Cardio FA-Vasculitis

Question 1

what demographic does temporal (giant cell) arteritis typically affect

Answer 1

elderly females

Question 2

what are two common symptoms associated with temporal arteritis and what is one severe possible consequence of temporal arteritis

Answer 2

unilateral headaches (around the temporal artery) and jaw claudication are commonly seen; 
irreversible blindness can result from opthlamic artery occlusion

Question 3

what inflammatory disease is giant cell arteritis associated with

Answer 3

polymyalgia rheumatica

Question 4

what would you expect to see for gross pathology and histology for temporal (giant cell) arteritis

Answer 4

most commonly the branches of the carotid arteries are affected
on histology there is focal granulomatous inflammation

Question 5

how are temporal arteritis and Takayasu arteritis treated? what lab value is elevated?

Answer 5

corticosteroids;

elevated ESR

Question 6

what’s the typical demographic for Takayasu arteritis

Answer 6

young Asian females ( <40 years old)

Question 7

what symptoms are seen in Takayasu arteritis patients

Answer 7

weak upper extremity pulses, const. symptoms (fever, myalgia, night sweats), arthritis, skin nodules, ocular disturbances

Question 8

what kind of lesion or anatomical problem is caused by Takayasu arteritis

Answer 8

granulomatous thickening of the aortic arch and proximal great vessels

Question 9

in what age group does polyarteritis nodosa typically occur

Answer 9

young adults

Question 10

30% of patients with polyarteritis nodosa also have __________

Answer 10

Hepatitis B seropositivity

Question 11

what symptoms are seen in polyarteritis nodosa (besides the constitutional symptoms, which are common to all vasculitidies)

Answer 11

abdominal pain, melena, hypertension, neurologic dysfunction, cutaneous eruptions and renal damage

Question 12

what is a key pertinent negative for polyarteritis nodosa

Answer 12

no pulmonary involvement

Question 13

explain the pathophysiology of polyarteritis nodosa

Answer 13

immune complexes –> transmural inflammation of arterial walls with fibrinoid necrosis –> many microaneurysms and spasms

Question 14

what demographic is commonly affected by Kawasaki disease

Answer 14

Asian children < 4 years old

Question 15

what symptoms are commonly seen in Kawasaki disease

Answer 15

fever, cervical LAD, conjunctival injection, mucosal flushing (strawberry tongue), hand and foot erythema, desquamating rash

Question 16

what serious complications exist for children with Kawasaki disease

Answer 16

coronary artery aneurysm, CA aneurysm rupture or thrombosis –> MI

Question 17

what is the treatment for polyarteritis nodosa

Answer 17

corticosteroids and cyclophosphamide

Question 18

what is the treatment for Kawasaki disease

Answer 18

IVIG and aspirin

Question 19

in what demographic is Buerger disease (thromboangitis obliterans) usually seen

Answer 19

heavy smokers, males > 40 years old

Question 20

what symptoms are seen in Buerger disease

Answer 20

intermittent claudication (may lead to gangrene),
amputation of digits, superficial nodular phlebitis, Raynaud phenomenon

Question 21

what is seen on histology

Answer 21

segmental thrombosing vasculitis, often of the tibial and radial arteries

Question 22

how is thromboangitis obliterans treated

Answer 22

smoking cessation

Question 23

what is the triad of Granulomatosis with polyangitis (aka Wegener’s)

Answer 23

1. focal necrotizing vasculitis
2. necrotizing vasculitis in the lung and upper airway
3. necrotizing glomerulonephritis
   => vessel death, lung death, kidney death

Question 24

what symptoms are commonly seen in patients with granulomatosis with polyangitis

Answer 24

upper respiratory tract Sx: nasal septum perforation, chronic sinusitis, otitis media, mastoiditis
lower respiratory tract Sx: hemoptysis, cough, dyspnea
renal Sx: hematuria, red cell casts

Question 25

what results will antibody testing reveal for granulomatosis with polyangitis

Answer 25

c-ANCA (aka PR3-ANCA)

note: PR3= anti-proteinase 3

Question 26

what will imaging reveal for granulomatosis with polyangitis

Answer 26

large nodular densities

Question 27

how are granulomatosis with polyangitis and microscopic polyangitis treated

Answer 27

corticosteroids and cyclophosphamide

Question 28

name a few ways that microscopic polyangitis is different from granulomatosis with polyangitis

Answer 28

unlike Wegener’s, microscopic polyangitis does not involve the nasopharynx and does not cause granulomas
also, microscopic polyangitis is p-ANCA (MPO-ANCA) positive rather than c-ANCA in Wegener’s

Question 29

what’s a key way microscopic polyangitis can be differentiated from polyarteritis nodosa

Answer 29

polyarteritis nodosa has lesions in various stages of development while all lesions in microscopic polyangitis are in the same stage

Question 30

what symptoms are seen in Churg-Strauss syndrome

Answer 30

Sx: asthma, sinusitis, palpable purpura, peripheral neuropathy (foot/wrist drop)

Question 31

what organs are affected by Churg-Strauss syndrome

Answer 31

heart, GI, kidney (pauci-immune glomerulonephritis)

note: pauci-immune= little evidence of hypersensitivity

Question 32

what is seen on histology for Churg-Strauss syndrome

Answer 32

eosinophilila, granulomas, necrosis

Question 33

what is seen on labs for Churg-Strauss syndrome (eosinophilic granulomatosis with polyangitis

Answer 33

high IgE

p-ANCA (MPO-ANCA or myeloperoxidase-ANCA) positive

Question 34

what is the most common childhood vasculitis

Answer 34

Henoch-Schonlein purpura

Question 35

what is the classic triad for Henoch-Schonlein purpura

Answer 35

1. palpable purpura on buttocks or legs
2. arthralgias
3. abdominal pain, melena

Question 36

how is Henoch-Scholein purpura to microscopic polyangitis

Answer 36

both are small vessel vasculitidies in which the lesions are all the same age

Question 37

what antibody type is HSP associated with

Answer 37

IgA-mediated or IgA-triggered vasculitis

therefore, it is associated with IgA nephropathy