Treatment:

• Immunosuppression drugs
• Ig PLEX - Plasma exchange

Vessels involved: Small–medium vessels.

Hallmark pathology: Granulomatous inflammation + necrotizing vasculitis.

Organs:

• ENT/airway: chronic sinusitis, nasal septum perforation (“saddle nose”), otitis, subglottic stenosis.
• Lungs: cavitating nodules, alveolar hemorrhage.
• Kidneys: rapidly progressive GN (crescentic).

ANCA: Classically PR3-ANCA (c-ANCA).

Buzzwords: Sinus + lungs + kidneys.