Vascular Pathology: Vasculitis Flashcards
what are three layers of the arterial wall
endothelial intima
smooth muscle media
connective tissue adventitia
What is the etiology of vasculitis
unknown
not infectious
what are 2 broad categories of clinical symptoms for vasulitis
- nonspecific symptoms of inflammation (fever, fatigue, weight loss, myalgias)
- symptoms of organ ischemia
what can cause symptoms of organ ischemia
- luminal narrowing
2. thrombosis of inflamed vessels
large-vessel vasculitis involves what
aorta and its major branches
medium-vessel vasculitis involves
muscular arteries that supply organs
small-vessel vasculitis involves
arterioles, capillaries and venules
Name 2 large-vessel vasculitis
- Temporal (Giant Cell) Arteritis
2. Takayasu Arteritis
Temporal ( Giant Cell ) Arteritis is commonly found in who
older adults greater than 50
female
clinical symptom for temporal ( giant cell) arteritis and where the vasculitis has to occur for that symptom
headache ( temporal artery)
visual disturbances (ophthalmic artery)
jaw claudication
flu-like with joint and muscle pain (polymyalgia rheumatica)
What would be abnormal lab values for temporal ( giant cell) arteritis
elevated ESR greater than 100
what do ESR values mean?
erythrocyte sedimentation rate
red blood cells sediment in a period of one hour
hematology test
non-specific measure of inflammation
what will the biopsy show for temporal (giant cell )arteritis
inflamed vessel wall with giant cells and intimal fibrosis
segmental
-must do biopsy of long segmnet of vessel
how is temporal (giant cell) arteritis treated
corticosteroids
- need immediate treatment
- high risk of blindness without treatment
Where does takayasu arteritis occur
aortic arch at branch points
Takayasu arteritis occurs in who
adults less than 50
young asian females
clinical symptoms of takayasu arteritis
visual and neurological symptoms with weak or absent pulse in upper extremity (Pulseless disease)
what are abnormal lab values for Takayasu
ESR elevated
treatment for Takayasu
corticosteroids
what is the difference between Temporal (giant cell) arteritis and Takayasu
Temporal: branches of carotid artery
Takayasu: aortic arch at branch points
temporal: greater 50 years
Takayasu: less 50 years, asian
Name 3 medium-vessel vasculitis
- polyarteritis nodosa
- Kawasaki disease
- Buerger disease
Polyarteritis Nodosa is what
- Necrotizing vasculitis involving multiple organs
- spares lungs
Polyarteritis nodosa commonly presents in who
young adults
what are clinical symptoms for polyarteritis nodosa
hypertension ( renal artery )
abdominal pain with melena ( mesenteric artery)
neurological disturbances
skin lesions
melena
dark sticky feces containing partly digested blood
what are abnormal values associated with polyarteritis nodosa
associated with serum HBsAg
hepatitis B surface antigen
How do lesions present in polyarteritis nodosa
- varying stages present at once
- Early: transmural inflammation with fibrinoid necrosis
- early then heals making ‘string-of-pearls’ appearance
treatment for polyarteritis nodosa
corticosteroids and cyclophosphamide
- fatal if not treated
Kawasaki disease classically affects what group of people
asian children less than 4 yrs old
how does Kawasaki present
non-specific signs: fever, conjunctivitis, erythematous rash of palms and soles, enlarged cervical lymph node
what part of the body is commonly involved with Kawasaki disease? what does this lead to
coronary artery
- thrombosis with myocardial infarction
- aneurysm with rupture
what is the treatment for Kawasaki
aspirin and IVIG
disease is self-limited
what is the caution with giving aspirin with an asian child presenting with Kawasaki
clinical features of Kawasaki similar to viral disease
- not suppose to give children with viral infection aspirin
- Reye syndrome
What is Buerger disease
necrotizing vasculitis involving digits
how does Buerger disease clinically present
- ulceration, gangrene, autoamputation of fingers and toes
- Raynaud phenomenon is often present
Buerger disease is associated with what habit?
heavy smoking
what is treatment for Buerger disease
smoking cessation
What are the small-vessel vasculitis
- Wegener Granulomatosis
- Microscopic Polyangiitis
- Churg-Strauss Syndrome
- Henoch-Schonlein Purpura
What is Wegener Granulomatosis
necrotizing granulomatous vasculitis involving nasopharynx, lungs, and kidneys
what is the classical presentation of Wegener Granulomatosis
middle-aged male
- sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, hematuria due to rapidly progressive glomerulonephritis
what lab values go with Wegener Granulomatosis
c-ANCA levels
what does biopsy reveal of Wegener Granulomatosis
large necrotizing granulomas with adjacent necrotizing vasculitis
what is the treatment for Wegener Granulomatosis
Cyclophosphamide and steroids
-relapses are common
what is microscopic polyangiits
necrotizing vasculitis involving multiple organs, especially lungs and kindey
clinical presentation of Microscopic polyangiitis
similar to Wegener granulomatosis,
but nasopharyngeal involvement and granulomas are absent
what are lab values for microscopic polyangiitis
p-ANCA
treatment for microscopic polyangiitis
corticosteroids and cyclophosphamide
relapses are common
What is Churg-Strauss syndrome
Necrotizing granulomatous inflammation with eosinophils involving multiple organs, especially lungs and heart
what is often in people who have Churg-Strauss syndrome
-Asthma and peripheral eosinophilia
what are lab values for Churg-Strauss syndrome
p-ANCA
What is Henoch-Schonlein Purpura
vasculitis due to IgA immune complex deposition
Henoch-Schonlein Purpura is a vasculitis most common found in who
children
how does Henoch-Schonlein Purpura clinically present
palapble purpura on buttocks and legs, GI pain and bleeding and hematura ( IgA nephropathy)
Henoch-Schonlein Purpura is usually occurs after what infection
upper respiratory tract infection
- because IgA production increased
treatment for Henoch-Schonlein Purpura
steroids if severe
- self-limited but may recur
