Wegeners Granulomatosis

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Wegeners Granulomatosis is an uncommon disorder that causes inflammation in blood vessels, which 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. Granulomas can destroy normal tissue.

Early diagnosis and treatment may lead to a full recovery. Without treatment, Wegener's granulomatosis can be fatal!

(Disturbing discoveries about Dr. Friedrich Wegener's controversial ties to the German Nazi regime of the 1930s have called into question the continued use of his name in identifying this condition, but an alternative has not been settled on.)

Signs and symptoms of Wegeners granulomatosis may develop suddenly or gradually.

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
  • Nosebleeds
  • Sinus pain and inflammation
  • Ear infections
  • Cough
  • Coughing up blood
  • Shortness of breath
  • General ill feeling
  • 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

  • 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.

    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.

    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 Wegeners 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 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 Wegeners 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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