Pathoma- Vasculitis Flashcards

1
Q

What symptoms are common to all vasculidities?

A
  1. nonspecific symptoms (myalgia, malaise, etc)

2. symptoms of organ ischemia (depending on the organ)

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2
Q

What are 2 ways organ ischemia can occur?

A
  1. thrombosis in a BV

2. fibrosis of the BVs

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3
Q

What are the 2 large vessel vasculidities?

A
  1. temporal (Giant Cell) arteritis

2. Takayasu Arteritis

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4
Q

What is temporal (Giant Cell) arteritis?

A

the most common form of vasculitis in older adults, usually affects females over age 50; manifests in carotid artery and its branches

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5
Q

What are the s/s of temporal (Giant Cell) arteritis?

A
headache
visual disturbances
jaw claudication
flu-like symptoms (joint and muscle pain)
elevated ESR (>100)
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6
Q

What is seen in the biopsy of temporal (Giant Cell) arteritis?

A

inflamed vessel wall
giant cells (due to granulomatous vasculitis)
intimal fibrosis
segmental lesions

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7
Q

What is the tx for temporal (Giant Cell) arteritis?

A

corticosteroids ASAP

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8
Q

Why is tx so important in temporal (Giant Cell) arteritis?

A

high risk of blindness w/o tx

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9
Q

How are Takayasu Arteritis and temporal (Giant Cell) arteritis different?

A

in Takayasu:

  • younger than age 50, usually young Asian females
  • usu aortic arch at branch points
  • weak or absent pulse in upper extremities
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10
Q

What BVs do the medium-vessel vasculidities supply?

A

they’re muscular arteries that supply organs

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11
Q

Name the medium vessel vasculidities.

A
  1. Polyarteritis Nodosa
  2. Kawasaki Disease
  3. Buerger Disease
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12
Q

What are the features of Polyarteritis Nodosa?

A
  1. multiple artery inflammation
  2. necrotizing vasculitis
  3. involves most organs EXCEPT THE LUNG
  4. in young adults
  5. serum HBsAg (hep B surface antigen)
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13
Q

How do the Polyarteritis Nodosa lesions look on imaging?

A

string-of-pearls nodulation (fibrinoid necrosis)

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14
Q

What is the tx for Polyarteritis Nodosa?

A

corticosteroids and cyclophosphamide

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15
Q

What happens in untreated Polyarteritis Nodosa?

A

death

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16
Q

Who does Kawasaki Disease affect?

A

Asian children <4yo

17
Q

What is the presentation for Kawasaki Disease?

A

fever
conjunctivitis
erythematous rash of palms and soles
enlarged cervical lymph nodes

18
Q

What artery is preferred in Kawasaki Disease?

A

the coronary artery

19
Q

What is the tx for Kawasaki Disease?

A

aspirin and IVIG

20
Q

What vessels does Buerger Disease affect?

A

BVs of the fingers

21
Q

Buerger Disease is only associated with _____.

A

smokers

22
Q

What is Raynaud phenomenon?

A

discoloration of the fingers/toes bc of vasospasm

23
Q

What is the presentation for Buerger Disease?

A
  1. necrotizing vasculitis of digits (ulcers, gangrene, autoamputation)
  2. Raynaud phenomenon
24
Q

What is the tx for Buerger Disease?

A

smoking cessation

25
Q

What are the small vessel vasculidies?

A
  1. Wegener Granulomatosis
    * WeCKener
  2. Microscopic Polyangiitis
  3. Churg-Strauss Syndrome
  4. HSP
26
Q

What does Wegener Granulomatosis look like?

A
  1. necrotizing granulomatous vasculities involving the nasopharynx, lungs, and kidneys
  2. serum C-ANCA
  3. sinusitis/nasopharyngeal ulceration
  4. hematuria due to rapidly progressive glomerulonephritis (RPGN)
27
Q

What is the tx for Wegener Granulomatosis?

A

cyclophosphamide and corticosteroids

28
Q

What is the ANCA test?

A

neutrophil on a slide is given pt’s serum- if Abs are present, they will present near the nucleus (P-ANCA) or on the outer rim of the nucleus (C-ANCA)

29
Q

What are the features of Microscopic Polyangiitis?

A
  1. necrotizing vasculitis of multiple organs, esp lung and kidney
  2. P-ANCA
  3. NO granulomas or nasopharyngeal involvement
30
Q

What is the tx for Microscopic Polyangiitis?

A

cyclophosphamide and corticosteroids

31
Q

What is Churg-Strauss Syndrome?

A
  1. necrotizing granulomatous vascultis with eos and asthma
  2. multiple organs, esp lungs and heart
  3. P-ANCA
32
Q

What does HSP stand for?

A

h___ine purpura

33
Q

What is HSP?

A
  1. vasculitis due to IgA immune complex deposition

2. most common vasculitis of children

34
Q

What is the classical presentation of HSP?

A
  1. classical purpura on buttocks and legs
  2. GI pain and bleeding
  3. hematuria (kidney IgA nephropathy)
  4. post URI/viral infection