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.

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