Wegener's granulomatosis (patient information)

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Editors-in-Chief: C. Michael Gibson, M.S., M.D. Associate Editor-In-Chief: Ujjwal Rastogi, MBBS [mailto:urastogi@perfuse.org]

Overview
Wegener's granulomatosis is a rare disease. It is a type of vasculitis, or inflammation of the blood vessels. This limits the flow of blood to important organs, causing damage. It can affect any organ, but it mainly affects the sinuses, nose, trachea (windpipe), lungs and kidneys.

What are the symptoms?
Frequent sinusitis is the most common symptom. Other early symptoms include a fever that continues without an obvious cause, night sweats, fatigue, and a general ill feeling ( malaise). Chronic ear infections are common. Other upper respiratory symptoms include nose bleeds, pain, and sores around the opening of the nose. Loss of appetite and weight loss are common. Skin changes are also common, but there is no one typical lesion associated with the disease. There may be symptoms of kidney disease. The urine may be bloody. Eye problems develop in many people with Wegener's granulomatosis. The eye problems range from mild conjunctivitis to severe swelling of the eye. Other symptoms include:
 * Chest pain
 * Cough, with or without blood
 * Joint pain
 * Shortness of breath
 * Weakness
 * Wheezing

What are the causes?
The cause is unknown. It is thought to be an autoimmune disorder.

Who is at highest risk?
Wegener's granulomatosis is most common in middle-aged adults. It is rare in children, but has been seen in infants as young as 3 months old. Whites are more likely to get it as compared to Blacks.

When to seek urgent medical care?
Call your health care provider if you develop chest pain, coughing up blood, blood in the urine, or other symptoms of this disorder.

Diagnosis
Your doctor may order a blood test that looks for proteins called antineutrophil cytoplasmic antibodies (ANCA), which are often found in people with active Wegener's granulomatosis. However, it is not foolproof -- the test may be negative in some patients.

Urinalysis is done to look for signs of kidney disease such as protein and blood in the urine. Sometimes urine is collected over 24 hours to better check how the kidneys are working.

A biopsy is sometimes needed to confirm the diagnosis. The exact type of biopsy depends on which area of the body the doctor wants to look at. Your doctor may suggest one of the following:


 * Bronchoscopy with biopsy
 * Kidney biopsy
 * Nasal mucosal biopsy
 * Open lung biopsy
 * Skin biopsy
 * Upper airway biopsy

Other tests that may be done include:


 * Bone marrow aspiration
 * Chest CT scan
 * Chest x-ray

Treatment options
Your doctor may suggest treatment with glucocorticoids (such as prednisone) which are given along with other medicines that slow down the immune response.

Medicines used to treat Wegener's granulomatosis include:


 * Azathioprine (Imuran)
 * Cyclophosphamide (Cytoxan)
 * Methotrexate

These medicines may cause serious side effects. You should carefully discuss your treatment plan with your doctor.

Other medicines may be prescribed, including:


 * Bisphosphonate (Fosamax) to prevent bone loss caused by prednisone
 * Folic acid or folinic acid (leucovorin) -- if you are taking methotrexate
 * Trimethoprim/sulfamethoxazole to prevent lung infections

Where to find medical care for Wegener's granulomatosis?
Directions to Hospitals Treating Wegener’s granulomatosis

What to expect (Outlook/Prognosis)?
Without treatment, people with this disease can die within a few months.

With treatment, the outlook for most patients is good. Studies have shown that most people who receive corticosteroids and cyclophosphamide get much better.

However, the disease may return in about half of all patients. In these cases, the disease usually comes back within 2 years of stopping treatment.

Possible complications
Complications usually result from lack of treatment. Patients with Wegener's granulomatosis develop sores (lesions) in the respiratory tract and the kidneys. Kidney lesions cause glomerulonephritis, which may result in blood in the urine and kidney failure. Kidney disease can quickly get worse. Kidney function may not improve even when the condition is controlled by medicines.

If untreated, kidney failure and death occur in more than 90% of patients.

Other complications may include:


 * Eye swelling
 * Lung failure
 * Nasal septum perforation (hole inside the nose)
 * Side effects from medicines used to treat the disease