What is the non-eponymous name for Wegener's granulomatosis?

Granulomatosis with polyangiitis

What is Granulomatosis with polyangiitis?

an autoimmune condition associated with a necrotizing granulomatous vasculitis, affecting both the upper and lower respiratory tract as well as the kidneys.

What organs are typically effected in Granulomatosis with polyangiitis?

• respiratory tract
• kidneys

What are the features of Granulomatosis with polyangiitis?

• 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

Which antibody is most related to Granulomatosis with polyangiitis?

cANCA positive in > 90%

What is seen in CXR in Granulomatosis with polyangiitis?

wide variety of presentations, including cavitating lesions

What is seen in renal biopsy in Granulomatosis with polyangiitis?

epithelial crescents in Bowman's capsule

What is the management of Granulomatosis with polyangiitis?

• steroids
• cyclophosphamide (90% response)
• plasma exchange

What is the typical length of survival for someone diagnosed with Granulomatosis with polyangiitis?

median survival = 8-9 years