Vasculitis I

Question 1

What is vasculitis and what is the mechanism?

Answer 1

vasculitis is an inflammation of blood vessels that causes damage to the vessel wall
may be from pathologic immune complex formation or from damage associated with antibodies against lysosomal enzymes

Question 2

How does endothelial inflammation contribute to vasculitis?

Answer 2

Endothelium does many things to perpetuate injury following activation by proinflammatory cytokines (TNF-a and IL-1)

1. Production of tissue factor: pro-coagulation
2. production of angiognenic factors like VGEF and EGF
3. adhesion molecules that help WBCs adhere to the wall (VCAM-1; ICAM; VLA-4)
4. Recruitment of neutrophils: E-selectin made and pre-made P-selectin released from Weibel Palade bodies

Question 3

How do ANCAs contribute to the pathogenesis of vasculitis?

Answer 3

ANCA = anti-neutrophil cytoplasmic antibody
ANCAs are directed against either myeloperoxidase (pANCA- because it is Perinuclear and myeloPeroxidase). Looks like the Petals of a flower on fluorescent microscopy.
or Proteinase 3- which is cANCA becasue it is cytoplasmic.
these antibodies activate neutorphils, which can bind to endothelial cells and cause damage. Therefore, pANCA and cANCA have a pathogenic role in the development of disease.

Question 4

Histo features of vasculitis

Answer 4

1. infiltration of vessel wall by neutrophils, mononuclear cells, and/or giant cells (I will focus on neutrophils)
2. Fibrinoid necrosis: a PANMURAL destruction of the vessel wall.

Question 5

What are the ANCA negative vasculitides? Positive?

Answer 5

negative: henoch schonlein purpura and cryoglobulinemia
positive: granulomatosis with polyangiitis (Wegeners) microscopic polyangitis, churg-strauss

Question 6

What are the large vessel vasculitises?

Answer 6

Giant cell/temporal arteritis (one disesae, 2 names)

and Takayasu arteritis

Question 7

What are the medium vessel vasculitities?

Answer 7

PAN (polyarteritis nodosa)

Question 8

localizing symptoms of giant cell arteritis

Answer 8

temporal headache, scalp pain and tenderness, visual distubances including decr. acuity, diplopia, and blindness with retinal infarction (cotton wool spots), jaw claudication (pain or feeling tired during mastication- dt insufficient blood supply to muscles of the jaw). may have a hard, palpable artery in the forehead that looks like a wire loop, or may have scalp necrosis

Question 9

systemic symptoms of giant cell arteritis

Answer 9

polymalgia rheumatica: sudden aching and stiffness- it may be that one day they literally can’t get out of bed
fever, anemia, anorexia, malaise, weight noss

Question 10

typical pt for giant cell arterritis and tx

Answer 10

older adult, may have headaches and VISION CHANGES.

treatment is High dose predinsone to prevent blindness

Question 11

Fast facts about Takayasu’s arteritis

Answer 11

Large vessel vasculitis that predominantly affects young women.
Pulseless disease of the aorta and main branches.
can cause limb claudication and constitutional symptoms.

Question 12

Dx of takayasu’s arteritis

Answer 12

tight stenosis with near occlusion of vessels providing cerebral circulation. seen by ARTERIOGRAPHY.
may also see irregularity and stenosis of abdominal aorta and renal arteries by MRI.

Question 13

Polyarteritis nodosa salient features (aka PAN)

Answer 13

systemic necrotizing vasculitis affectin musclar arteries in the systemic circulation. SPARES the pulmonary circ.
saccular aneurysms common
Causes testicular/epididymal tenderness.
medium vessel vasculitis
associated with mononeuritis multiplex and stroke

Question 14

complications of PAN

Answer 14

HTN, renal failure, bowel infarction/bleeding, pancreatitis, MI

Question 15

dx of PAN

Answer 15

see lots of blebbing and saccular aneurysms on angiograms. may see digital tip infarction and necrosis (black and EXTREMELY painful)