Overview

Granulomatosis with polyangiitis is an uncommon disorder that causes inflammation of the blood vessels in your nose, sinuses, throat, lungs and kidneys.

Formerly called Wegener's granulomatosis, this condition is one of a group of blood vessel disorders called vasculitis. It slows blood flow to some of your organs. The affected tissues can develop areas of inflammation called granulomas, which can affect how these organs work.

Early diagnosis and treatment of granulomatosis with polyangiitis might lead to a full recovery. Without treatment, the condition can be fatal.

Symptoms

Signs and symptoms of granulomatosis with polyangiitis can develop suddenly or over several months. The first warning signs usually involve your sinuses, throat or lungs. The condition often worsens rapidly, affecting blood vessels and the organs they supply, such as the kidneys.

Signs and symptoms of granulomatosis with polyangiitis might include:

  • Pus-like drainage with crusts from your nose, stuffiness, sinus infections and nosebleeds
  • Coughing, sometimes with bloody phlegm
  • Shortness of breath or wheezing
  • Fever
  • Fatigue
  • Joint pain
  • Numbness in your limbs, fingers or toes
  • Weight loss
  • Blood in your urine
  • Skin sores, bruising or rashes
  • Eye redness, burning or pain, and vision problems
  • Ear inflammation and hearing problems

For some people, the disease affects only the lungs. When the kidneys are affected, blood and urine tests can detect the problem. Without treatment, kidney or lung failure can occur.

When to see a doctor

See your doctor if you have a runny nose that doesn't respond to over-the-counter cold medicines, especially if it's accompanied by nosebleeds and pus-like material, coughing up blood, or other warning signs of granulomatosis with polyangiitis. Because this disease can worsen quickly, early diagnosis is key to getting effective treatment.

Causes

The cause of granulomatosis with polyangiitis isn't known. It's not contagious, and there's no evidence that it's inherited.

The condition can lead to inflamed, narrowed blood vessels and harmful inflammatory tissue masses (granulomas). Granulomas can destroy normal tissue, and narrowed blood vessels reduce the amount of blood and oxygen that reaches your body's tissues and organs.

Risk factors

Granulomatosis with polyangiitis can occur at any age. It most often affects people between the ages of 40 and 65.

Complications

Besides affecting your nose, sinuses, throat, lungs and kidneys, granulomatosis with polyangiitis can affect your skin, eyes, ears, heart and other organs. Complications might include:

  • Hearing loss
  • Skin scarring
  • Kidney damage
  • A loss of height in the bridge of the nose (saddling) caused by weakened cartilage
  • A blood clot forming in one or more deep veins, usually in your leg

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References
  1. AskMayoExpert. Granulomatosis with polyangiitis. Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2018.
  2. Granulomatosis with polyangiitis (Wegener's). American College of Rheumatology. http://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Granulomatosis-with-Polyangitis-Wegners. Accessed Nov. 20, 2018.
  3. Granulomatosis with polyangiitis (Wegener's). Vasculitis Foundation. https://www.vasculitisfoundation.org/education/granulomatosis-with-polyangiitis-gpa-wegeners/. Accessed Nov. 20, 2018,
  4. Falk RJ, et al. Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis. https://www.uptodate.com/contents/search. Accessed Nov. 20, 2018.
  5. Wojciechowska J, et al. Granulomatosis with polyangiitis in otolaryngologist practice: A review of current knowledge. Clinical and Experimental Otolaryngology. 2016;9:8.