Table 1.

Locations and forms of GPA presentation

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)