A disease, occurring mainly in the fourth and fifth decades, characterized by necrotizing granulomas and ulceration of the upper respiratory tract, with purulent rhinorrhea, nasal obstruction, and sometimes with otorrhea, hemoptysis, pulmonary infiltration and cavitation, and fever; exophthalmos, involvement of the larynx and pharynx, and glomerulonephritis may occur; the underlying condition is a vasculitis affecting small vessels, and is possibly due to an immune disorder.
Alternative Name: Lymphomatoid granulomatosis.
Wegener's granulomatosis is an uncommon disorder that causes inflammation of your blood vessels, which in turn restricts blood flow to various organs. Most commonly Wegener's granulomatosis affects your kidneys, lungs and upper respiratory tract. The restricted blood flow to these organs can damage them.
Besides inflamed blood vessels, Wegener's granulomatosis produces a type of inflammatory tissue known as granuloma, found around the blood vessels. Granulomas can destroy normal tissue. What causes Wegener's granulomatosis is uncertain, but it's not an infection nor is it a type of cancer.
Signs and symptoms of Wegener's granulomatosis include:
Sinus pain and inflammation (sinusitis).
Eye redness, burning or pain.
Coughing up blood.
Shortness of breath.
General ill feeling (malaise).
Joint aches and swelling.
Double or decreased vision.
Weakness and fatigue, possibly associated with low red blood cell count (anemia).
Constantly runny nose.
Causes and Risk Factors:
The cause of Wegener's granulomatosis is unknown, but it appears to develop as a result of an initial inflammation-causing event that triggers an abnormal reaction from your immune system. The combination of these events can lead to inflamed, constricted blood vessels and harmful inflammatory tissue masses (granulomas).
Scientists speculate that the initial event may be an infection. This has been documented in nearly half the people with Wegener's, but the infections aren't overwhelming. Other possible contributing causes include an environmental toxin, a genetic predisposition to the condition or even a combination of all of these.
Tests and diagnosis:
Abnormal lab findings in patients with Wegener's granulomatosis 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 Wegener's granulomatosis 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 Wegener's granulomatosis.
Blood tests: If your doctor suspects Wegener's granulomatosis, he or she may order a blood test that can detect certain proteins (autoantibodies) in your blood called anti-neutrophil cytoplasmic autoantibodies (ANCA). These autoantibodies appear in the blood of 80 to 95 percent of people with active Wegener's granulomatosis. The presence of these autoantibodies lends support to a diagnosis of Wegener's granulomatosis, but isn't enough to confirm that you have the disease.
Your blood test also can measure your erythrocyte sedimentation rate — commonly referred to as sed rate. By measuring how quickly red blood cells fall to the bottom of a tube of your blood over a period of one hour, this test may indicate the level of inflammation in your body. Generally, red blood cells fall faster when inflammation is present. Another blood test can check for anemia, which is common in people with this disease. A blood test for creatinine will assess if your kidneys aren't properly filtering waste products from your blood.
Wegener's granulomatosis 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.
Your doctor may prescribe corticosteroids, such as prednisone, to treat the early signs and symptoms of Wegener's granulomatosis. For some people, a corticosteroid may be enough. However, most people require another immunosuppressive drug, such as cyclophosphamide (Cytoxan), azathioprine (Imuran) or methotrexate (Rheumatrex), to counteract the body's immune reaction.
If you've had kidney failure as a result of Wegener's granulomatosis, you may want to consider a kidney transplant to restore normal kidney function.
Early diagnosis and treatment of Wegener's granulomatosis may lead to a full recovery. Without treatment.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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