vasculitis Flashcards

1
Q

large vessel
granulomatous inflammation of ARTERIES off of AORTIC arch →↓ blood flow to arms →↓ pulses in arms “pulseless disease”
↑ ESR

A

Takayasu arteritis

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2
Q

poor pulses in extremities

young asian women: teens -20 yo

A

Takayasu arteritis

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3
Q

most common form of vasculitis

A

giant cell arteritis (temporal arteritis)

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4
Q

large vessel
inflammation of branches of ECA (temporal arteries)
↑ ESR (screen if unilateral headache + jaw claudication - sensitive test)

A

giant cell arteritis (temporal arteritis)

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5
Q

elderly women
unilateral headache: temporal artery
jaw claudication: painful when chewing food, jaw muscles become ischemic with activity due to vasculitis
palpable, tender, prominent temporal artery
temporal wasting
later sx:
irreversible blindness: occlusion of opthalmic artery

A

giant cell arteritis (temporal arteritis)

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6
Q

claudication in legs

A

peripheral vascular disease

pain with exercise due to ischemia to muscles

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7
Q

associated with polymalgia rheumatica: pain + joint stiffness in neck, shoulder, hips (proximal joints)

A

giant cell arteritis (temporal arteritis)

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8
Q

diagnosis of giant cell arteritis (temporal arteritis)

A

temporal artery biopsy: multinucleated giant cells + granulomas in media + adventitia

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9
Q

treatment of giant cell arteritis (temporal arteritis)

A

high dose steroids for several mos

prednisone for 1 mo →taper dose

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10
Q

medium-sized vessel

A

Kawasaki disease

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11
Q

infant + young children
asian
CRASH + burn: fever for 5 or more days + 4 of following CRASH:
Conjunctivitis: bilateral, non-exudative, painless
Rash on trunk
Adenopathy of cervical lymph nodes
Strawberry tongue: beefy red+ course texture + diffuse erythema of mucous membranes (lips)
Hands + feet (erythema or desquamation)

A

Kawasaki disease

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12
Q

desquamation of hands + feet

A

Kawasaki disease

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13
Q

complication of Kawasaki disease

A

coronary aneurysms (be able to diagnose on angiogram)
rupture → lethal
or pool blood → thrombosis → MI

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14
Q

treatment of Kawasaki disease

A
IV immunoglobulin (IVIG): suppress immune system
high dose aspirin: prevent thrombosis of CA aneurysms (usually not given to children due to Reye but exception)
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15
Q
small + medium-sized arteries + veins
40 yo male HEAVY SMOKER 
intermittent claudication → gangrene → autoamputation of digits
superficial nodular phlebitis
cold insensitivity
A

thromboangiitis obliterans (Buerger disease)

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16
Q

treatment of thromboangiitis obliterans (Buerger disease)

A

stop smoking and vasculitis goes away

17
Q

medium-sized
immune complex inflammation of renal + visceral vessels (skin, GI tract, heart)
ab pain, melena, myalgia, neuro sx = vague
SPARES LUNGS
associated with HEP B/ HEP C!!!

A

polyarteritis nodosa (PAN)

18
Q

(-) p-ANCA

A

polyarteritis nodosa (PAN)

19
Q

treatment of polyarteritis nodosa (PAN)

A

corticosteroids (suppress immune system) +/- cylophosphamide (reduce need for high dose corticosteroids)

20
Q

small vessel
most common form of systemic vasculitis in childhood
self-limiting disease

A

henoch-schonlein purpura (IgA vasculitis)

21
Q
child with recent URI - 50% cases
PALPABLE purpura on butt + legs
arthralgia (knees)
ab pain (intestinal hemorrhage)
IgA nephropathy (Berger's disease): IgA immune complex in kidney (nephritis + hematuria)
A

henoch-schonlein purpura (IgA vasculitis)

22
Q

PALPABLE purpura

A

a vascultis

23
Q

small-vessel
granulomas (multinucleated giant cells) + eosinophils
sinusitis
skin lesions
peripheral neuropathy( foot drop, wrist drop)
associated with asthma

A

eosinophilic granulomatosis with polyangiitis (churg-strauss syndrome)

24
Q

vasculitis in young asthmatic

A

eosinophilic granulomatosis with polyangiitis (churg-strauss syndrome)

25
Q

+ p-ANCA

A

eosinophilic granulomatosis with polyangiitis (churg-strauss syndrome) or
microscopic polyangiitis

26
Q
small-vessel
triad:
focal necrotizing vasculitis
granulomas in lung (dyspnea,hemoptysis)  + UPPER airway (soft palate, oropharynx, nasal cavity, sinuses, collapsed bridge of nose = SADDLE NOSE)
glomerulonephritis (hematuria)
A

granulomatosis with polyangiitis (GPA) (wegener’s granulomatosis)

27
Q

diseases that affect LUNG + KIDNEY

A

1) granulomatosis with polyangiitis (GPA) (wegener’s granulomatosis) = lung + UPPER AIRWAY, +c-ANCA
2) Goodpasture syndrome
3) microscopic polyangiitis: +p-ANCA

28
Q

lung biopsy:
lymphocytes around vessel
macrophages fused to form granulomas (multinucleated giant cells)

A

granulomatosis with polyangiitis (GPA) (wegener’s granulomatosis)

29
Q

+ c-ANCA

A

granulomatosis with polyangiitis (GPA) (wegener’s granulomatosis)

30
Q

treatment of granulomatosis with polyangiitis (GPA) (wegener’s granulomatosis)

A

corticosteroids + cyclophosphamide (like polyarteritis nadosa)

31
Q

small-vessel
LUNG
KIDNEY: pauci-immune crescentic GN
+p-ANCA

A

microscopic polyangiitis