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STEP 1 Pathoma > Chapter 7 > Flashcards

Chapter 7 Flashcards

1
Q

What are the clinical features of a vasculitis?

A
  • Nonspecific features of inflammation (e.g. fever, fatieu, weight loss, myalgias)
  • Symptoms of organ ischemia due to luminla narrowing or thrombosis
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2
Q

Note that vasculites are divided into large (the aorta and its major branches), medium (Muscular arteries that supply organs), and small vessel vasculitis (arterioles, capillaries, and venules).

What are the large vessel vasculites?

A
  1. Temporal (Giant cell) arteritis
  2. Takayasu Arteritis
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3
Q

What is Giant cell arteritis?

A

Granulomatous vasculitis that classically involves branches of the carotid artery (most common form of vaculitis in 50+; affects females more)

Presents as a temporal HA, visual disturbances, and jaw claudication. ESR will be elevated and joint and muscle pain due to polymyalgia rheumatica may be present

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4
Q

What is Takayasu Arteritis?

A

granuomatous vasculitis that classically involves the aortic arch at branch pts. Presents in adult LESS than 50 yo (classically young Asian females) as visual and neurologic symptoms with a weak or absnet pulse in the upper extremity

Tx with steroids

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5
Q

What are the medium sized vasculites?

A

Polyarteritis Nodosa

Kawasaki Disease

Buerger Disease

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6
Q

Describe Polyarteritis Nodosa

A

Necrotizing vasculitis involving multiple organs (lungs are SPARED) classically presenting in young adults as HTN (renal a. involvment), abdominal pain with melena, neurologic disturbances, and skin lesions

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7
Q

Polyarteritis Nodosa is associated with ______

A

HBsAg

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8
Q

What is this?

A

Lesions of PN may vary in stages. Early lesions consist of transmural inflammation with fibrinoid necrosis that eventually heal with fibrosis, producing a ‘string of pearl’s appearance’ on imaging

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9
Q

How is PN tx?

A

Steroids and cyclophosphamide (fatal if not tx)

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10
Q

Describe Kawasaki disease

A

Disease of (classically) less than 4 yo Asian children presenting with nonspecific signs including fever, conjunctivitis, erythematous rash on the palms and soles, and enlarged lymph nodes

Coronary artery involvemnt is common and leads to risk for thrombosis with MI and aneurysm with rupture

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11
Q

How is Kawasaki disease tx?

A

ASPIRIN and IVIG

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12
Q

Describe Buerger Disease

A

Necrotizing vasculitis of the digits presenting with ulceration, gangrene, and autoamputation

Highly associated with smoking

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13
Q

What are the small vessel vasculities?

A
  • Wegener Granulomatosis
  • Microscopic Polyangiitis
  • Churg-Strauss Syndrome
  • Henoch-Schonlein Purpura
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14
Q

Describe Wegener Granulomatosis

A

Necrotizing granulomatous vasculitis involving the nasopharynx, lungs, and kidneys

Classically presents in MIDDLE AGED MEN with sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, and hematuria due to rapidly progressive glomerulonephritis

Associated with c-ANCA

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15
Q

How is Wegener Granulomatosis tx?

A

Steroids and cyclophosphamide; relapses are common

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16
Q

Describe Microscopic Polyangiitis

A

Necrotizing vaculitis involving multiple oragns, especially the LUNGS and KIDNEYS with a presentation similar to Wegener’s, but without nasopharyngeal involvment and no granulomaes

Associated with p-ANCA

Tx with cyclophosphamide and steroids; relapses common

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17
Q

Describe Churg-Strauss Syndrome

A

Necrotizing granulomatous inflammation with EOSINOPHILS involving multiple oragsn, especially the lungs and HEART. ASthma and peripheral eosinophilia are often present

Associated with p-ANCA

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18
Q

Describe Henoch-Schenlein Purpura

A

Vasculitis due to IgA complex deposition (most common vaculitis in CHILDREN) presenting with palpable purpura on buttocks and legs, GI pain and bleeding, and hematuria (IgA nephropathy) usually following an URTI

Self limited but may recur; tx wit steroids

19
Q

Systemic HTN is defined as BP > 140/90 and its causes are divided into primary and 2ndary causes. Primary causes (95%) are of unknown etiology and risk factors include AA, age, obesity, stress, and a high-salt diet.

What are some common causes of 2ndary HTN (5%)?

A

Renal artery stenosis- stenosis decreases blood flow to the glomeruli. the Juxtagomerular apparatus responds by secreting renin, which converts angiotensinogen to angiotensin I. ATI to ATII by ACE. ATII then raises BP by contracting ateriolar smooth muscle increasing TPR, and promoting adrenal release of aldosterone.

All of this leads to HTN with increased plasma renin and unilaterla atrophy of the affected kidney

20
Q

Important causes of renal artery stenosis include:

A
  • Atherosclerosis in elderly
  • Fibromuscular dysplasia (young females)
21
Q

What is Fibromuscular dysplasia?

A

A developmental defect of the blood vessel wall, resuting in irregular thickening of large and medium sized arteries, especially the renal artery

22
Q

Difference between benign and malignant HTN

A

Benign: Mild elevation that is clinically silent and slowly leads to organ damage

Malignant: Severe elevation that precipitates end organ failure

23
Q

Atherosclerosis

A
24
Q

What is arteriosclerosis?

A

Hard arteries due to the blood vessel wall thickening

Three pathologic patterns- atherosclerosis, arteriolosclerosis, and Monckeberg medial calcific sclerosis

25
Q

What is atherosclerosis?

A

Intimal plaque composed of a necrotic lipid core (mostly cholesterol) with a fibromuscular cap that often undergoes ystrophic calcification

26
Q

What are the most common places for atherosclerosis?

A

abdominal aorta, coronary artery, popliteal artery, and internal carotid artery

27
Q

How does atherosclerosis occur?

A

Damage to endothelium allows lipids to leak into the intima where they are oxidized and then consumed by macrophages via scavenger receptors to form foam cells. Inflammation and healing leads to deposition of extracellular matrix and proliferation of smooth muscle

28
Q

What are the morphologic stages of atherosclerosis?

A
  • begins as fatty streaks (consiss of lipid-laden macrophages)- arise in early life (teen yrs)
  • progresses to plaque
29
Q

What is arteriolosclerosi?

A

Narrowing of small arterioles- divided into hyaline and hyperplastic types

30
Q

Describe Hyaline arteriolosclerosis

A

Caused by proteins leaking into the vessel wall producing vascular thickening (seen as pink hyaline on microscopy) as a conseqeunce of long-standing benign HTN or diabetes

Results in reduced vessel caliber with end-organ ischemia; classically produces glomerular scarring (arteriolonephrosclerosis) that slowly progresses to chronic renal failure

31
Q

Describe Hyperplastic arteriolosclerosis

A

Involves thickening of vessel walls by hyperplasia of smooth muscle (onion skin appearance) as a consequence of MALIGNANT HTN.

May lead to fibrinoid necrosis of the vessel wall with hemorrhage

32
Q

What is Monckeberg Medial Calcific Sclerosis?

A

Calcification of the MEDIA of muscular arteries (nonobstructive)

Not clinially signifcant- incident finding on XRAY or mammography

33
Q

What is an aortic dissection?

A

An intimal tear with dissection of bloof through the media of the aortic wall. Typically occurs in the proximal 10cm of the aorta (high stress region) with preexisiting weakness of the media

34
Q

What are the causes aortic dissection?

A

HTN in older adults- causes hyaline arteriolsoclerosis of the vasa vasorum resulting in atrophy

inherited defects in CT in younger pts.- Marfan and Ehlers-Danlos

35
Q

How does aortic dissection present?

A

sharp, tearing chest pain that radiates to the back

36
Q

What are the main complications of aortic dissection?

A

pericardial tamponade (most common cause of death), rupture with fatal hemorrhage, and obstruction with end-organ ischemia

37
Q

Common cause of thoracic aneurysm?

A

Syphillis

38
Q

Abdominal aortic aneurysms

A
  • Usually arises below the renal arteries but above the aortic bifurcation
  • primarily due to atherosclerosis; classically seen in male smokers 60+ yo with HTN
  • Major compliation is rupture, esp. when 5+ cm in diameter
39
Q

What is a hemangioma?

A

Benign tumor comprised of blood vessels

commonly presents at birth and often regresses in childhood

most often on skin and liver

40
Q

What is an angiosarcoma?

A

A MALIGNANT proliferation of endothelial cells (highly agressive)

Commonly on the skin, breast, and liver

41
Q

Liver angiosarcoma is associated with _______

A

polyvinyl chloride, arsenic, and thorotrast

42
Q

Kaposi Sarcoma

A

Low-grade MALIGNANT proliferation of endothelial cells assocaited with HHV-8

43
Q

Associations of Kaposi Sarcoma?

A
  1. Older Eastern European males- tumors localized to skin; tx with removal
  2. AIDS- tumors spread early; tx with HAART therapy to boost immune system
  3. Transplant rejection- tumor spreads early; tx if decreasing immunosuppression

STEP 1 Pathoma (18 decks)

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