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Path Vasculitis
KYCOM Block 13
125
Pathology
Graduate
01/14/2014

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Term
define vasculitis
Definition
inflammation of and damage to walls of blood vessels
Term
what vessels does vasculitis affect, what vessels are in each category
Definition
large (elastic): aorta, aortic brnaches, pulmonary arteries

medium (muscular): small branches of aorta (coronary and renal A)

small arteries and arterioles
Term
what are 5 common infections that cause vasculitis
Definition
bacterial: neisseria
rickettsial: RMSF
spirochetal: syphilis
fungal: aspergullosis
viral: HSV
Term
what are 3 ways the immune system causes vasculitis
Definition
immune complexes
antineutrophil cytoplasmic antibody (ANCA) mediated
direct antibody mediated
Term
what are 3 diseases that use immune complexes to causes vasculitis
Definition
henoch schonlein purpura
SLE
RA
Term
what are 3 diseases that use ANCA to cause vasculitis
Definition
wegener granulomatosis
microscopic polyangiitis
churg strauss syndrome
Term
what are two diseases that use antibodies to cause vasculitis, what are the names of the antibodies
Definition
kawasaki disease: anti-endothelial
goodpasture syndrome: anti-glomerular basement membrane
Term
what are three diseases that use the immune system to cause vasculitis but we dont know how they do it
Definition
giant cell (temporal arteritis)
takaysu arteries
polyarteritis nodosa
Term
what are 4 general signs in someone who has vasculitis
Definition
fever, malaise, arthragias, weight loss
Term
what 2 vasculitis syndromes like large vessels
Definition
temporal arteritis
takayasu
Term
what 2 vasculitis syndromes like medium vessels
Definition
PAN
kaesakai
Term
what vasculitis syndromes like small vessels
Definition
everything else
ANCA associated vasculitis
Term
what is ANCA
Definition
anti-neutrophil cytoplasmic antibodu

antibodies to proteins inside neutrophil. body becomes sensitized to them because there is something that causes the protein to be expressed and move to the surface of cells
Term
what things can cause proteins that ANCA attacks to move to the cell surface
Definition
drugs, TNF
Term
what are the two types of ANCA, what do they attack
Definition
perinuclear (p): antibodies to myeloperoxidase

cytoplasmic (C): antibodies to protenase (PR) 3 in the cytoplasm
Term
what can the presence of ANCA diagnose
Definition
it cant.
it only helps diagnose.
negative does not RO disease
positive does not RI disease
Term
what are commonalities in people who develop ANCA (3)
Definition
respiratory tract infection
older
caucasian
Term
what three vasculitis are due to ANCA, which ANCA
Definition
wegerners- cytoplasmic
microscopic polyangiitis- perinuclear
churg struss syndrome - perinuclear
Term
clinical abnormalities that suggest vasculitis (7)
Definition
palpable purpura
pulmonary infiltrates
microscopic hematuria
chronic inflammatory sinusitis
mononeuritis multiplex
unexplained ischemic events
glomerulonephritis with evidence of multisystem disease
Term
why is it essential you RO infection as a cause to vasculitis
Definition
immunosupressive treatment is often used
Term
what are infectious conditions that can mimic vasculitis (8)
Definition
bacterial endocarditis
disseminated gonoccal infection
pulmonary histoplasmosis
coccidioidomycosis
syphilis
lyme disease
RMSF
whipple's disease
Term
when do you angiogram or biopsy for vasculitis
Definition
when you have STRONG evidence
Term
what is the most common form of vasculitis
Definition
giant cell (temporal) arteritis
Term
who gets giant cell arteritis
Definition
female > male
elderly, rare <50yo
Term
where is giant cell arteritis (6)
Definition
small and medium arteries
cranial arteries: temporal (headache), facial, opthalamic (blindness)
aortic arch (rare)
Term
what are the early symptoms of giant cell arteritis (3)
Definition
fever, malaise, weight loss
Term
what are the symptoms of temporal arterieis (6)
Definition
weak pulse in UE

occular: blurred vision and blindness due to opthalamic A

systemic HTN due to renal A narrowing

throbbing headache (temporal A)

artery is very tender
Term
what are the temporal arteritis lab findings (1)
Definition
elevated ESR due to immune mediated inflammation - very non-specific
Term
what do the arteries look like in temporal arteritis (4)
Definition
nodular thickening

granulomatous inflammation

fragmentation of internal elastic lamina (due to inflammation)

T cell dependent disease
Term
how is temporal arteritis diagnosed (1)
Definition
biopsy of temporal artery
classic presentation
Term
how is temporal artritis treated
Definition
corticosteroids
Term
what is the prognosis of temporal arteritis
Definition
treated: excellent
untreated: blindness
Term
which condition is very similar to takayasu arteritis
Definition
giant cell/temporal arteritis
Term
who gets takaysau arteritis (3)
Definition
women
15-45yo
asian
Term
where is takaysau arteritis (4)
Definition
large to medium sized arteries
aortic arch
major aortic branches
Term
what are early signs of takaysau arteritis (3)
Definition
fever, weight loss, malaise
Term
what are signs of takaysau arteritis (6)
Definition
loss of pulse in UE
ocular disturbances
retinal hemorrhage
neurological abnormalities
HTN from renal A stenosis
stroke from carotid A occlusion
Term
what does the artery look like in takaysau arteritis takaysau arteritis (2)
Definition
granulomatous vasculitis
fibrous thickening of artery wall
Term
how is takaysau arteritis diagnosed (2)
Definition
angiographi
MRI
Term
what is the treatment for takaysau arteritis (2)
Definition
prednisone for symptoms
surgical correction of stenosed vessels
Term
what is the prognosis of takaysau arteritis (2)
Definition
variable
chronic and relapsing
Term
who gets polyarteritis nodosa (PAN) (3)
Definition
young adults
male > female
30% have HepB antigen
Term
where is polyarteritis nodosa (PAN) (3)
Definition
small to medium arteries
SYSTEMIC vasculitis EXCEPT pulmonary capillaries or glomeruli
Term
what arteries is polyarteritis nodosa (PAN) most frequently in (most to least (9))
Definition
kidney
heart
liver
GI
pancreas
testis
skeletal muscle
nervous system
skin
Term
what are the common initial symptoms of polyarteritis nodosa (PAN) (2)
Definition
sudden elevation in BP due to kidney involvement

abdominal pain and melena (blood in stool)
Term
what are the symptoms of polyarteritis nodosa (PAN) (8)
Definition
fever
malaise
weight loss
hematuria
renal failure
HTN: rapid onsetmuscular aches and pains
peripherial neuritis (mononeuritis)
livedo reticularis
Term
what is livedo reticularis
Definition
skin lesion in dependent areas in polyarteritis nodosa (PAN)
Term
what are the lab findings in polyarteritis nodosa (PAN) (4)
Definition
disturbances in BUN and CR
elevated ESR
low complement
Term
what does the artery look like in polyarteritis nodosa (PAN) (4)
Definition
segmental necrotizing vasculitis
thrombosis
infarction
aneurysms
Term
how is polyarteritis nodosa (PAN) diagnosed (2)
Definition
arterial biopsy (hopefully from livedo reticularis skin lesion)
angiography
Term
how is polyarteritis nodosa (PAN) treated (2)
Definition
corticosteroids: decrease inflammation

cyclophosphamide
Term
how does cyclophosphamide work
Definition
chemotherapeutic drug that targets cells replicating rapidly like marrow which knocks down immune system helping in vasculitis
Term
what is the prognosis of polyarteritis nodosa (PAN)
Definition
treated 90% long term remission
untreated is fatal
Term
who gets kawasaki disease (4)
Definition
kids <4yo
japan, hawaii, main US
Term
where is kawasaki disease (4)
Definition
large, medium, small arteries
70% in coronary artery
antibody attacks endothelium which is in ALL vessels
Term
signs of kawasaki disease (9)
Definition
fever
lymphadenopathy
conjunctivitis: bilateral, no exudate
erythema/cracking of lips
strawberry tongue
erythema and erosions of oral mucosa
edema of hands and feet
desqumation of fingertips
polmorphous exanthema
Term
what are the lab findings in kawasaki disease (2)
Definition
elevated ESR
leukocytosis
Term
what do the vessels look like in kawasaki disease (3)
Definition
necrotizing vasculitis
anti-endothelial antibodies
aneurysms
Term
how is kawasaki disease officially diagnosed (6)
Definition
fever for 5d+ and 4 of the following...

1. acute changes in extremities: erythema/edema of hands/feet

2. convalescent changes in extremities: membranous desquamation of fingertips

3. polmorphous exanthema: rash with many sizes and shapes

4. bilateral conjunctival infection without exudates (blood shot eyes)

5. erythema and cracking of lips/strawberry tongue
cervical lymphadenopathy >1.5cm
Term
treatment of kawasaki disease (2)
Definition
aspirin
IV immunoglobulins
Term
prognosis of kawasaki disease (3)
Definition
treated: coronary artery disease risk 4%
untreated: coronary artery disease risk 20%
may resolve spontaneously
Term
who gets microscopic polyangiitis (MPA) (2)
Definition
40-60yo avg 57yo
male > females
Term
where is microscopic polyangiitis (MPA)
Definition
small, medium, capillaries, venules
skin, mucous membranes, lungs, brain, heart, GI tract, kidneys, muscle
Term
what are the signs of microscopic polyangiitis (MPA) (12)
Definition
fever
weight loss
myalgias
acute
GLOMERULONEPHRITIS common
hemoptysis
hematuria
proteinuria
GI pain or bleeding
palpable purpura
mononeuritis: can progress to involve multiple sites
Term
how is microscopic polyangiitis (MPA) diagnosed (4)
Definition
p-ANCA in 75%
elevated ESR
leukocytosis
thrombocytosis
Term
what do the arteries look like in microscopic polyangiitis (MPA) (3)
Definition
fibrnoid necrosis of media
leykocytoclastic angiitis
RBC casts in urine: their close and stick thgother in damaged glomeruli
Term
how is microscopic polyangiitis (MPA) diagnosed (1)
Definition
biopsy
Term
how is microscopic polyangiitis (MPA) treated (1)
Definition
remove inciting agents: drug antigens, microorganisms, tumors
Term
what is the prognosis of microscopic polyangiitis (MPA) (4)
Definition
treated: 75% 5y survival
prednisone
daily cyclophosphamide if life threatning
35% have disease relapse
Term
what needs to be excluded when diagnosing kawasaki disease (9)
Definition
staph infection: SSSS, TSS
strap infection: scarlet fever
measles, other viral exanthems
leptospirosis
rickettsial disease
SJS, drug reaction
juvenile RA
Term
who gets henoch-schonlein pupura (2)
Definition
usually kids 4-7yo
usually follows respiratory infection
Term
where is henoch-schonlein pupura
Definition
small vessels
Term
what are the signs of henoch-schonlein pupura (8)
Definition
palpable purpura (butt and lower extremities)
arthralgias
GI: colicky abdominal pain, nausea, vomiting, diarrhea, constipation, blood mucous in stool

glomerulonephritis- rare
Term
what are the lab signs of henoch-schonlein pupura (3)
Definition
mild leukocytosis
necrotizing vasculitis
IgA deposition in lesions detectable by flourescence microscopy
Term
how is henoch-schonlein pupura officially diagnosed (3)
Definition
rash
GI complaints or hematuria
arthritis
Term
henoch-schonlein pupura treatmetn
Definition
prednisone alleviates symptoms
Term
henoch-schonlein pupura prognosis
Definition
mortality rare (1-5% due to end stage renal disease)
10-40% disease reocurrance
Term
who gets wegner granulomatosis (2)
Definition
rare
male > female
40-60yo
Term
where is wegner granulomatosis (5)
Definition
small and medium arteries
lower lung and upper respiratory
kidneys
Term
what are the signs of wegner granulomatosis (4)
Definition
bilateral pneumonia
chronic sinusitis
nasopharyngeal ulcerations
evidence of renal disease
Term
what are the lab findings in wegner granulomatosis
Definition
cytoplasmic ANCA
Term
what do the vessels look like in wegner granulomatosis (3)
Definition
acute necrotizing granulomas of upper respiratory tract
necrotizing granulomas vaculitis
renal disease: crescentic glomerulonephritis
Term
how is wegner granulomatosis diagnosed
Definition
arterial biopsy: evidence of necrotizing granulomatous lesions in vessels
Term
how is wegner granulomatosis treated (2)
Definition
cyclophosphamide
corticosteroids
Term
what is the prognosis of wegner granulomatosis
Definition
treated: 99% long term remission
untreated: 80% 1 year mortality
Term
who gets buerger disease (thromboangiitis obliterans)(7)
Definition
usuallu <35 yo
male > female
heavy cigarette smoke damages vessels
most common in isreal, india, japan, south america
Term
where is buerger disease (thromboangiitis obliterans)(5)
Definition
small and medium arteries
tibial and radial arteries
extends into veins and nerves
Term
signs of buerger disease (thromboangiitis obliterans)(4)
Definition
superificial nodular phlebitis
raynaud phenomenon
instep claudication
chronic ulceration of extremities (gangrene)
Term
what are the lab findings of buerger disease (thromboangiitis obliterans)(1)
Definition
negative for acute phase reactants
Term
what do the vessels look like in buerger disease (thromboangiitis obliterans)(4)
Definition
granulomatous inflammation
thrombosos if lumen
microabscesses
arteries, veins, nerves encased by fibrous tissue
Term
how is buerger disease (thromboangiitis obliterans)diagnosed (2)
Definition
exclude other diseases
angiography
Term
how is buerger disease (thromboangiitis obliterans)treated (1)
Definition
smoking cesation
Term
what is the prognosis of buerger disease (thromboangiitis obliterans)
Definition
amputation
Term
who gets chrug-strauss syndrome (3)
Definition
any age usually 15-70yo, avg 48yo
vasculitis develops within 3y of asthma onset
Term
where is chrug-strauss syndrome (8)
Definition
small and medium arteries
lung involvement predominates
skin, CV, kidney, PNS, GI tract
Term
signs of chrug-strauss syndrome (6)
Definition
fever
weight loss
malaise
severe asthmatic attacks
pulmonary infiltrates
72% exhibit mononeuritis multiplex
51% have skin lesions (purpura, nodules)
Term
what are the lab findings for chrug-strauss syndrome (3)
Definition
eosinophilis >1000 cells/ul or >10%
elevated ESR
p-ANCA in 48%
Term
what do the vessels look like in chrug-strauss syndrome (2)
Definition
granulomatous inflammation
infiltration of tissues with eosinophils
Term
how is chrug-strauss syndrome diagnosed (4)
Definition
biopsy
asthma
peripherial eosinophilia
clinical features
Term
how is chrug-strauss syndrome treated
Definition
glucocorticoids
Term
what is the prognosis of chrug-strauss syndrome (3)
Definition
treated: 75% 5y survival
untreated: 25% 5y survival
mycardial involvement most frequent cause of death
Term
what are the types of cryoglobulinemia (2)
Definition
type 1: lymphoproliferative diseases, chronic infections

type II: drug users (usually with hep C infection)
Term
where is cryoglobulinemia (2)
Definition
small arteries and veins
Term
what are the signs of cryoglobulinemia 1 (1)
Definition
hyperviscosity syndrome
Term
what are the signs of cryoblobulinemia 2 (6)
Definition
mild recurrent bouts of LE purpura
glomerulonephritis, arthralgia, distal necrosis, abd pain, peripherial neuropathy
Term
what are the lab signs of cryoglobulinemia (4)
Definition
cryoglobulins: percipitate at cold temp so lesions show in hands and feet
rheumatoid factor
hypocompletenemia (esp C4)
usually hepC positive
Term
what do the vessels look like in cryoglobulinemia (2)
Definition
leukocytoclastic vasculitis
proliferative glomerulonephritis with IgM capillary loops
Term
how is cryoglobulinemia diagnosed (7)
Definition
cutaneous vasculitis
hypocomplemtemeia
elevated liver enzymes
hepatomeagly
caput medusa
cirosis
cryoglobulinemia
Term
treatment of cryoglobulinemia (3)
Definition
mild to moderate: interferon a if hepC
severe: immunosupressive therapy (steroids) and or plasmapheresis
Term
who gets bechet's disease (3)
Definition
old silk route japan to mediterranean
mid 20-30s
genetic predisposition HLA-B51
Term
whereis bechet's disease
Definition
all arteries and veins
Term
signs of bechet's disease (9)
Definition
A/P uveitis, can cause blindness
aphthous ulcers in mouth

headaches, confusion, stroke due to white matter involvement and aseptic meninges

folliculitis

pulmonary artery aneurysm

arthralgias

GI ulcers- mouth and internall

genital ulcers
Term
lab findings for bechet's disease (1)
Definition
postive pathergy test is SUPPORTIVE: blank sterile needle makes prick and mark it, several days leter the site has palpable reaction to skin
Term
what do vessels look in in bechet's disease (2)
Definition
neutrophilc vascular reaction
+/- fibrinoid necrosis
Term
how is bechet's disease diagnosed (6)
Definition
biopsy if possible
supportive findings
recurrent for 3y
oral ulcerations
uveitis
skin lesions
Term
how is bechet's treated (3)
Definition
topical steroids if mucocutaneous
systemic corticosteroids or immunosupressents
Term
what is the prognosis of bechet's (3)
Definition
most relapse and remitt
poor if CNS involved
most common morbidity: blindness
Term
cause of cutaneous leukocytoclastic angiitis (hypersensitivity vasculitis)
Definition
7-21d after exposure to antigen (iusually drug, less commonly vaccination)

circulating immune complexes form after drug exposure
Term
what drugs trigger cutaneous leukocytoclastic angiitis (hypersensitivity vasculitis) (7)
Definition
anti-HTN
anti-convulsants
anti-thyroid
GF
antibiotics
NSAIDS
SSRI
Term
where is cutaneous leukocytoclastic angiitis (hypersensitivity vasculitis)
Definition
all vessels IN SKIN
Term
signs of cutaneous leukocytoclastic angiitis (hypersensitivity vasculitis) (3)
Definition
palpable purpure usually in LE and dependent areas

confined to skin

1-3 wks after starting a drug
Term
what do vessels look like in cutaneous leukocytoclastic angiitis (hypersensitivity vasculitis)
Definition
isolated cutaneous leukocytoclastic angiitis
Term
how is cutaneous leukocytoclastic angiitis (hypersensitivity vasculitis) diagnosed
Definition
skin lesions with hx of exposure to new agent
Term
how is cutaneous leukocytoclastic angiitis (hypersensitivity vasculitis) treated (2)
Definition
withdrawl of offending agent
corticosteroids if needed
Term
what is the prognosis of cutaneous leukocytoclastic angiitis (hypersensitivity vasculitis) (2)
Definition
complete recovery common
skin hyperpigmentation may me sequela
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