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Granulomatosis with polyangiitis

Granulomatosis with polyangiitis, also known as GPA or Wegener's granulomatosis, is a rare life-threatening condition where blood vessel walls become inflamed and leak, affecting blood flow.

Granulomatosis with polyangiitis can affect the nasal sinuses, nose and trachea, lungs and kidneys.

Other organ systems that can be affected by the disease include the nervous system, ears, eyes, heart, skin and joints.

There are around 500 new cases of granulomatosis with polyangiitis in the UK each year.

What are the symptoms of granulomatosis with polyangiitis?

The symptoms and their severity vary among patients. General signs of the disease may include loss of appetite, weight loss, fever and fatigue.

Most patients first notice symptoms in the respiratory tract, including:

  • Persistent runny nose or the formation of nasal crusts and sores
  • Nasal or facial pain
  • Nosebleeds or unusual nasal discharge, caused by inflammation of the nose or sinuses
  • Cough that may include bloody phlegm caused by upper airway or lower airway (lung) inflammation
  • Chest discomfort
  • Middle ear inflammation, pain, or hearing loss
  • Voice change, wheezing, or shortness of breath caused by inflammation of the trachea

Other possible symptoms include:

How is granulomatosis with polyangiitis diagnosed?

Because granulomatosis with polyangiitis has symptoms similar to a number of other disorders, diagnosing it can be difficult.

Your doctor makes the diagnosis based on the combination of symptoms and results of physical examinations, laboratory tests and X-rays. Sometimes a biopsy (tissue sample) of affected tissue (skin, nose, sinus, lung, or kidney) is taken and analysed.

A positive blood test for anti-neutrophil cytoplasmic antibodies (ANCA) can support a suspected diagnosis of the disease. However, this blood test does not, by itself, prove the diagnosis of granulomatosis with polyangiitis. In addition, a negative test does not rule out the diagnosis of granulomatosis with polyangiitis.

How is granulomatosis with polyangiitis treated?

Because granulomatosis with polyangiitis is often a life-threatening disease, it is treated with a variety of powerful medications that have been shown to save lives.
Treatment usually includes corticosteroid medications such as prednisolone, and other immune system suppressing drugs such as cyclophosphamide, methotrexate, and azathioprine.

These drugs suppress the immune system and usually induce remission (complete absence of all signs of the disease). Improvement usually occurs within days to weeks. When the disease is in remission, dosage of these medications can be reduced. However, medication will be continued until a prolonged period of remission has been achieved at which point they may be stopped.

Because the medications used to treat granulomatosis with polyangiitis can have serious side effects, patients are monitored closely by their doctors. The dosage of medication is adjusted as needed throughout the course of treatment.

What is the outlook for people with granulomatosis with polyangiitis?

After treatment begins and improvement occurs, relapses of the disease may occur in half of all patients. Flare-ups may follow reductions in the doses of prednisolone or other drugs but can usually be controlled by increasing the doses of these medications.

Granulomatosis with polyangiitis is a very serious disease and its treatments are not without risk. However, the treatments are life-saving for almost everyone when the diagnosis is timely and appropriate medications are started.

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WebMD Medical Reference

Medically Reviewed by Dr Rob Hicks on July 27, 2016

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