Vasculitides Flashcards
Complication of giant cell (temporal) arteritis?
- Elderly female > 50
- Unilateral HA, jaw claudication, flu-like sx
BLINDNESS (ophthalmic artery occlusion)
What condition is giant cell (temporal) arteritis associated with?
Polymyalgia rheumatica
What artery is giant cell (temporal) arteritis associated with?
Branches of carotid –> temporal
giant cell (temporal) arteritis bx shows?
Focal granulomatous inflammation (segmental)
giant cell (temporal) arteritis labs?
Elevated ESR
giant cell (temporal) arteritis Rx?
Corticosteroids to prevent blindness
What do people call Takayasu arteritis?
- Asian females
“Pulseless DZ” - weak UE pulses
Takayasu arteritis bx?
Granulomatous thickening
What artery is involved with Takayasu arteritis?
Aortic arch and proximal great vessels
Takayasu arteritis labs?
Elevated ESR
Takayasu arteritis Rx?
Corticosteroids
What infection is polyarteritis nodosa associated with?
- Young adult male
- Fever, weight loss malaise, HA
- Abdominal pain, melena
- HTN, neuro dysfunction, cutaneous eruptions, renal damage
Hepatitis B
What vessels does polyarteritis nodosa involve?
Renal and visceral vessels
NOT pulmonary arteries (lungs are spared)
Pathogenesis of polyarteritis nodosa?
Immune complex mediated
polyarteritis nodosa bx?
Transmural inflammation of arterial wall with fibrinoid necrosis
- Different stages of inflammation in different vessels
polyarteritis nodosa arteriogram?
Innumerable renal microaneurysms and spasms
polyarteritis nodosa rx?
Corticosteroids, cyclophosphamide
Kawasaki Disease presentation?
Asian children
Complication of Kawasaki?
Coronary artery aneurysms
Kawasaki rx?
IV Ig and aspirin
Presentation of Buerger DZ?
Male
Complications of Buerger DZ?
- Gangrene, autoamputation of digits, superficial nodular phlebitis
Buerger DZ bx?
Segmental thrombosing vasculitis
Buerger DZ treatment?
Smoking cessation
Wegener’s Granulomatosis with polyangiitis presentation
Middle aged male
- URT: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis
- LRT: hemoptysis, cough, dyspnea
- Renal: hematuria, RBC casts
Wegener’s Granulomatosis with polyangiitis triad?
- Focal necrotizing vasculitis
- Necrotizing granulomas in the lung and upper airway (giant cells)
- Necrotizing GN
Wegener’s Granulomatosis with polyangiitis marker?
c-ANCA (PR3-ANCA) - antiproteinase 3 (neutrophil)
Wegener’s Granulomatosis with polyangiitis CXR?
Large nodular densities
Wegener’s Granulomatosis with polyangiitis Rx?
Cyclophosphamide, corticosteroids
Microscopic polyangiitis presentation?
Like Wegener’s but no URT involvement
Microscopic polyangiitis bx?
- Necrotizing vasculitis in lungs, kidneys, skin
- Pauci-immune GN
- Palpable purpura
- NO GRANULOMAS
Microscopic polyangiitis marker?
p-ANCA (MPO-ANCA) - anti-myeloperoxidase
Microscopic polyangiitis rx?
Cyclophosphamide, corticosteroids
Churg-Strauss (eosinophilic granulomatosis with polyangiitis) presentation?
Asthma, sinusitis, skin nodules/purpura, peripheral neuropathy (wrist/foot drop)
Churg-Strauss (eosinophilic granulomatosis with polyangiitis) bx?
Granulomatous, necrotizing vasculitis with eosinophilia
Churg-Strauss (eosinophilic granulomatosis with polyangiitis) marker?
p-ANCA, increased IgE
Henoch-Schonlein purpura presentation?
KIDS, follows URI
- Skin: palpable purpura on buttocks/legs
- Arthralgias
- GI: abdominal pain and bleeding
Henoch-Schonlein purpura pathogenesis?
IgA immune complex deposition
Type III HSN
What renal thingamajig is Henoch-Schonlein purpura associated with?
IgA Nephropathy (Berger DZ)