Upper respiratory, pulmonary and renal disease + constitutional symptoms
White, older patients
Constitutional symptoms: Fever, malaise, weight loss
Upper airway: Serous otitis media, hearing loss, sinusitis, nasal mucosal ulcerations, septal perforation, epistaxis, laryngotracheal disease
Subglottic stenosis is most common laryngotracheal lesion (16% patients)
Lower respiratory: Cough, dyspnea, pleuritis, hemoptysis, diffuse alveolar hemorrhage
Renal failure, glomerulonephritis
Ophthalmologic: scleritis, episcleritis, uveitis
Cutaneous: Palpable purpura, nodules, ulcers
Neurologic: Mononeuropathy and polyneuropathy, cerebral vasculitis, cerebral hemorrhage or thrombosis
Cardiac: Pericarditis, myocarditis
Subglottic stenosis is most common laryngotracheal lesion (16% patients)
Granulomatosis with polyangiitis
Polyarteritis nodosa, Eosinophilic granulomatosis with polyangiitis 
Polyarteritis nodosa
Lymphoma, lung cancer
Thromboangiitis obliterans
Pneumonia, infective endocarditis, HUS
Eosinophilic granulomatosis with polyangiitis
Glomerulonephritis