Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a rare form of vasculitis. In this disorder, small-sized blood vessels in the nose, sinuses, ears, lungs and kidneys become inflamed and damaged.
Criteria for the Classification of Wegener’s Granulomatosis (WG)
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Nasal or oral inflammation: Development of painful or painless oral ulcers or purulent or bloody nasal discharge
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Abnormal chest radiograph: Chest radiograph showing the presence of nodules, fixed infiltrates, or cavities
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Urinary sediment: Microhematuria (>5 red blood cells per high power field) or red cell casts in urine sediment
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Granulomatous inflammation on biopsy: Histologic changes showing granulomatous inflammation within the wall of an artery or in the perivascular or extravascular area (artery or arteriole)
For purposes of classification, a patient shall be said to have Wegener’s granulomatosis if at least 2 of these 4 criteria are present. The presence of any 2 or more criteria yields a sensitivity of 88.2% and a specificity of 92.0%
References:
- Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, Calabrese LH, Fries JF, Lie JT, Lightfoot RW Jr, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum. 1990 Aug;33(8):1101-7. [Medline]
Created: May 16, 2005
