Lecture 13 Vasculitis Flashcards
Vasculitis is ____ of the blood vessel wall which can lead to _____ and _____
inflammation;
thrombus, fibrosis
Takayasu stereotypic patient:
Temporal/giant cell stereotypic patient:
asian females less than 40;
elderly females
2 large vessel vasculitis:
takayasu; temporal/giant cell
Takayasu classically causes _____ thickening and narrowing of the ____ ____ and proximal great vessels
granulomatous;
aortic arch
Takayasu is called ____ disease due to a blood pressure difference between the upper and lower extremities
pulseless
Treatment for both large vessel vasculitides:
high dose corticosteroids
Giant cell arteritis (GCA) typically affects branches of the ____ artery
carotid (ie temporal)
in GCA, temporal artery involvement causes ____;
opthalmic artery involvement can cause ____
headache, jaw claudication;
blindness/visual problems
GCA is associated with _______ _____, seen as flu-like symptoms, myalgia, and joint pain
polymyalgia rheumatica
The 2 large cell vasculitides present with _____ ESR
elevated
What kind of inflammation do GCA and takayasu show?
granulomatous
What are the 2 medium vessel vasculitides mentioned in the notes?
polyarteritis nodosum (PAN), kawasaki disease
What is the most commonly affected organ in PAN, and what are the characteristic findings?
Kidney–>hypertension, elevated BUN
If there is ischemia to the GI, symptoms such as ____ and ____ can occur
pain, melena
What is characteristically seropositive in PAN?>
HBSAg (hep b surface antigen)
treatment for PAN?
corticosteroids, cyclophosphamide
Kawasaki disease classically affects ____
asian kids less than 4 years
Most important artery that can be affected in kawasaki?
coronary artery
‘CRASH and burn’ symptoms associated with kawasaki:
Conjunctivitis, Rash, Adenopathy, Strawberry tongue (oral mucositis), Hand and foot rash, and Fever (high)
Treatment for kawasaki?
IV immunoglobulin and Aspirin
ANCA positive small vessel vasculitides?
microscopic polyangiitis, granulomatosis with polyangititis (wegener’s), eiosinophillic granulomatosis with polyangitis (churg strauss)
what immunoassay enzyme is associated with c-ANCA? what is the characteristic staining?
proteinase 3 (PR3); cytoplasmic
what immunoassay enzyme is associated with p-ANCA?; what is the characteristic staining?
myeloperoxidase (MPO); perinuclear
Memory hook for Wegener’s/granulomatosis with polyangiitis (GPA):
characterisitc areas affected:
“C” disease (from pathoma)
nasopharynx, lungs, kidneys
ANCA associated with GPA?
c-anca/PR3
remember C disease
treatment for GPA?
Corticosteroids and cyclophosphamide
remember C disease
Nasal involvement with GPA presents as _____
saddle nose
Microscopic polyangiitis (MPA) classically affects the ____ and _____
lungs, kidneys
(similar to GPA minus nasopharynx involvement
what ANCA is associated with MPA?
p-anca/myeloperoxidase
What kind of skin/renal infection does MPA cause?
“pauci-immune”
Cardinal features of eosinophillic granulomatosis with polyangiits (CS for churg strauss)? ____, ____, and lung disease
asthma, sinusitis
Churg strauss is associated with _____neuritis
mono (ie wrist drop)
3 elevated labs with churg strauss:
eiosinophillia, P-ANCA (MPO-ANCA), IgE
What can Churg strauss can be a complication of treatment with?
montelukast (singulair) or zafirlukast (accolate)
What lung symptom does antiglomerular basement membrane disease (goodpasture’s) often present with?
pulmonary hemorrhage
anti ______ ____ _____ antibodies should be demonstrated in diagnosis of goodpasture’s
glomerular basement membrane
_____ vasculitis is due to precipitation of blood proteins at temperatures lower than 37 degree C
cryoglobulinemic
Type 1 cryoglubulins are ______ while type 2 and 3 are _____
monoclonal; polyclonal
Type 1 cryoglobulinemia can cause _____ of blood in the brain, leading to neurologic symptoms
hyperviscosity
IgA associated vasculitis (Henoch Scholein Purpura) is characterized by what skin condition, and where is it located?
palpable purpura–lower extremities ie buttocks
What is Henoch Scholein Purprua secondary to?
IgA immune complex deposition
What is another name for palpable purpura?
leukocytic vasculitis
______ _____ vasculitis usually presents as uticaria that persists for up to 72 hours (more than normal hives)
hypocomplementic uticarial
What is seen in hypocomplementemic uticaria that is not seen in regular uticaria?
pain
Classic lab findings of hypocomplementmic uticaria?
decreased complements, positive ANA
pathology of hypocomplementimic uticaria?
leukocytic vasculitis
Classic presentation of behcet’s disease? ulceration of the ____ and ____
mouth and genitals o_o
the ____ test is when pustule like lesions appear 48 hours after a prick with a needle. it is positive in ____
pathergy; behcet’s disease
behcet’s is common along the _____ ____
silk road (ie east asia to mediterranean)
Classic symptoms of cogan’s syndrome: ____ ____ of the eyes and ______ dysfunction
interstitial keratitis; vestibuloauditory
what blood vessel is commonly associated with cogan’s vasculitis?
aorta
what is the classic appearance of drug associated immune complex vasculitis?
cutaneous leukocytoplastic angiitis (palpable purpura)
what is the name of a single organ vasculitis that presents as seizures, stroke, TIA, or other neurological defects?
primary CNS vasculitis
Characteristic CSF findings of primary CNS vasculitis:
_____ pleocytosis with oligoclonal bands;
elevated _____
lymphoctyic
protein
What is the angiographic pattern seen in CNS vasculitis?
alternating stenosis and dilations (beading pattern)
what is commonly mistaken for primary CNS vasculitis?
reversible cerebral vasoconstriction syndrome (RCVS)
what is the presenting symptom most commonly seen in RCVS?
hyperacute thunderclap headache
‘treatment’ of RCVS?
typically observation
What disease in usually seen in heavy male smokers
thromboangiitis obliterans (buerger’s disease)
what is the most common symptom of buerger’s disease?
digital finger ischemia (ie raynauds, gangrene, pain)
Most important aspect of treatment for buerger disease?
STOP SMOKING
