Granulomatosis with polyangiitis (GPA)

• Granulomatous inflammation affecting small and medium sized vessels in the upper and lower respiratory tract, eyes and/or kidneys (necrotising glomerulonephritis common)

Aetiology

• Autoimmune disorder of unknown aetiology
• More common in northern Europeans
• Slightly higher incidence in males (1.5:1)
• Typically age 35-55 years

Clinical features

• Constitutional symptoms and arthralgia common
• ENT: sinusitis, nasal crushing, epistaxis, mouth ulcers, sensorineural deafness, otitis media, 'saddle nose' (due to cartilage damage from ischaemia), subglottic inflammation
• Respiratory: cough, haemoptysis, pulmonary infiltrates, diffuse alveolar haemorrhage, cavitating nodules on CXR
• Occular: conjunctivitis, episcleritis, uveitis, optic nerve vasculitis, retinal artery occlusion, proptosis
• Cutaneous: palpable purpura, cutanous ulcers
• Renal: necrotising glomerulonephritis
• Nervous system: mononeuritis multiplex, sensorimotor polyneuropathy, cranial nerve palsies

Eosinophilic granulomatosis with polyangiitis (EPGA)

• Eosinophilic granulomatous inflammation affecting small and medium sized vessels most commonly in the respiratory tract and skin, but can also affect the renal, cardiovascular, gastrointestinal, central and peripheral nervous system

Clinical features

• Many features similar to GPA, main difference is late onset asthma, high eosinophil count and ANCA specificity