vasculitis

Question 1

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

Answer 1

Takayasu arteritis

Question 2

poor pulses in extremities

young asian women: teens -20 yo

Answer 2

Takayasu arteritis

Question 3

most common form of vasculitis

Answer 3

giant cell arteritis (temporal arteritis)

Question 4

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

Answer 4

giant cell arteritis (temporal arteritis)

Question 5

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

Answer 5

giant cell arteritis (temporal arteritis)

Question 6

claudication in legs

Answer 6

peripheral vascular disease

pain with exercise due to ischemia to muscles

Question 7

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

Answer 7

giant cell arteritis (temporal arteritis)

Question 8

diagnosis of giant cell arteritis (temporal arteritis)

Answer 8

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

Question 9

treatment of giant cell arteritis (temporal arteritis)

Answer 9

high dose steroids for several mos

prednisone for 1 mo →taper dose

Question 10

medium-sized vessel

Answer 10

Kawasaki disease

Question 11

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)

Answer 11

Kawasaki disease

Question 12

desquamation of hands + feet

Answer 12

Kawasaki disease

Question 13

complication of Kawasaki disease

Answer 13

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

Question 14

treatment of Kawasaki disease

Answer 14

IV immunoglobulin (IVIG): suppress immune system
high dose aspirin: prevent thrombosis of CA aneurysms (usually not given to children due to Reye but exception)

Question 15

small + medium-sized arteries + veins
40 yo male HEAVY SMOKER 
intermittent claudication → gangrene → autoamputation of digits
superficial nodular phlebitis
cold insensitivity

Answer 15

thromboangiitis obliterans (Buerger disease)

Question 16

treatment of thromboangiitis obliterans (Buerger disease)

Answer 16

stop smoking and vasculitis goes away

Question 17

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!!!

Answer 17

polyarteritis nodosa (PAN)

Question 18

(-) p-ANCA

Answer 18

polyarteritis nodosa (PAN)

Question 19

treatment of polyarteritis nodosa (PAN)

Answer 19

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

Question 20

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

Answer 20

henoch-schonlein purpura (IgA vasculitis)

Question 21

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)

Answer 21

henoch-schonlein purpura (IgA vasculitis)

Question 22

PALPABLE purpura

Answer 22

a vascultis

Question 23

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

Answer 23

eosinophilic granulomatosis with polyangiitis (churg-strauss syndrome)

Question 24

vasculitis in young asthmatic

Answer 24

eosinophilic granulomatosis with polyangiitis (churg-strauss syndrome)

Question 25

+ p-ANCA

Answer 25

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

Question 26

``` 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) ```

Answer 26

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

Question 27

diseases that affect LUNG + KIDNEY

Answer 27

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

Question 28

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

Answer 28

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

Question 29

+ c-ANCA

Answer 29

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

Question 30

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

Answer 30

corticosteroids + cyclophosphamide (like polyarteritis nadosa)

Question 31

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

Answer 31

microscopic polyangiitis