Vasculitides, Hypertensive diseases and hemodynamics Flashcards
what is vasculitis?
Inflammation attacking the wall of the vessel
examples of large vessels
aorta and its major branches and the analogous veins
examples of medium vessels
main visceral
examples of small vessels
intraparenchymal
T/F: all 3 major categories of vasculitis can affect any size artery
true
Small vessel vasculitis predominantly affects small vessels, but –
medium arteries and veins may be affected
all vessel types may be affected by –
variable vessel vasculities
large vessel vasculitis
Giant cell (temporal) arteritis (GCA)
Takayasu arteritis
medium vessel vasculitis
Kawasaki disease
Polyarteritis nodosa (PAN)
small vessel vasculitis
Microscopic polyangiitis (MPA)
Granulomatosis with polyangiitis (aka Wegener’s granulomatosis, GPA)
Churg-Strauss syndrome (CSS)
all size vessels vasculitis
Behçet’s disease
Most common systemic vasculitis in older adults (rare before 50 years of age)
giant cell/temporal arteritis
symptoms of giant cell arteritis
headache, polymyalgia rheumatic (stiffness), visual loss, pain with chewing (jaw claudication)
histology of giant cell arteritis
Chronic inflammation with multinucleated giant cells
don’t need giant cells to be diagnosed
gold standard for diagnosis of giant cell arteritis
temporal artery biopsy
another name for Takayasu arteritis
pulseless disease
Takayasu arteritis mostly affects –
women < 40 years old
what structure does the Takayasu arteritis usually involve?
aortic arch +/- great vessels
Takayasu arteritis: inflammation leads to –>
vessel wall thickening and stenosis
early symptoms of Takayasu arteritis
systemic phase: fever, malaise, arthralgia, myalgia
late occlusive phase of Takayasu arteritis causes symptoms due to –
ischemia (low O2 to tissues)
Takayasu arteritis is histologically similar to –
giant cell arteritis
– rarely affects arteries
immune complex small vessel vasculitis
who does polyarteritis nodosa affect?
(systemic vasculitis of) adults
what structures does polyarteritis nodosa involve?
small to medium arteries, typically renal, GI, skin
acute phase of polyarteritis nodosa
Segmental transmural necrotizing acute inflammation with fibrinoid necrosis and luminal thrombosis
chronic phase of polyarteritis nodosa
Fibrous thickening of vessel wall and aneurysm formation at branch points
stages of activity for polyarteritis nodosa
All stages of activity may coexist in same or different vessels
clinical manifestations of polyarteritis nodosa are due to –
distal organ ischemia/infarction
prevalence of Wegener granulomatosis
affects males > females
Wegener granulomatosis peak incidience in –
5th decade
histologic features of Wegener granulomatosis
“blue” necrosis Vasculitis of small to medium-sized vessels Granulomatous inflammation Eosinophils Neutrophilic microabscesses
what is present in 95% patients with active Wegener granulomatosis
C-ANCA (autoantibody)
Wegener granulomatosis often affects –
lung and kidney
symptoms of Wegener granulomatosis
Ulcerative/destructive lesions in sinonasal area and oral cavity
what is microscopic polyangiitis?
Necrotizing vasculitis affecting small arteries, arterioles, capillaries, and venules
what organs does microscopic polyangitiis affect?
kidney (90%), lung, skin, GI, nervous system,
ear/nose/throat (30%)
ENT symptoms of microscopic polyangitiis
mouth ulcers, epistaxis, sinusitis
ANCA-association of microscopic polyangiitis
P-ANCA/anti-MPO (70%)
what is not associated with microscopic polyangiitis?
giant cells, granulomas, immunoglobulin deposition