Vasculitis
Michael P. Federle, MD, FACR
Amir A. Borhani, MD
Key Facts
Terminology
Diverse group of diseases characterized by inflammation and necrosis of blood vessels
Classified based on type of vessels predominantly involved
Large-vessel vasculitides
Takayasu arteritis, giant cell arteritis
Medium-sized vessel vasculitides
Polyarteritis nodosa (PAN), Kawasaki disease
Small-vessel vasculitides
Wegener granulomatosis, Churg-Strauss disease, microscopic polyangiitis, Henoch-Schönlein purpura, lupus vasculitis, Behçet disease
Imaging
Takayasu arteritis
Stenosis of aorta and its main branches
PAN
Multiple aneurysms in renal and mesenteric arteries
HSP
Bowel wall thickening and luminal narrowing; purpuric rash
Wegener granulomatosis
Renal and perirenal hemorrhage are common
Lupus vasculitis
Hypercoagulable state; bowel and renal ischemic injury
Behçet disease
Deep ulceration of bowel with danger of perforation
Diagnostic Checklist
CT findings that look like bowel or renal ischemia in young person should raise concern for vasculitis
TERMINOLOGY
Definitions
Diverse group of diseases characterized by inflammation and necrosis of blood vessels
Takayasu arteritis: Vasculitis affecting aorta and its main branches
Polyarteritis nodosa (PAN): Fibrinoid necrotizing vasculitis involving small and medium-sized vessels
Henoch-Schönlein purpura (HSP): Hypersensitivityrelated acute vasculitis affecting small vessels
Wegener granulomatosis: Granulomatous vasculitis of respiratory tract and kidneys
Lupus vasculitis: Necrotizing vasculitis affecting small vessels
Behçet disease: Necrotizing vasculitis of small vessels affecting multiple organs
IMAGING
General Features
Best diagnostic clue
Takayasu arteritis
Irregularity and stenosis of aorta &/or its main branches on angiography
PAN
Multiple aneurysms in renal and gastrointestinal (GI) arteries
HSP
Multisegmental bowel wall thickening and luminal narrowing on CT
Location
Takayasu arteritis
Typically involves aortic arch
Also involves remainder of aorta in 32% of cases
Only involves descending thoracic and abdominal aorta in 12% of cases
Polyarteritis (PAN)
At bifurcations of medium-sized arteries
Renal (80-90%) and mesenteric arteries (50-70%) are most commonly affected
Other locations: Liver, spleen, and pancreas
HSP
Mesenteric small vessels involved in 60% of cases
Wegener granulomatosis
Kidneys are involved in 80% of cases
May involve any part of GI tract
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