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Wegener's granulomatosis

Wegener's granulomatosis (VEG-eh-nerz gran-u-loe-muh-TOE-sis) is an uncommon disorder that causes inflammation of your blood vessels. This inflammation restricts blood flow to various organs.

Wegener's granulomatosis, which is also called granulomatosis with polyangiitis (GPA), often affects your kidneys, lungs and upper respiratory tract. The restricted blood flow to these organs can damage them. Wegener's can affect other organs, but this isn't as common, and generally isn't as serious.

Wegener's granulomatosis also produces a type of inflammatory tissue known as a granuloma that's found around the blood vessels. Granulomas can destroy normal tissue. There is no known cause for Wegener's granulomatosis.

Early diagnosis and treatment of Wegener's granulomatosis may lead to a full recovery. Without treatment, Wegener's granulomatosis can be fatal, most commonly from kidney failure.

Symptoms Causes Risk factors Complications

Signs and symptoms of Wegener's granulomatosis may develop suddenly or over several months.

The first Wegener's granulomatosis symptoms usually involve areas of your respiratory tract, such as your sinuses, throat or lungs. However, the condition often worsens rapidly, affecting blood vessels and the organs they supply, such as the kidneys. Signs and symptoms may include:

  • Constantly runny nose, with pus-filled discharge
  • Nosebleeds
  • Sinus pain and inflammation (sinusitis)
  • Ear infections
  • Cough
  • Chest pain
  • Coughing up blood (hemoptysis)
  • Shortness of breath
  • General ill feeling (malaise)
  • Unintended weight loss
  • Joint aches and swelling
  • Blood in urine (hematuria)
  • Skin sores
  • Eye redness, burning or pain
  • Fever

For some people, the disease is limited to the lungs and doesn't involve the kidneys. Kidney involvement usually doesn't cause symptoms early in the course of the disease, and may be detected only by blood and urine tests. However, in time kidney failure and anemia often occur.

When to see a doctor

See your doctor if you have a persistently runny nose that doesn't respond to over-the-counter (OTC) cold medicines, especially if it's accompanied by nosebleeds and pus-like material, coughing up blood, or other possible signs and symptoms of Wegener's granulomatosis. Because this disease can get worse quickly, early diagnosis is critical to receiving effective treatment.

The cause of Wegener's granulomatosis is unknown. It appears to develop after an initial inflammation-causing event 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). The triggering event may be an infection, but no specific infection has been identified as the cause.

Wegener's granulomatosis can begin at any age, but it most often starts in middle age, with an average onset between 40 and 65. Whites are more likely than blacks to develop Wegener's granulomatosis.

Besides affecting your upper and lower respiratory tracts, Wegener's granulomatosis may affect other organs, including your skin, eyes, ears, kidneys, spinal cord and heart. Complications may include:

  • Hearing loss. Inflammation of 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 a heart attack, though this is rare.
  • 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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