Vasculidities

Question 1

Giant Cell Arteritis (Temporal Arteritis)

Answer 1

• granulomatous inflammation of the media
• damage to carotid artery
• blindness is severe complication
• require immediate glucocorticoid therapy
• sx are HA and jaw pain when chewing
• polymyalgia rheumatica (neck, torso, shoulder, or pelvic girdle pain) common
• in pts > 50 yrs
• IL6 is an important mediator of this reaction, also cell mediated response

Question 2

Polyarteritis Nodosa

Answer 2

Transmural inflammation with fibrinoid necrosis.
Assoiated with hepatitis B.
Lung tissue will likely be spared.
Small aneurysms strung like beads on rosary: rosary sign.
-affects small and medium vessels in many organs

Question 3

Henoch Schonlein Purpura

Answer 3

• most common childhood vasculitis
• IgA mediated type 3 (immune complex) hypersensitivity rxn that follows infection
• IgA nephropathy that follows mucosal infection
• Complexes deposit in small vessels resulting in vasculitis.
• Sx: triad of palpable purpura, arthralg it’s, and GI abdominal pain

Question 4

Kawasaki Disease

Answer 4

Vasculitis of medium sized arteries.
>5 days fever, conjunctivitis, cervical lymphadenopathy, and mucocutaneous involvement.
-Coronary artery aneurysms are serious complication.
-Think Asian, with red tongue, and conjunctival injection.
-kills young kids arteries
-strawberry tongue
-desquamation of finger tips (skin falls off)
-edema of hands and feet
-tx: high dose aspirin and IVIGi

Question 5

Thromboangiitis Obliterans (Buerger’s Disease)

Answer 5

• heavy cigarette smokers
• before age 35
• associated with hypersensitivity to intradermal injections of tobacco extracts
• segmental thrombosing vasculitis extends to contiguous veins and nerves, encasing them in fibrous tissue
• may lead to gangrene
• Raynauds often present
• tx: smoking cessation

Question 6

Large Vessel Vasculidities

Answer 6

• takayasu arteritis

- giant cell arteritis

Question 7

Medium Vessel Vasculidities

Answer 7

• kawasaki

- polyarteritis nodosa

Question 8

Takayasu Arteritis

Answer 8

• vessel wall thickening and variable inflammation, medial granulomas
• Asian females
• weak upper extremity pulses (pulseless dz)
• granulomatous thickening of aortic arch
• tx with steroids

Question 9

Small Vessel Vasculidities

Answer 9

• granulomatosis with polyangiitis (wegners)

- eosinophilic granulomatosis with polyangiitis (Churg Strauss)

Question 10

Granulomatosis with Polyangiitis

Answer 10

• Wegners
• granulomas of the lung, nose, and kindney
• PR3-ANCA (C-ANCA) present (anti proteinase 3)
• tx: cyclophosphamide and corticosteroids

Question 11

Eosinophilic Granulomatosis with Polyangiitis

Churg Strauss

Answer 11

• associated with asthma, neuropathy
• MPO ANCA (P-ANCA)
• eosinophilic
• inc. IgE

Question 12

Microscopic Polyangiitis

Answer 12

• necrotizing vasculitis involving lungs, kidneys, and skin
• pauci immune glomerulonephritis and palpable purpura
• no nasopharyngeal involvement
• no granulomas
• MPO-ANCA/pANCA
• tx: cyclophosphamide and corticosteroids

Question 13

Hyperplastic Arteriolosclerosis

Answer 13

• onion like concentric thickening of arteriolar walls

- in renal arterioles can cause malignant HTN by activating RAAS pathway

Question 14

Atherosclerotic plaques are most likely to develop where?

Answer 14

-large elastic arteris, particularly abdominal aorta

Question 15

Angtiodysplasia

Answer 15

• painless GI bleeding

- vascular malformation of gut