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Table 1 Locations and forms of GPA presentation

From: Granulomatosis with polyangiitis (Wegener’s) as a necrotizing gingivitis mimic: a case report

Site

Presentations

Frequency of disease occurrence at site throughout disease course (%)

Upper airway[1],[2]

Oral or nasal ulcers, strawberry gums, strawberry tongue, unresponsive upper respiratory tract or ear ‘infections’ , otitis, vestibular nerve injury, conductive or sensorineural deafness

92

Lower airways[1],[2]

Subglottic stenosis, pulmonary nodular infiltrates, pulmonary hemorrhage ‘ground-glass’ infiltrations, pleurisy

85

Kidneys[1],[3]

Rapidly progressing glomerulonephritis - necrotizing

80

Musculoskeletal[1, 2]

Myalgias, migratory pauciarticular or polyarticular arthritis, arthralgias

67

Eye[1],[2],[4]

Scleritis, episcleritis, orbital pseudotumor, proptosis, dacrocystis

52

Skin[1],[2]

Palpable purpura, ulcers, leucocytoclastic vasculitis, subcutaneous nodules, gangrene

46

Nervous system[1],[2]

Peripheral neuropathy, mononeuritis multiplex, cranial neuropathy, meningitis

20

Additional rare presentations[2],[3]

Myocarditis, pericarditis, cerebral vasculitis, mass lesions (brain, renal, lung, orbit, breast, prostate, ovary, parotid)