meaning it affects the body as a whole. It affects the upper (sinuses and nose), and lower (lungs), respiratory system and frequently involves the kidneys, lungs, eyes, ears, throat, skin and other body organs. For reasons not clear, blood vessels in those areas may become inflamed and clusters of certain cells (granulomas) may occur.

WG is an uncommon disease, which can occur at any age. It most often occurs in the 4th and 5th decade of life. Patients are divided equally between males and females. It appears that Caucasians are far more commonly affected than other racial groups.

Symptoms: Onset of WG may be indolent, slow moving with few symptoms, or have a rapid and severe onset. About 90% of patients have symptoms of a cold or runny nose or sinusitis that fail to respond to the usual therapeutic measures and last considerably longer than the usual upper respiratory tract infection. Other symptoms include nasal membrane ulcerations and crusting, saddle-nose deformity, inflammation of the ear with hearing problems, inflammation of the eye with sight problems, cough (with or without the presence of blood), pleuritis, (inflammation of the lining of the lung), rash and/or skin sores, fever, lack of energy, weakness, fatigue, loss of appetite, weight loss, arthritic joint pain, night sweats, and blood in urine which may or may not be indicated by a change in urine color.

Be aware that not all WG patients experience all symptoms. Different patients experience different symptoms, and the severity of the disease is also different for each WG patient. If any of the above symptoms persist, consider a possible diagnosis of WG and arrange to have a complete evaluation, including health history, physical exam, laboratory studies, including a urinalysis and an ANCA test.

Diagnosis is established by clinical and laboratory findings such as the ANCA blood test, other blood and urine tests, x-rays, and tissue biopsy, if needed.

The treatment of WG can be divided into two stages: firstly, the induction of disease remission, and secondly, the maintenance of disease remission. Medication usually consists of cytotoxic agents (a form of chemotherapy), using relatively low doses of Cytoxan, and/or methotrexate and/or azathioprine (Imuran), and glucocorticoids (prednisone).

Treatment will vary based on patient symptoms, disease activity, organ involvement and lab test results. Patients with kidney involvement and more severe WG are commonly prescribed Cytoxan and prednisone as initial treatment. Ideally, the use of Cytoxan will be limited to a three to six month period and then replaced, based on kidney function, by methotrexate or azathioprine.

Those with milder forms of WG are commonly prescribed methotrexate and prednisone. These medications will be reduced over time, and even eliminated, if the patient remains in a stable remission. WG patients may also be prescribed calcium supplements and other medications to prevent osteoporosis from extended prednisone use.

Many patients will also be prescribed the antibiotic Bactrim to help prevent secondary lung infections with a dangerous “bug” called Pneumocystis carinii pneumonia (PCP). In addition, there is some evidence that Bactrim, used cautiously, can have the beneficial effects of reducing relapses and upper airway infections.

Remission: There is no cure for WG, but early diagnosis and proper treatment will be effective and the disease can be brought into remission with complete absence of all signs of disease.

Long-term remission can be induced and maintained with medications, close management and regular lab tests to help monitor the disease. Treatment can produce symptom-free intervals of 5 to 20 years or more. Some patients will achieve a drug-free remission. However, relapses are common but can be caught at their earliest and most treatable stage, for most patients, by paying attention to patient symptoms and lab tests. WG patients in remission must not hesitate to see a doctor if any WG symptoms return or if they are not feeling well.