Clinical manifestations of Granulomatosis with polyangiitis

Upper respiratory tract:

perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis.

Lower respiratory tract:

hemoptysis, cough, dyspnea.

Renal:

hematuria, red cell casts.

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Constitutional: fever, fatigue, malaise, anorexia, weight loss

Respiratory (90%):

Upper:

recurrent sinusitis, rhinitis, oral/nasal ulcers, nasal crusting, saddle-nose deformity, otitis, hearing loss, subglottic stenosis

Lower:

infiltrates, nodules, & hemorrhage → cough, dyspnea, hemoptysis, pleurisy

Skin (50%):

palpable purpura, livedo reticularis

Renal (80%):

RPGN, microscopic hematuria (dysmorphic RBCs and casts)

Ocular (50%):

episcleritis, scleritis, uveitis, orbital granulomas → proptosis, corneal ulcer

Neuro:

cranial + peripheral neuropathies, mononeuritis multiplex. Heme: ↑ incidence DVT/PE (20×) when disease active (Ann Intern Med 2005;142:620)