Vasculitides

Question 1

Layers of vessels

Answer 1

Tunica Intima
Tunica Media
Tunica Adventitia

Question 2

Typical symptoms

Answer 2

Organ ischemia (via thrombosis and inflammation- healing/ fibrosis)

Question 3

Large vessel vasculitides

Answer 3

Affects Aorta/ Carotid

Giant Cell (Temporal)- most common (seen in females > 50)
Takayasu (Asian females < 50)

Question 4

Giant cell arteritis

Answer 4

Seen in females >50
Generally affects branches of carotid artery
Headache (temporal))
Visual disturbance
Jaw claudication
Polymyalgia rheumatica (flue like symptoms with myalgia)
Elevated ESR
Granulomatous vasculitis- inflamed vessel wall with giant cell and intimal fibrosis

Question 5

Biopsy of giant cell

Answer 5

Need to take a large sample, because it is “segmental” in the way it affects
Negative biopsy doesn’t exclude giant cell arteritis, because it may be affecting a different area

Question 6

Giant cell

Answer 6

Large cells with multiple nuclei

Question 7

Treatment for giant cell arteritis

Answer 7

Corticosteroids- immediately when suspected

Because of high risk of irreversible blindness

Question 8

Takayasu Arteritis

Answer 8

Similar to giant cell with a few exceptions:

• Adults <50 (typically young asian females)
• Affects aortic arch branch points (rather than more distal carotid artery)

Question 9

Takayasa arteritis- treatment

Answer 9

Also corticosteroids

think of Takayasu as part of the spectrum of giant cell arteritis

Question 10

Medium-vessel vasculitis

Answer 10

Affects muscular arteries (e.g. renal)
Polyarteritis Nodosa
Kawasaki
Buerger

Question 11

Polyarteritis Nodosa

Answer 11

Necrotizing vasculitis
Involves most organs (but not lung)
Presents in young adults (renal- HTN, mesenteric- abdominal pain, neuro probs, skin probs)
Generally HBsAg +

Question 12

Polyarteritis nodosa- histology

Answer 12

String of pearls appearance on imaging
Typically see transmural inflammation with fibrinoid necrosis (very pink on histology)- seen in vasculitis and malignant HTN
“Nodosa” because it appears like nodes after fibrosis occurs
Lesions are in different stages of healing (aneurysm- early lesions, fibrosis- late lesions)

Question 13

Polyarteritis nodosa- tx

Answer 13

Corticosteroids or cyclophosphamide

Fatal if not treated

Question 14

Kawasaki

Answer 14

Affects Asian children < 4
Looks like infection (fever, conjunctivitis, erythema in palms and soles, enlarged cervical lymph nodes)
Preferentially affects coronary arteries (can cause thrombosis and aneurysm)

Question 15

Kawasaki- Tx

Answer 15

Aspirin- prevent thrombus formation in coronary arteries (important to distinguish that they do not have a viral illness)
IVIG
Disease is self limited

Question 16

Buerger

Answer 16

Necrotizing- ulcerations and autoamputation
High association with smoking
Raynaud phenomenon seen in these patients

Question 17

Small vessel vasculitis

Answer 17

Affects arterioles, capillaries, and venules
Wegener granulomatosis
Microscopic polyangiitis
Churg-Strauss

Question 18

Wegener Granulomatosis “C”

Answer 18

Affects middle-aged mem
Involves nasopharynx, lungs, and kidneys
Patients present with rapidly progressing glomerulonephritis and hemoptysis
Necrotizing granulomas
c-ANCA positive
Cyclophosphamide & corticosteroids is the tx

Question 19

Microscopic Polyangiitis

Answer 19

Similar to Wegner, but do not see:
No nasopharyngeal involvement (only affects lung and kidneys)
No granulomas seen
See p-ANCA not c-ANCA
Tx is the same as Wegner (cyclophosphamide and corticosteroids)

Question 20

Churg-Strauss

Answer 20

p-ANCA +
Necrotixing granulomatous vasculitis with EOSINOPHILS
Typically have hx of asthma
Affects multiple organs: lungs and heart

Question 21

Henoch Schonlein Purpura

Answer 21

Most common vasculitis in kids
PALPABLE purpura on buttocks and leg (because of inflammation)
GI pain and bleeding
Hematuria (IgA nephropathy)
Usually occurs following an upper respiratory tract infection- because of excess production of IgA
Generally self-limited