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Definition of Polyangiitis, granulomatosis

Polyangiitis, granulomatosis: An inflammatory disease of small arteries and veins (vasculitis) that classically involves vessels supplying the tissues of the lungs, nasal passages (sinuses), and kidneys.

Symptoms include fatigue, weight loss, fevers, shortness of breath, bloody sputum, joint pains, and sinus inflammation, sometimes with nasal ulcerations and bloody nasal discharge. Granulomatosis with polyangiitis most commonly affects young or middle-aged adults.

The diagnosis of granulomatosis with polyangiitis is confirmed by finding evidence of vasculitis and abnormal cellular formations called granulomas on biopsy of tissue involved by the inflammatory process.

Granulomatosis with polyangiitis is a serious disease. Without treatment, it can be fatal within months. Treatment is directed toward stopping the inflammation process by suppressing the immune system.

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Reviewed on 12/27/2018
References
Kelley and Firestein's Textbook of Rheumatology 10th edition. 2016.
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