Granulomatosis with polyangiitis (Wegener's granulomatosis)
Granulomatosis with polyangiitis is now the preferred term for Wegener's granulomatosis. It is an autoimmune condition associated with a necrotizing granulomatous vasculitis, affecting both the upper and lower respiratory tract as well as the kidneys.
Features
Investigations
Management
Features
- upper respiratory tract: epistaxis, sinusitis, nasal crusting
- lower respiratory tract: dyspnoea, haemoptysis
- rapidly progressive glomerulonephritis ('pauci-immune', 80% of patients)
- saddle-shape nose deformity
- also: vasculitic rash, eye involvement (e.g. proptosis), cranial nerve lesions
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Comparison of granulomatosis with polyangiitis and Churg-Strauss syndrome
Investigations
- cANCA positive in > 90%, pANCA positive in 25%
- chest x-ray: wide variety of presentations, including cavitating lesions
- renal biopsy: epithelial crescents in Bowman's capsule
Management
- steroids
- cyclophosphamide (90% response)
- plasma exchange
- median survival = 8-9 years
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| © Image used on license from Radiopaedia | |
Chest x-ray from a young male patient with granulomatosis with polyangiitis. Whilst the changes are subtle it demonstrates a number of ill-defined nodules the largest of which projects over the dome of the right hemidiaphragm. This nodule appears to have a central lucency suggesting cavitation
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| © Image used on license from Radiopaedia | |
CT of the same patient showing the changes in a much more obvious way, confirming the presence of at least 2 nodules, the larger of the two having a large central cavity and and air-fluid level