Chapter 9 - blood vessles Flashcards

1
Q

Giant cell (temporal) arteritis treatment:

A
  • croticosteroid

- anti-TNF therapies

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2
Q

Clinical features of Giant cell (temporal) arteritis:

A
  • rare before 50yr
  • fever, fatigue, weight loss, malaise, muscle aches
  • polymalgia rheumatica (flu-like+joint pain)
  • facial pain/headache, intense -> superficial temporal artery (painful to palpation)
  • vision loss (sudden and permanent).
  • Double or blurred vision.
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3
Q

What is the most common form of vasculitis?

A

Giant cell (temporal) arteritis

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4
Q

Distribution of Giant cell (temporal) arteritis:

A
  • small and medium sized arteries
  • cranial arteries (temporal(important), facial, and opthalmic)
  • Aortic arch - giant cell aortitis (uncommon)
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5
Q

Giant cell (temporal) arteritis LAB:

A

elevated ESR (erythrocyte sedimentation rate)

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6
Q

Giant cell (temporal) arteritis morphology:

A
  • patchy along the length of the affected vessel
  • nodular intimal thickening (and occasional thromboses) that reduce the lumen diameter and cause distal ischemia
  • granulomatous inflammation
  • fragmentation of the internal elastic lamina
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7
Q

Pulseless disease=

A

Takayasu arteritis

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8
Q

Takayasu arteritis epidemiology:

A
  • most common in Asia

- affects young and middle-aged women (ages 15-45)

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9
Q

Takayasu arteritis (pulseless disease) distribution:

A
  • medium-sized to large arteries

- aortic arch and major branches

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10
Q

Takayasu arteritis (pulseless disease) characteristics:

A
  • ocular disturbance

- weakening of the pulses of the upper extremeties

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11
Q

Takayasu arteritis (pulseless disease) pathology:

A
  • transmural scarring
  • irregular fibrous thickening of the aorta (especially the aortic arch and great vessels)
  • narrowing of the orifices of the major arterial branches
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12
Q

Takayasu arteritis (pulseless disease) clinical features:

A
  • reduced blood pressure and pulse strength in the upper extremities
  • fatigue, wight loss, and fever
  • neurologic deficit/abnormalities
  • visual field defects, retinal hemorrhages, and total blindness
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13
Q

Takayasu arteritis (pulseless disease) treatment:

A

steroids

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14
Q

What can Takayasu arteritis (pulseless disease) lead to:

A
  • pulmonary artery -> pulmonary hypertention

- renal arteries -> systemic hypertention

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15
Q

Takayasu arteritis (pulseless disease) morphology:

A
  • affect the aortic arch and arch vessels
  • dialtion and aortic valve insufficiency
  • pulmonary artery. renal and coronary arteries may be affected
  • takeoffs of great vessels are narrowed
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16
Q

Polyarteritis nodosa clinical:

A
  • malaise, fever, weight loss
  • hypertention due to renal artery involvement
  • abdominal pain and bloody stools caused by vascular gastrointestinal lesions
  • diffuse muscular aches and pains
  • peripheral neuritis
  • predominantly affecting motor nerves
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17
Q

What is polyarteritis nodosa?

A

Necrotizing immune complex inflammation of small or medium-sized muscular arteries, typically involving renal or visceral vessels.

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18
Q

What is the treatment of polyarteritis nodosa?

A

Corticosteroids, azathioprine, and/or cyclophosphamide

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19
Q

What is an association of polyarteritis nodosa?

A

Hepatitis B infection (30% of patients)

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20
Q

What are the signs of polyarteritis nodosa?

A
  1. Cotton wool spots
  2. Microaneurysms
  3. Pericarditis
  4. Myocarditis
  5. Palpable purpura
  6. Elevated ESR
  7. P-ANCA positive serum
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21
Q

PAN =

A

polyarteritis nodosa

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22
Q

Polyarteritis nodosa epidemiology:

A
  • young adults

- male>female

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23
Q

Polyarteritis nodosa distribution:

A
  • systemic vasculitis- any organ except for lungs
  • kidney, heart, GI tract, muscle etc.
  • small and medium size arteries
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24
Q

Polyarteritis nodosa clinical features:

A
  • low-grade fever, malaise, wight loss
  • hematouria, renal failure, hypertention
  • abdominal pain, diarrhea, and GI bleeding
  • myalgia and arthralgia
25
Q

Polyarteritis nodosa pathology three stages:

A
  • acute lesion: fibrinoid necrosis and neutrophils
  • healing lesion: fibroblast proliferation
  • healed lesion: nodular fibrosis and loss of internal elastic lamina
26
Q

Polyarteritis nodosa sequela:

A
  • thrombosis and infarction

- aneurysm (kidneys, heart, and GI tract)

27
Q

Polyarteritis nodosa LAB:

A
  • antibodies against myeloperoxidase

- P-ANCA

28
Q

Polyarteritis nodosa treatment:

A

Corticosteroids and Cyclophosphamide

29
Q

Kawasaki disease epidemiology:

A
  • Commonly affects infants and young children (age <4)

- Japan, Hawaii, and U.S. mainland

30
Q

Kawasaki disease clinical features

A
  • Acute febrile illness
  • Conjunctivitis; erythema, blistering and erosions of the oral mucosa
  • Generalized maculopapular skin rash (desquamative rash)
  • edema of the hands and feet
  • erythema of the palms and soles
  • Lymphadenopathy, cervical lymph node enlargment
31
Q

Kawasaki disease distribution:

A
  • Large,medium-sized, and small arteries

- Coronary artery commonly affected (70%)

32
Q

Kawasaki disease pathology:

A
  • Segmental necrotizing vasculitis

- Weakened vascular wall may undergo aneurysm formation

33
Q

Kawasaki disease def. #1:

A

is a febrile lymphadenopathy with rash with an associated segmental necrotizing vasculitis with a predilection for the coronary arteries

34
Q

Kawasaki disease def. #2:

A

acute, febrile, usually self-limited illness of infancy and childhood (80% of the patients are younger than 4 yrs old) associated with an arteritis of mainly large to medium-sized vessels

35
Q

(Kawasaki disease) What can coronary arteritis cause?

A

aneurysm that rupture or thrombose, resulting in myocardial infarction.

36
Q

Kawasaki disease =

A

mucocutaneous lymph node syndrome

37
Q

How are the cardiovascular sequelae that develops because of the Kawasaki disease?

A

they range from asymptomatic coronary arteritis, to coronary artery ectasia, to large coronary artery aneurysm with rupture or thrombosis, myocardial infarction, and sudden death.

38
Q

Microscopic polyangiitis =

A

hypersensitivity vasculitis or leukocytoclastic vasculitis

39
Q

Microscopic polyangiitis distribution:

A
  • (necrotizing vasculitis)

- capillaries, small arterioles and venules

40
Q

What is involved in Microscopic polyangiitis?

A
  • skin
  • mucous membranes
  • lungs
  • brain
  • heart
  • GI tract
  • kidneys
  • muscle
41
Q

What is common in Microscopic polyangiitis?

A
  • necrotizing glomerulonephritis (90%)

- pulmonary capillaritis

42
Q

Microscopic polyangiitis is associated with?

A

MPO-ANCA

43
Q

Microscopic polyangiitis is characterized by….

A

segmental fibrinoid necrosis of the media with focal transmural necrotizing lesions; granulomatous inflammation is absent

44
Q

are macroscopic infarctions common in Microscopic polyangiitis?

A

nope

45
Q

lesions that are “pauci-immune” ->

A

Microscopic polyangiitis

46
Q

pauci-immune =

A

show little or no antibody

47
Q

Microscopic polyangiitis features:

A
  • hemoptysis
  • hematuria
  • proteinuria
  • abdominal pain or bleeding
  • muscle pain or weakness
  • palpable cutaneous purpura
48
Q

C-ANCA

A

Wegerner’s granulomatosis

49
Q

What is the treatment of wegener’s granulomatosis?

A

cyclophosphamide, corticosteroids, and/or methotrexate

50
Q

What are the symptoms of Wegeners granulomatosis?

A
  1. Perforation of nasal septum
  2. Chronic sinusitis
  3. Otitis media
  4. Mastoiditis
  5. Cough
  6. Dyspnea
  7. Hemoptysis
51
Q

What are the findings of Wegener’s granulomatosis?

A
  1. C-ANCA positive
  2. CXR reveals large nodular lesions
  3. Hematuria and red cell casts
52
Q

Wegener’s granulomatosis epidemiology:

A
  • Rare; males> females

- Peak incidence: ages 40-60

53
Q

Wegener’s granulomatosis distribution:

A
  • Necrotizing vasculitis with granulomas
  • Classically involves the nose,sinuses,lungs,and kidneys
  • Small size arteries and veins
  • small to medium sized vessels (capillaries, venules, arterioles, and arteries)
54
Q

Wegener’s granulomatosis clinical features:

A
  • Bilateral pnuemonitis with nodular and cavitary pulmonary infiltrates
  • Chronic sinusitis
  • Nasopharyngeal ulcerations
  • Renal disease
    • Focal necrotizing glomerulonephritis
    • Crescentic glomerulonephritis
55
Q

Wegener’s granulomatosis LAB:

A
  • Cytoplasmicantineutrophil cytoplasmic autoantibodies (C-ANCA)
    • Autoantibody againstproteinase3
    • Correlates with disease activity
56
Q

Wegener’s granulomatosis treatment:

A

immunosuppressive drugs (cyclophosphamide)

57
Q

Wegener’s granulomatosis is charachterized by:

A
  • granulomas of the lung and/or upper respiratory tract (ear, nose, sinuses, throat)
  • vasculitis of small to medium sized vessels most prominently in lungs and upper respiratory tract
  • glomerulonephritis
58
Q

The widespread form of Wegener’s granulomatosis:

A

can affect, eyes, skin, and other organs, notably the heart