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56 Cards in this Set

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Large Vessel Vasculitis
Temporal Giant Cell Arteritis
Takayasu Arteritis
Medium Vessel Vasculitis
Polyarteritis Nodosa
Kawasaki Disease
Buerger Disease
Small Vessel Vasculitis
Wegener Granulomatosis(Granulomatosis with Polyangiitis)
Microscopic Polyangiitis
Churg-Strauss Syndrome
Henoch-Schonlein Purpura
MOST COMMON vasculitis in older adults
Temporal(Giant Cell) Arteritis

vasculitis in older adults(>50) usually affects females
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.
What is at risk to be damaged if Giant Cell Arteritis is not treated?
Ophthalmic Nerve ischemic damage causing blindness
Takayasu Arteritis demographic
Young Asian females less than 50 years old
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
Polyarteritis Nodosa demographic
young adults
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
Diseases associated with 'String of pearls'
Polyarteritis Nodosa
Fibromuscular Dysplasia
Polyarteritis Nodosa treatment
cyclophosphamide and corticosteroids
Kawasaki Disease demographic
Asian children less than 4 years old
Kawasaki Disease presentation
non-specific similar to infection in beginning but coronary artery involvement common leading to infarction and/or aneurysm.
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.
Kawasaki Disease treatment
Aspirin -- prevents platelet aggregation via COX inhibitors which block TXA2.

DESPITE possibility of Reye syndrome which could cause encephalopathy
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.
Wegener's Granulomatosis
Involves nasopharynx, lungs and kidneys.
c-ANCA positive
Treatment is Cyclophosphamide

Rapidly Progressive Glomerulonephritis
middle aged male with nasopharyngeal ulceration, lung infiltrates and hematuria

What likely renal problem arises?
Wegener's Granulomatosis
Renal problem is Rapidly Progressive Glomerulonephritis
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.
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)
Atherosclerotic Clefts characterized by cholesterol clefts
Etiology of aneurysm
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
Etiology of Aortic Dissection due to HTN
Describe clinical syndromes that could predispose to aortic dissection
Marfan's Syndrome(fibrillin defect) or Ehlers-Danlos Syndrome(collagen defect)
Complications of aortic dissection
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