Vasculitis - Darrow Flashcards
vasculitis
immune complex deposition**
activation of complement**
leukocytoclstic changes - remnants of WBC lying around
get necrosis and thrombosis
classifiction of vasculitis
based on vessel size
large vessel
limb claudication
absent pulses
bruits
aortic dilation
medium vessel
livedo reticularis
cutaneous nodules
arthritis
small vessel
purpura - small spots
mononeuritis multiplex
medium vessel disease**
behcets
variable vessel vasculitis
-hits small, medium, and large vessels
takayasu arteritis
large vessel
giant cell arteritis
large vessel
polyarteritis nodosa
medium vessel
kawasaki disease
medium vessel
cogans syndrome
IgG4 related disease
single organ vasculitis
only involve one organ
ANCA vasculitis
small vessel
immune complex
small vessel
ischemic necrosis
in vasculitis
palpable purpura
presentation of vasculitis syndrome
cutaneous manifestation**
inflammatory palpable purpura
leukocytoclastic vasculitis
tx of vasculitis
start with steroids
-to bigger guns eventually
large vessel vasculitis
takayasu arteritis
temporal arteritis
granulomatous or giant cell arteritis
temporal arteritis
fever, malaise, weight loss, easy fatigue, amaurosis, BP difference arms, aortic insufficiency, right brachial index 0.4
women most likely has?
takayasus arteritis
- early inflammatory and late occlusive phase
- aka pulseless disease**
- oriental disease
EQ**
fever of undetermined origin
temporal arteritis
pain, stiff shoulders and hips, pain with shewing, sed rate elevated (very high)
temporal arteritis
may have subclavian stenosis**
subclavian stenosis
with temporal arteritis
polymyalgia rheumatica**
aches pains in shoulder, neck, hips
- stiff in morning
- limited ROM
check for temporal arteritis
proximal muscle pain and stiffness with no weakness
giant cell (temporal) arteritis
giant cell temporal arteritis
can have increased alk phosphate
-because hepatic artery obstruction
fever, arthralgia, abdominal pain, left wrist drop and right foot drop, hematuria, hep B positive, medium vessel aneurysm
polyarteritis nodosa**
wrist and foot drop
medium vessel
microaneurysms
polyarteritis nodosa
polyarteritis nodosa
HTN livedo reticualris abdominal pain testicle pain/tender** foot drop or polyneuropathy
livedo reticularis
PAN
cryoglobulinemia
anti-phospholipid Ab syndrome
polyarteritis nodosum association
hepatitis B
-not so much anymore - because of vaccine
but its on boards
fever 5 days, erythema and edema palms and soles, cervical adenopathy
kawasakis**
fever 5 days cervical lymphadenopathy strawberry tongue conjunctivitis rash acral - palm and sole edema
strawberry tongue
kawasakis
buergers disease
smokers
askanazi jews
not on test
immune complex small vessel vasculitis
hypocomplementic urticarial vasculitis (anti-C1q vasculitis)
henoch schonlein purpura
mixed cryoglobulinemia
anti-GBM
urticarial vasculitis
anti-complement vasculitis
get urticaria >24 hours**
C3a and C5a - produce leukocytoclastic nuclear debris
ecchymosis after it clears up**
urticarial vasculitis
with lupus** - high association
anti-CD20
rituximab
18yo viral prodrome, fever, palpable skin lesions
IgA immunoglobulin
-henoch scholnien purpura
IgA
henoch schonlein purpura
-activates alternative complement pathway
henoch schonlein purpura
small vessel disease - palpable purpura
IgA immune complexes
GI sx - gut has lots of IgA
hematuria, raynauds, hepatosplenomegaly, sat on cold bleacher
cryoglobulinemia
-cold exposure**
should evaluate for hepatitis C and sjogrens
mixed cryoglobulinemia
hep C and sjogrens associations
polyclonal mixed IgG-IgM complexes
anti-glomerular BM disease
goodpastures
ANCA associated small vessel vasculitis
wegeners granulomatosis
microscopic polyangiitis
churg-strauss disease
eosinophilic granulomatosis with polyangiitis
churg strauss disease
granulomatosis with polyangiitis
wegeners granulomatosis
anti-PR3
C-ANCA - granulomatosis with polyangiitis
cytoplasm distribution
anti-MPO
P-ANCA - churg strauss and microscopic polyangiitis
around nucleus distribution
ANCAs
activate neutrophils
-activate alternate complement pathway
granulomatous rxn to extravascular necrosis
microscopic polyangiitis
no granulomas
P-ANCA positive, pauci-immune nongranulomatous necrotizing vasculitiz of arterioles, hematuria and RBC casts, coughing up blood, pulmonary infiltration
microscopic polyangitis
hemoptysis, stridor, prior DVT, conductive hearing loss, C-ANCA, granulomatous inflammation
conductive hearing loss - upper respiratory
granulomatous polyagiitis - wegeners
eosinophilia
churg strauss
iranian patient, blurry vision, oral and penile pain, hx of pathergy
behcets disease
oral lesions, genital lesions, ocular involvement
behcets disease
behcets
multi vessel vasculitis
HLA-B51
behcet syndrome
strep sanguis and heat shock protein
behcet syndrome
pain in right knee, abdominal pain, fever, rub over right chest, rebound tenderness, red raised lesion, CRP elevated, proteinuria
familial mediterranean fever
familial mediterranean fever
get a cytokine storm
-IL-1 beta
get lots of inflammation
lack of pyrin
normally inactivates IL8 and C5a
familial mediterranean fever
recurrent fever and hot ankle rash
familial mediterranean fever
string of beads
single organ vasculitis
skin - cutaneous leukocytoclastic vasculitis - only have palpable purpura
drug reaction
tx of SLE
arthritis and dermatitis**
NSAIDs
corticosteroids
hydroxychloroquine - prevents flares
get a flare - tx with corticosteroids**
