Chapter 7: Vascular Pathology Flashcards by Talha Masood

Large Vessel Vasculitis

Temporal Giant Cell Arteritis

Takayasu Arteritis

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Medium Vessel Vasculitis

Polyarteritis Nodosa
Kawasaki Disease
Buerger Disease

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Small Vessel Vasculitis

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

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MOST COMMON vasculitis in older adults

Temporal(Giant Cell) Arteritis

vasculitis in older adults(>50) usually affects females

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Temporal Arteritis clinical symptoms?

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

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

Ophthalmic Nerve ischemic damage causing blindness

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Takayasu Arteritis demographic

Young Asian females less than 50 years old

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Takayasu Arteritis clinical symptoms

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

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Polyarteritis Nodosa demographic

young adults

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

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

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Diseases associated with ‘String of pearls’

Polyarteritis Nodosa

Fibromuscular Dysplasia

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Polyarteritis Nodosa treatment

cyclophosphamide and corticosteroids

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Kawasaki Disease demographic

Asian children less than 4 years old

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Kawasaki Disease presentation

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

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Kawasaki Disease clinical signs

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

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Kawasaki Disease treatment

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

DESPITE possibility of Reye syndrome which could cause encephalopathy

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Buerger Disease HY associations

SMOKING disease. Treatment is to STOP smoking.

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

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Wegener’s Granulomatosis

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

Rapidly Progressive Glomerulonephritis

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middle aged male with nasopharyngeal ulceration, lung infiltrates and hematuria

What likely renal problem arises?

Wegener’s Granulomatosis

Renal problem is Rapidly Progressive Glomerulonephritis

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Compare Wegener’s to Microscopic Polyangiitis

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

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Compare Microscopic Polyangiitis to Churg-Strauss Syndrome

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

They BOTH have lung involvement, both express p-ANCA

What drug treats vasculitis?

Cyclophosphamide

Nephropathy following Upper Respiratory infection?

IgA nephropathy associated with Henoch-Schonlein Purpura

Henoch-Schonlein Purpura demographic

most common vasculitis in children

Henoch-Schonlein Purpura clinical signs

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

Important cause of stenosis in young females?

fibromuscular dysplasia(frequently involves renal artery)

Arteriolosclerosis types...

Hyaline and hyperplastic type Hyaline arteriolosclerosis caused by proteins leaking into vessel wall causing vascular thickening. Hyperplastic arteriosclerosis caused by malignant hypertension

Hyaline Arteriolosclerosis caused by...

Benign Hypertension and diabetes. Hypertension forces protein into membrane. Diabetes -- non-enzymatic glycosylation of basement mebrane making them more leaky to proteins

Hyaline Arteriolosclerosis classically causes...?

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

Hyperplastic arteriosclerosis caused by

malignant hypertension

Hyperplastic arteriosclerosis leads to...

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

'flea bitten' appearance of kidney

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

Describe clinical syndromes that could predispose to aortic dissection

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

Most common cause of death due to aortic dissection

pericardial tamponade

Classically causes thoracic aneurysm

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

Major complication of thoracic aneurysm

dilation of the aortic valve root, resulting in regurgitation

Abdominal Aortic Aneurysm demographic

male smokers >60 with history of hypertension etiology: Atherosclerosis increases the diffusion barrier to the media, resulting in atrophy and weakness of vessel wall

Reddish growth that blanches on pressure

Hemangioma -- will go away with growth of child

Reddish rash that does not blanch on pressure

Kaposi's Sarcoma

Kaposi's Sarcoma demographic

Older Eastern European males -- tumor remains localized to skin AIDS and Transplant patients -- tumor spreads early

Associated with exposure to PVC?

Liver angiosarcoma also a/w arsenic and Thorotrast

Liver angiosarcoma association?

Poly-vinyl Chloride

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

Giant Cell Temporal Arteritis Prednisone, or other corticosteroid

Weak or absent pulse in upper extremities

Takayasu's Arteritis

Takayasu involves which branches of vasculature?

Branch points of Aortic Arch

Organ spared in PAN

lungs

associated with HBsAg

PAN

transmural inflammation with fibrinoid necrosis

PAN

intimal fibrosis with _______ is what?

with giant cells is Giant Cell Temporal Arteritis

Which vasculitis is fatal if not treated

PAN - treat with cyclophosphamide and corticosteroids

myocardial infarction in young asian child | treat with?

Kawasaki Disease - can also result in aortic aneurysms with rupture aspirin and IVIG

large necrotizing granulomas with adjacent necrotizing vasculitis

Wegeners