Arthritis: Wegener's Granulomatosis

Wegener's granulomatosis is a rare but potentially deadly disease of uncertain cause. It is characterized by inflammation of small and medium-sized blood vessels ( vasculitis). This inflammation can damage many organs of the body.

Wegener's granulomatosis primarily affects the upper respiratory tract (nasal sinuses, nose and trachea, the upper airway), lungs, and kidneys. Other organ systems that can be affected by the disease include the nervous system, ears, eyes, heart skin, and joints.

Who Gets the Disease?

Wegener's granulomatosis can affect people of all ages but is most commonly seen in the 4th and 5th decades of life. It affects men and women equally.

What Are the Symptoms?

The symptoms and their severity vary among patients. General signs of the disease may include loss of appetite, weight loss, fever, and fatigue.

Most patients first notice symptoms in the respiratory tract, including:

• Persistent runny nose or the formation of nasal crusts and sores
• Nasal or facial pain
• Nosebleeds or unusual nasal discharge, caused by inflammation of the nose or sinuses
• Cough that may include bloody phlegm caused by upper airway or lower airway (lung) inflammation
• Chest discomfort
• Middle ear inflammation, pain, or hearing loss
• Voice change, wheezing, or shortness of breath caused by inflammation of the trachea

Other possible symptoms include:

• Eye inflammation
• Joint or muscle pain
• Rashes or skin sores
• Kidney inflammation (although kidney inflammation is common, it is not usually associated with symptoms such as pain)

How Is the Disease Diagnosed?

Because Wegener's granulomatosis has symptoms similar to a number of other disorders, diagnosing it can be difficult.

Your doctor makes the diagnosis based on the combination of symptoms and results of physical examinations, laboratory tests, and X-rays. Sometimes a biopsy (tissue sample) of affected tissue (skin, nose, sinus, lung, or kidney) is taken and analyzed.

A positive blood test for antineutrophil cytoplasmic antibodies (ANCA) can support a suspected diagnosis of the disease. However, this blood test does not, by itself, prove the diagnosis of Wegener's granulomatosis. In addition, a negative test does not rule out the diagnosis of Wegener's granulomatosis.

How Is the Disease Treated?

Because Wegener's granulomatosis is often a life-threatening disease, it is treated with a variety of powerful medications that have been shown to save lives.

Treatment usually includes corticosteroid medications such as prednisone, and chemotherapy drugs such as Cytoxan, lmuran or Rheumatrex. When used to treat Wegener's granulomatosis and other similar conditions, chemotherapy drugs are used at much lower doses than when treating cancer. Thus, there are typically fewer side effects than with cancer treatment.

These drugs suppress the immune system and usually induce remission (complete absence of all signs of the disease). Improvement usually occurs within days to weeks. When the disease is in remission, patients can reduce the dosage of these medications but will continue taking them until the disease has been in continuous remission for one year.

Because the medications used to treat Wegener's granulomatosis can have serious side effects, patients are monitored closely by their doctors. The dosage of medication is adjusted as needed throughout the course of treatment.