Symptoms and Signs of Granulomatosis with Polyangiitis

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Medically Reviewed on 1/22/2022

Doctor's Notes on Granulomatosis with Polyangiitis Symptoms, Treatment, and Life Expectancy

Granulomatosis with polyangiitis (also termed Wegener's) is a rare disease that results in inflamed blood vessels (vasculitis or polyangiitis) and nodules of inflammatory cells (granulomas) in affected tissues. The most common signs and symptoms are

Often, people can develop shortness of breath, chest pain and coughs with some bloody sputum. Less common signs and symptoms include

Urine may be reddish or bloody. Eye involvement can show redness, burning, and eye swelling; if double vision or decreased vision occurs, it is a medical emergency.

Granulomatosis with polyangiitis is rare. Its cause is unknown but thought to be an autoimmune disorder. Most signs and symptoms are due to organ damage that happens when its blood supply is compromised by the disease.

What Are the Treatments for Granulomatosis with Polyangiitis (Also Termed Wegener's)?

The treatments for granulomatosis with polyangiitis vary with the severity of the disease. Early diagnosis and appropriate treatment may allow you to recover within a few months although relapses may occur. There are two major therapeutic methods:

  • Medications (immune system suppression and inflammation reduction of blood vessels)
  • Plasmapheresis removes plasma, the liquid part of the blood, that contains compounds that cause the disease and substitutes it with disease-free plasma.

Some of the medications may be used together while some may be used to prevent relapses. Your doctors can design treatments that address your specific problems.

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Kasper, D.L., et al., eds. Harrison's Principles of Internal Medicine, 19th Ed. United States: McGraw-Hill Education, 2015.