Vasculitis

Question 1

Vasculitis

Answer 1

inflammation of blood vessels

Question 2

Vasculitis 2o/2

Answer 2

Infectious (Hep B/C, Endocarditis), Drug hypersensitivity, Connex Tissue disease (RA, SLE, Sjogren’s), Cryoglobulins, Malignancies

Question 3

Vasculitis ? S/S

Answer 3

skin lesions, constitutional (fever, anorexia, weight loss, fatigue, weakness), MSK (arthralgias, arthritis, myalgias, peripheral neuropathies)

Question 4

Vasculitis ? Lab

Answer 4

systemic inflamm (anemia of inflamm disease, thrombocytosis, low albumin, elevated ESR/CRP, polyclonal gammopathy); possible elevated liver enzyme, low complement levels, cryoglobulins

Question 5

Vasculitis ? Dx

Answer 5

Hx and Phys Ex (organ involvement); Serum Abs (RF, ANA, ANCA); organ biopsy or angiogram (pearls on a string); tapered occlusions; thrombosis

Question 6

Vasculitis ? Incidence

Answer 6

mean age of onset 5th decade; equal sex distribution (except F>M in Takayasu’s and giant cell arteritis)

Question 7

Vasculitis ? Pathology

Answer 7

both focal and segmental, may not involve entire circumference of vessel

Question 8

Vasculitis ? Pathology, Large/Med

Answer 8

panarteritis w/ infiltration; disruptions of elastic lamina; possible giant cell or granuloma

Question 9

Vasculitis ? Pathology, Small

Answer 9

fibrinoid necrosis of vessel wall w/ transmural inflamm; Ig and complement vascular deposition; leukocytoclastic vasculitis

Question 10

Vasculitis ? Pathophysiology

Answer 10

Immune complex, ANCA, Antiendothelial Abs, T-cell mediated endothelial cell injury, infex of endothelial cells

Question 11

Vasculitis ? Treatment

Answer 11

(1) Remove inciting agent or Ag; (2) Glucocorticoids, cyclophosphamide; (3) for ANCA-pos, plasmapheresis, rituximab

Question 12

leukocytoclastic vasculitis

Answer 12

necrotizing vasculitis w/ PMN nuclear debris

Question 13

Large-vessel vasculitis

Answer 13

Giant cell arteritis, Takayasu’s arteritis

Question 14

Giant cell arteritis ? aa

Answer 14

Temporal aa, vessels originating from aortic arch

Question 15

Giant cell arteritis ? S/S

Answer 15

temporal headache, jaw claudication, scalp tenderness, visual loss

Question 16

Giant cell arteritis ? pathology

Answer 16

giant cell arthritis w/ disruption of internal eleastic lamina

Question 17

Takayasu’s arteritis ? aa

Answer 17

aortic arch and its branches, any part of aorta

Question 18

Takayasu’s arteritis ? S/S

Answer 18

claudication of upper > lower extremities, CNS events

Question 19

Takayasu’s arteritis ? pathology

Answer 19

granulomatous panarteritis

Question 20

Medium-vessel vasculitis

Answer 20

Polyarteritis nodosa, Kawasaki’s disease

Question 21

Polyarteritis nodosa ? S/S

Answer 21

any organ, esp. skin, joints, peripheral nn, gut, kidney

Question 22

Polyarteritis nodosa ? pathology

Answer 22

focal but panmural necrotizing arteritis w/ tendency @ vessel bifurcation

Question 23

Kawasaki’s disease ? S/S

Answer 23

primary infants/young children, fever, mucocutaneous changes, cervical lymphadenopathy, rash/erythema/edema of hands and feet, desquamation, myocarditis, coronary vasculitis

Question 24

Kawasaki’s disease ? pathology

Answer 24

probable infectious vector resulting in cytokine-mediated endothelial damage

Question 25

Small-vessel vasculitis, ANCA-pos.

Answer 25

GPA (Wegener's), Churg-Strauss, MPA

Question 26

GPA (Wegener's)

Answer 26

Granulomatosis w/ polyangiitis

Question 27

GPA (Wegener's) ? S/S

Answer 27

Upper respiratory (sinuses), lungs, kidneys

Question 28

GPA (Wegener's) ? pathology

Answer 28

Pauci-immune, necrotizing, granulomatous arteritis; c-ANCA

Question 29

Chrug-Strauss

Answer 29

Eosinophilic granulomatosis w/ polyangiitis

Question 30

Chrug-Strauss ? S/S

Answer 30

asthma, eosinophilia, multiorgan (lungs, skin, peripheral nn, gut, heart, renal [rare])

Question 31

Chrug-Strauss ? pathology

Answer 31

necrotizing extravascular granukomas + vasculitis of small aa/vv

Question 32

MPA

Answer 32

Microscopic polyangiitis

Question 33

MPA ? S/S

Answer 33

pulmonary hemorrhage, glomerulonephritis, palpable purpura, peripheral neuropathy, joint/abdominal pain

Question 34

MPA ? pathology

Answer 34

Pauci-immune, necrotizing arteritis; p-ANCA

Question 35

Small-vessel vasculitis, ANCA-neg.

Answer 35

Henoch-Schoenlein Purpura (HSP), Essential Cryoglobulinemic Vasculitis, Cutaneous Leukocytoclastic angiitis

Question 36

HSP ? S/S

Answer 36

palpable purpura of LE, arthritis, abdominal pain, hematuria,

Question 37

HSP ? pathology

Answer 37

leukocytoclastic or necrotizing vasculitis w/ IgA deposition [often]

Question 38

Essential Cryoglobulinemic Vasculitis ? S/S

Answer 38

purpura, arthalgias, weakness, peripheral neuropathy, Raynaud's phenom, glomerulonephritis, pulmonry hemmorhage; often RF pos, Hep C Ab pos

Question 39

Essential Cryoglobulinemic Vasculitis ? pathology

Answer 39

cryoglobulin deposition in small vessels (glomerulocaps) stimulates complement activation, cellular inflamm response

Question 40

Cutaneous Leukocytoclastic angiitis ? S/S

Answer 40

palpable purpura, arthralgias, systemic symptoms possible, often 2o/2 immune response

Question 41

Cutaneous Leukocytoclastic angiitis ? pathology

Answer 41

leukocytoclastic vasculitis