Wegener's Granulomatosis


Wegener's granulomatosis is an uncommon disorder that causes inflammation in blood vessels (vasculitis), which restricts blood flow to various organs. Although the disease may involve any organ, most commonly it affects your kidneys, lungs and upper respiratory tract, including your sinuses, your nose and your windpipe (trachea). 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. Granulomas can destroy normal tissue.

Onset of Wegener's granulomatosis can occur at any age, but you're more likely to develop the condition between the ages of 30 and 50. Whites are most likely to develop Wegener's granulomatosis.

Signs and symptoms of Wegener's granulomatosis may develop suddenly or gradually. Early diagnosis and treatment may lead to a full recovery. Without treatment, this disease can be fatal.

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Signs and Symptoms

The first signs and symptoms of Wegener's granulomatosis usually involve your respiratory tract. However, the condition often worsens rapidly, affecting blood vessels and the organs they supply with blood throughout your body. Signs and symptoms may include:
    Constant runny nose (rhinorrhea)
    Nosebleeds
    Sinus pain and inflammation (sinusitis)
    Ear infections (otitis media)
    Cough
    Coughing up blood (hemoptysis)
    Shortness of breath (dyspnea)
    General ill feeling (malaise)
    Appetite loss
    Joint aches and swelling
    Skin sores
    Eye redness, burning or pain
    Double or decreased vision
    Fever
    Weakness and fatigue, possibly associated with low red blood cell count (anemia)
For some people, the disease limits itself to the respiratory tract and doesn't involve the kidneys. Kidney involvement usually doesn't cause symptoms, and may be detected only by blood and urine tests.

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Causes

Wegener's granulomatosis acts like an infection, but no one has found an infectious agent that causes the disease. It may involve an overactive immune system response to an allergic reaction. No one knows for sure.

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Screening and Diagnosis

Besides asking you about your signs and symptoms, conducting a physical exam and taking a medical history, your doctor may request several tests, including:
    Blood tests. When your immune system attacks your body's own cells, as happens with Wegener's granulomatosis, it forms proteins called autoantibodies. A blood test can detect certain autoantibodies in your blood that lend support to a diagnosis of Wegener's granulomatosis, but their presence alone doesn't confirm that you have the disease. Antineutrophil cytoplasmic autoantibodies (ANCA) appear in the blood of 80 percent to 95 percent of people with active Wegener's granulomatosis.

    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, 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.

    Urine tests. These evaluate kidney function to determine whether the disease is affecting your kidneys.

    Biopsy of affected tissue. The only sure way to confirm a diagnosis of Wegener's granulomatosis is to remove a small piece of tissue from an affected organ (biopsy) and examine it under a microscope. Your doctor may remove tissue from your nasal passages, airways or lungs to confirm or rule out the presence of both vasculitis and granulomas. Other areas for biopsy may include your skin or your kidneys. Some biopsies can be performed in an outpatient setting using a numbing medication (local anesthetic). Others, such as a lung biopsy, may require hospitalization.

    Chest X-ray. This test shows cavities or masses in your lungs. However, it can't distinguish between Wegener's granulomatosis and other lung diseases.
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Complications

Besides your upper and lower respiratory tracts, Wegener's granulomatosis may affect multiple organs, including your skin, eyes, ears, kidneys, spinal cord and heart. Without treatment, the disease is likely to be fatal. Complications may include:
  • Hearing loss. Middle ear infections or granulated tissue in the middle ear can lead to hearing loss.
  • Skin scarring. Sores may develop and may lead to severe scarring.
  • Heart attack. Wegener's granulomatosis can affect the arteries of your heart, resulting in chest pain or heart attack.
  • Kidney (renal) damage. As Wegener's granulomatosis progresses, it's likely to affect your kidneys. It can lead to glomerulonephritis, a type of kidney disease that hampers your kidneys' ability to remove waste and excess fluid from your body, leading to a buildup of waste products in your bloodstream (uremia). Kidney failure is a leading cause of death from Wegener's granulomatosis.
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Treatment

With early diagnosis and appropriate treatment, you may recover from Wegener's granulomatosis within a few months. In some cases, longer treatment may be necessary. Because the disease can recur, your doctor is likely to continue monitoring your condition after treatment.

Medications
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 reduce the body's immune reaction.

Also, doctors who are experienced in treating Wegener's are using some experimental drugs on people with Wegener's for whom standard treatments have not been effective. One such drug, rituximab (Rituxan), which reduces the number of a type of cell in your body (B cell) that's involved with inflammation, is showing promise. Rituxan was developed to treat a type of cancer known as non-Hodgkins lymphoma and has since been approved for the treatment of rheumatoid arthritis.

Because of potential side effects of the drugs used to treat Wegener's, such as hampering your body's ability to fight off infection, your doctor will monitor your condition while you're taking them. Drugs your doctor may prescribe to help prevent drug-related side effects include:
  • Medicine such as bisphosphonates (Fosamax) to prevent bone loss (osteoporosis) associated with prednisone use
  • Folic acid, a synthetic form of the B vitamin folate, to prevent more sores and other signs and symptoms associated with the depletion of folate in your body from methotrexate use
Surgery
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. A successful transplant depends on finding the organ that minimizes the chances your body will reject it and following a treatment regimen that will allow your body to accept the new organ.

Talk to your doctor to determine whether a kidney transplant might be an option for you. Your eligibility to receive a donated kidney will depend on how your other organs have been affected by the disease.

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Information obtained from National Institute of Health
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