Wegener's Granulomatosis

Wegener's granulomatosis is an uncommon disease that begins as an inflamed mass in the mucous linings of the nose, throat or lungs. The condition can get worse and affect the blood vessels such as the small capillaries of the kidneys. Eventually, the inflammation can cause the tissues to begin to die.

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Symptoms

Wegener's granulomatosis may either begin suddenly and severely or come on more mildly and gradually. It can take years for the full range of the disease to develop.

A person usually goes to the doctor with symptoms of a chest condition, including:

  • A general feeling of not being well
  • Fever
  • Joint pain that comes and goes
  • Loss of appetite
  • Congestion of the nose and sinuses
  • Sores on the lining of the nose
  • Bloody runny nose
  • Cough, which causes blood to come up
  • Inflammation of the linings of the lungs which causes difficulty and pain when breathing
  • Inflammation of the middle ear, which is painful, can cause hearing loss or potential rupture the eardrum
  • Weight loss

Inflammation of the nose is what usually brings patients to a doctor first. At this stage, Wegener's granulomatosis can often be mistaken for chronic sinusitis. The mucous membrane that lines the nose looks red, has a raised grainy appearance and bleeds easily. Other symptoms may also appear including rough, bumpy skin rashes, eye problems including redness of the eye and blockages of the ducts between the eyes and the nose and inflammation of the cartilage of the outer ear.

Serious complications may also develop as a result of Wegener's granulomatosis, including heart attack, meningitis or inflamed masses of the central nervous system that don't heal. Eventually, the condition spreads to the blood vessels and causes complications such as fluid leaking into the lungs and inflammation of the tiny capillaries of the kidneys. This latter condition can cause rising blood pressure and blood leaking into the urine.

Causes and Risk Factors

It is not known what causes this disease. While it appears similar to an infection, no bacteria or virus has been identified as a cause. Changes in the cells caused by this disorder suggest that it may be caused a type of allergic reaction. It can affect persons of any age. It is twice as likely to affect men as women.

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Diagnosis

A doctor will do a physical examination and review the symptoms. He or she will take steps to rule out the presence of polyarteritis nodosalupuslymphoma or kidney disease.

In some cases, the disease involves only the lungs. If the disease is more generalized, it usually involves the kidneys. It may be necessary to take a sample of tissue from the kidneys to examine under a microscope (do a biopsy). This will help find out how much of the kidneys are involved. It may also be necessary to do a biopsy of a lesion in the lungs to identify the disease.

Some of the tests that your doctor may order include:

  • A urinalysis shows to check for protein or blood in the urine
  • Blood tests to check for anemia (a lack of iron), certain types of antibodies or bacteria that might be causing an infection. Certain antibodies are usually present if a patient has Wegener's granulomatosis.
  • Looking at a sample of sputum under a microscope. Sometimes the sputum of patients with Wegener's granulomatosis contains clusters of unusual, densely packed cells.
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Treatments

The outlook for persons who have been diagnosed with Wegener's granulomatosis has dramatically improved with the use of drugs that suppress the body's immune system.

The sooner the condition is diagnosed and treatment is started the better. The condition can move rapidly to kidney failure in its later stages, which can cause life-threatening complications.

Corticosteroids to reduce swelling of the blood vessels may also be given. Over two to three months, the dosage of corticosteroids is reduced until the drug can be stopped. Immune suppressing drugs may be needed for a year or more after all symptoms of the disease have gone away.

Sometimes, blood transfusions may be needed if there has been a great deal of blood loss.

Even when the disease is advanced and has involved the kidneys, recovery is possible. It may be necessary to have a kidney transplant if kidney failure occurs.

After many years of treatment, some persons with Wegener's granulomatosis develop bladder cancer.

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