Pathoma Chapter 7

Front 1

Large Vessel Vasculitis

Back 1

Temporal Giant Cell Arteritis
Takayasu Arteritis

Front 2

Medium Vessel Vasculitis

Back 2

Polyarteritis Nodosa
Kawasaki Disease
Buerger Disease

Front 3

Small Vessel Vasculitis

Back 3

Wegener Granulomatosis(Granulomatosis with Polyangiitis)
Microscopic Polyangiitis
Churg-Strauss Syndrome
Henoch-Schonlein Purpura

Front 4

MOST COMMON vasculitis in older adults

Back 4

Temporal(Giant Cell) Arteritis

vasculitis in older adults(>50) usually affects females

Front 5

Temporal Arteritis clinical symptoms?

Back 5

Affects branches of carotid artery leading to headache, and visual disturbances(if it hits ophthalmic artery) and jaw claudication.

Vascular lesions are SEGMENTAL so a negative biopsy does NOT exclude disease.

Front 6

What is at risk to be damaged if Giant Cell Arteritis is not treated?

Back 6

Ophthalmic Nerve ischemic damage causing blindness

Front 7

Takayasu Arteritis demographic

Back 7

Young Asian females less than 50 years old

Front 8

Takayasu Arteritis clinical symptoms

Back 8

involves the aortic arch at branch points, leading to upper extremity absent pulses(opposite to coarctation of aorta). Presents with visual and neurological symptoms similar to giant cell arteritis

Front 9

Polyarteritis Nodosa demographic

Back 9

young adults

Front 10

Polyarteritis Nodosa clinical symptoms? Classic finding? a/w?

Back 10

Hypertension(renal artery involvement), abdominal pain with melena(mesenteric artery involvement), neurologic symptoms and skin lesions.

'String of Pearls' appearance on imaging

a/w HBsAg

Front 11

Diseases associated with 'String of pearls'

Back 11

Polyarteritis Nodosa
Fibromuscular Dysplasia

Front 12

Polyarteritis Nodosa treatment

Back 12

cyclophosphamide and corticosteroids

Front 13

Kawasaki Disease demographic

Back 13

Asian children less than 4 years old

Front 14

Kawasaki Disease presentation

Back 14

non-specific similar to infection in beginning but coronary artery involvement common leading to infarction and/or aneurysm.

Front 15

Kawasaki Disease clinical signs

Back 15

Rash on the palms of their hands and soles of their feet.
(think of a kid on a motorcycle)
Coronary artery involvement is common! YOUNG CHILD with a thrombosis and MI or ruptured aneurysm.

Front 16

Kawasaki Disease treatment

Back 16

Aspirin -- prevents platelet aggregation via COX inhibitors which block TXA2.

DESPITE possibility of Reye syndrome which could cause encephalopathy

Front 17

Buerger Disease HY associations

Back 17

SMOKING disease. Treatment is to STOP smoking.

presents with necrotizing vasculitis involving digits. Associated with Raynauds(white-blue-red). autoamputation of digits without treatment.

Front 18

Wegener's Granulomatosis

Back 18

Involves nasopharynx, lungs and kidneys.
c-ANCA positive
Treatment is Cyclophosphamide

Rapidly Progressive Glomerulonephritis

Front 19

middle aged male with nasopharyngeal ulceration, lung infiltrates and hematuria

What likely renal problem arises?

Back 19

Wegener's Granulomatosis
Renal problem is Rapidly Progressive Glomerulonephritis

Front 20

Compare Wegener's to Microscopic Polyangiitis

Back 20

Microscopic Polyangiitis involves lung and kidney but NOT oropharynx. Also expresses p-ANCA NOT c-ANCA. Will also NOT have granulomas.

Front 21

Compare Microscopic Polyangiitis to Churg-Strauss Syndrome

Back 21

Churg-Strauss presents with Asthma, Granulomas and peripheral eosinophilia.

They BOTH have lung involvement, both express p-ANCA.

Front 22

What drug treats vasculitis?

Back 22

Cyclophosphamide

Front 23

Nephropathy following Upper Respiratory infection?

Back 23

IgA nephropathy associated with Henoch-Schonlein Purpura

Front 24

Henoch-Schonlein Purpura demographic

Back 24

most common vasculitis in children

Front 25

Henoch-Schonlein Purpura clinical signs

Back 25

palpable purpura on buttocks and legs, GI pain and bleeding, IgA nephropathy.

Front 26

Important cause of stenosis in young females?

Back 26

fibromuscular dysplasia(frequently involves renal artery)

Front 27

Back 27

Atherosclerotic Clefts characterized by cholesterol clefts

Front 28

Etiology of aneurysm

Back 28

Front 29

Arteriolosclerosis types...

Back 29

Hyaline and hyperplastic type

Hyaline arteriolosclerosis caused by proteins leaking into vessel wall causing vascular thickening.

Hyperplastic arteriosclerosis caused by malignant hypertension

Front 30

Hyaline Arteriolosclerosis caused by...

Back 30

Benign Hypertension and diabetes.
Hypertension forces protein into membrane.

Diabetes -- non-enzymatic glycosylation of basement mebrane making them more leaky to proteins.

Front 31

Hyaline Arteriolosclerosis classically causes...?

Back 31

Glomerular Scarring(arteriolonephrosclerosis) This is what causes chronic renal failure in chronic hypertension/diabetes.

Front 32

Hyperplastic arteriosclerosis caused by

Back 32

malignant hypertension

Front 33

Hyperplastic arteriosclerosis leads to...

Back 33

end organ ischemia due to reduced vessel caliber. FIBRINOID necrosis with possible hemorrhage. "onion skin" appearance

Classically causes acute renal failure with 'flea bitten' appearance

Front 34

'flea bitten' appearance of kidney

Back 34

acute renal failure due to hyperplastic arteriolosclerosis in response to malignant hypertension

Front 35

Etiology of Aortic Dissection due to HTN

Back 35

Front 36

Describe clinical syndromes that could predispose to aortic dissection

Back 36

Marfan's Syndrome(fibrillin defect) or Ehlers-Danlos Syndrome(collagen defect)

Front 37

Complications of aortic dissection

Back 37

Front 38

Most common cause of death due to aortic dissection

Back 38

pericardial tamponade

Front 39

Classically causes thoracic aneurysm

Back 39

tertiary syphilis which causes endartertis of the vaso vasorum resulting in 'tree barkin' of the aorta.

Front 40

Major complication of thoracic aneurysm

Back 40

dilation of the aortic valve root, resulting in regurgitation

Front 41

Abdominal Aortic Aneurysm demographic

Back 41

male smokers >60 with history of hypertension

etiology: Atherosclerosis increases the diffusion barrier to the media, resulting in atrophy and weakness of vessel wall

Front 42

Reddish growth that blanches on pressure

Back 42

Hemangioma -- will go away with growth of child

Front 43

Reddish rash that does not blanch on pressure

Back 43

Kaposi's Sarcoma

Front 44

Kaposi's Sarcoma demographic

Back 44

Older Eastern European males -- tumor remains localized to skin

AIDS and Transplant patients -- tumor spreads early.

Front 45

Associated with exposure to PVC?

Back 45

Liver angiosarcoma

also a/w arsenic and Thorotrast

Front 46

Liver angiosarcoma association?

Back 46

Poly-vinyl Chloride

Front 47

high risk of blindness without treatment in this disorder. What treatment?

Back 47

Giant Cell Temporal Arteritis

Prednisone, or other corticosteroid

Front 48

Weak or absent pulse in upper extremities

Back 48

Takayasu's Arteritis

Front 49

Takayasu involves which branches of vasculature?

Back 49

Branch points of Aortic Arch

Front 50

Organ spared in PAN

Back 50

lungs

Front 51

associated with HBsAg

Back 51

PAN

Front 52

transmural inflammation with fibrinoid necrosis

Back 52

PAN

Front 53

intimal fibrosis with _______ is what?

Back 53

with giant cells is

Giant Cell Temporal Arteritis

Front 54

Which vasculitis is fatal if not treated

Back 54

PAN - treat with cyclophosphamide and corticosteroids

Front 55

myocardial infarction in young asian child
treat with?

Back 55

Kawasaki Disease - can also result in aortic aneurysms with rupture

aspirin and IVIG

Front 56

large necrotizing granulomas with adjacent necrotizing vasculitis

Back 56

Wegeners