Granulomatosis with polyangiitis
Anti-neutrophil cytoplasmic antibodies (ANCA) – Associated vasculitides
Note that this was formerly called Wegener's granulomatosis, but this nomenclature is being phased out due to Wegener's associations with the Nazi party.
Characterized by necrotic granulomatous inflammation; granulomas comprise giant cells, plasma cells, lymphocytes,
neutrophils, and eosinophils.
The respiratory tract is typically involved, which can lead to sinusitis, otitis media, rhinorrhea, and epistaxis; the mucosa can become granular and crusted.
– Erosion and destruction of the nasal septum can case the nasal bridge to collapse.
– Destruction of the tracheobronchial tree can lead to stenosis and airway obstruction.
– When the lungs are involved, patients can develop a cough, difficulty breathing, hemoptysis, and hemorrhaging.
The kidneys are also commonly affected, leading to necrotizing crescent focal
glomerulonephritis and thrombosis.
Cutaneous manifestations vary, and include palpable purpura, livedo reticularis, ulcers, and the formation of deep, tender nodules.
The eyes, nervous system, heart, and musculoskeletal system can also be involved.
Granulomatosis with polyangiitis most commonly occurs in Caucasians; the average age of onset is 40 years.
