Wegener's Disease

Wegener's disease, called Wegener's granulomatosis, is a rare condition that causes inflammation of the blood vessels. If it is not treated, the inflammation blocks or limits the flow of blood throughout the body, potentially affecting every organ and tissue. There is not one definitive test, but an early diagnosis is critical as Wegener's granulomatosis can progress to renal failure.

Definition

• Wegener's granulomatosis (WG) may affect any part of the body, but it is most frequently found in the upper and lower respiratory tract and kidney, followed by involvement of the lungs, eyes, ears and throat. The small blood vessels in the affected area become inflamed and clusters of cells called granulomas develop. If it is not treated, WG can be fatal but with early diagnosis and treatment the outcome is positive.

Symptoms

• People with Wegener's granulomatosis may have no symptoms, mild symptoms or even experience the sudden onset of acute symptoms. The first symptoms in most patients may not be noticed because they resemble a cold involving the upper respiratory tract. Beginning with a runny nose, the nasal discharge can become discolored or bloody and there may be nasal pain. Over time, the ears, lungs and eyes can become involved, resulting in an ear infection, cough or eye inflammation. Other symptoms include a fever, low energy and fatigue, joint pain and loss of appetite. The kidneys are involved in more than 75 percent of those diagnosed with WG. Unfortunately, WG in the kidneys usually does not cause symptoms, yet it is important to diagnosis this accurately and start treatment to avoid long-term damage.

Diagnosis

• Diagnosis can be difficult because the symptoms and severity of the disease are different for each person. The physician will take a medical history, conduct an exam and run lab tests. There are not any tests that specifically diagnose WG, but it is important to rule out other potential diseases and to look for markers that may indicate WG. One diagnostic test measures the amount of ANCA in the blood. ANCAs (anti-neutrophil cytoplasmic antibodies) are antibodies that attack the body's immune system. High levels of ANCA are not definitive, but they indicate possible WG and they can be used to determine the progression of the disease.

Treatment

• Medication is necessary to treat WG and may include steroids, immunosuppressive drugs and cytotoxins that destroy or block the production of cells. The most frequently used steroid is prednisone. The cytotoxic medications include Cytoxan and methotrexate. Antibiotics may also be prescribed to treat infections.

Consortium for Treatment

• The National Institutes of Health provided a grant to establish the Vasculitis Clinical Research Consortium (VCRC). Consisting of a group of six academic medical centers and under the direction of Boston University, the VCRC is dedicated to continuing research to improve the care of patients with WG and other forms of vasculitis (inflammation of the vessels). VCRC members provide a great first step for those who need to find expert doctors, obtain help with WG or who need a support group.