Rare disorder characterized by inflammation of small- and medium-sized blood vessels (vasculitis) that results in damage to various organ systems of the body, most often the respiratory tract and kidneys
Aetiology
Autoimmune disorder of unknown aetiology
Characterised by a small vessel vasculitis and necrosis, usually limited to respiratory tract and kidneys
Rare, usually patients >40 years
Clinical presentation
Features depend on system(s) involved
ENT features: sinusitis, nasal crushing, epistaxis, mouth ulcers, sensorineural deafness, otitis media and deafness, 'saddle nose' (due to cartilage damage from ischaemia), subglottic inflammation
Investigations
Characterised by high frequency of anti-neutrophil cytoplasmic antibody levels - ANCA positive
cANCA - GPA
pANCA - microscopic polyangiitis
Management
Most cases require treatment with IV steroids and cyclophosphamide due to their aggressive disease course
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