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7 Vascular Pathology > 7.1 Vasculitis > Flashcards

7.1 Vasculitis Flashcards

1
Q

Wegener’s granulomatosis

-clinical presentation

A

Middle aged male with:

  1. Nasal: sinusitis/nasopharyngeal ulceration
  2. Lungs: hemoptysis with bilateral nodular lung infiltrates
  3. Kidneys: rapidly progressive glomerulonephritis
    - remember “weCener’s”
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1
Q

Churg-Strauss syndrome

  • what is it
  • what is characteristic of it
A
  • necrotizing granulomatous vasculitis, with eosinophils. Aka allergic granulomatosis
  • asthma, peripheral eosinophilia often present
  • many organs affected
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2
Q

Takaysu arteritis

-what vessels classically involved

A

aortic arch branch points

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3
Q

which vessels most classically involved in Temporal Giant cell arteritis?

A

branches of carotid

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3
Q

Kawasaki’s disease

-clinical presentation (4 general symptoms)

A
  • nonspecific signs, looks like viral infection in young child.
    1. fever
    2. conjunctivitis
    3. erythematous rash of palms, soles
    4. enlarged cervical lymph nodes (mucocutaneous lymph node syndrome)
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4
Q

Kawasaki’s disease

-treatment

A
  • aspirin and IVIG
  • yes, aspirin in a child even though Reye’s syndrome can occur in child with viral illness, which is what Kawasaki’s looks like.
  • disease is usually self limited
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4
Q

microscopic polyangiitis

-what organs most affected

A
  • necrotizing vasculitis, small vessels
  • multiple organs, esp lung and kidney
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5
Q

Takaysu arteritis

-symptoms

A
  • visual and neurologic symptoms
  • weak/absent pulse in upper extremity (“pulseless disease”)
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5
Q

Kawasaki’s disease

-what is the big risk/complication?

A

-coronary artery involvement, can lead to thrombus (MI) and aneurysm

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5
Q

p-ANCA vs c-ANCA:

-what diseases do these differentiate?

A

p-ANCA: microscopic polyangiitis and Churg-Strauss syndrome

c-ANCA: Wegener’s Granulomatosis (“WeCener’s”)

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6
Q

Why do you see organ ischemia in vasculitis?

-2 mechanisms

A
  1. luminal narrowing of vessels. inflammation, fibrosis
  2. thrombosis of inflamed vessels
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6
Q

Buerger’s disease

-clinical presentation

A

aka Thromboangiitis obliterans

  • necrotizing vasculitis and thrombosis involving digits–Raynaud’s phenomenon often present
  • painful ischemia, peripheral pulses diminished
  • ulceration, gangrene, autoamputation of fingers, toes.
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7
Q

Wegener’s granulomatosis

-classic population?

A

middle aged male

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8
Q

Wegener’s granulomatosis

  • lab test used for diagnosis
  • what do you see on biopsy?
  • tx
A

‘WeCener’s’

  • c-ANCA
  • large necrotizing granulomas with adjacent vasculitis
  • Cyclophosphamide
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9
Q

polyarteritis nodosa

-treatment

A

corticosteroids and cyclophosphamide

-fatal if untreated

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10
Q

what is ANCA test?

what is p-ANCA and c-ANCA?

A
  • ANCA: anti-neutrophil cytoplasmic antibody
    1. put neutrophil on slide
    2. Put pt’s serum on slide
    3. if serum has Ab against neutrophil, you will see ANCAs

c-ANCA: cytoplasmic Ab (cytoplasm away from nucleus)

p-ANCA: pernuclear Ab (next to nucleus)

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11
Q

polyarteritis nodosa

-what blood test finding associated with?

A

serum HBsAg

(hep B surface Ag)

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13
Q

temporal giant cell arteritis:

-ESR level?

A
  • ESR is elevated (>100)
  • often see flu-like symptoms in pt
13
Q

microscopic polyangiitis vs Churg-Strauss syndrome:

-how to differentiate? (3 things)

A

Both are p-ANCA

  1. C-S has granulomas
  2. C-S often has asthma
  3. C-S often has peripheral eosinophilia
13
Q

Henoch-Schonlein purpura

  • course of disorder
  • tx
A
  • typically self-limiting without tx
  • Tx with corticosteroids if severe
14
Q

Henoch-Schonlein purpura

-how does it present?

A
  • Purpura: palpable purpura on buttocks, legs
  • GI: GI pain/bleeding
  • Kidneys: hematuria (IgA nephropathy)
16
Q

temporal giant cell arteritis

-what does biopsy reveal?

A
  • See inflamed vessel wall with giant cells and intimal fibrosis.
  • lesions are segmental, so requires biopsy of long section of vessel
  • absence of lesion still doesn’t rule it out
17
Q

microscopic polyangiitis

-how is presentation different from Wegener’s granulomatosis (2 things)

A
  • microscopic polyangiitis is similar to Wegener’s granulomatosis except:
    1. no nasal pharyngeal involvement
    2. granulomas absent.
18
Q

Wegener’s granulomatosis

  • what is it
  • what organs involved?
A

-necrotizing granulomatous vasculitis, small vessels

Organs:

  1. nasopharynx
  2. lungs
  3. kidneys
18
Q

Henoch-Schonlein purpura

  • what is it
  • mech
  • when do pts typically get it
A
  • Vasculitis of small vessels from depositions of IgA complexes
  • typically occurs after upper respiratory infection, which creates IgA, which deposits throughout body
19
Q

polyarteritis nodosa

-classic presentation (4 symptoms)

A
  1. HTN from renal artery
  2. Abdominal pain with melena (mesenteric a)
  3. neurologic disturbances
  4. skin lesions
20
Q

polyarteritis nodosa

-what organs are affected, which ones not?

A

-all organs potentially affected except lungs

22
Q

Vasculitis in general:

-symptoms

A
  • nonspecific symptoms of inflammation (fever, fatigue, weight loss, mylagias)
  • organ ischemia, from:
    1. luminal narrowing (inflammation, then fibrosis)
    2. thrombosis of inflamed vessels
23
Q

What syndrome is Kawasaki’s syndrome also called, and why?

A

mucocutaenous lymph node syndrome

  • mucus membrane inflammation,
  • enlarged lymph nodes, esp cervical
25
Q

what is the most common form of vasculitis in older adults (>50)

A

temporal giant cell arteritis

27
Q

Vasculitis:

  • name disorders in each category
    1. large (2)
    2. med (3)
    3. small (4)
A
  1. large: Temporal Giant Cell arteritis, Takayasu’s arteritis
  2. medium: Polyarteritis nodosa, Kawasaki’s disease, Buerger disease
  3. small: Wegener’s granuomatosis, microscopic polyangiitis, churg-strauss syndrome, henoch-schonlein purpura
28
Q

Raynaud’s phenomenon:

  • color changes in fingers?
  • what are some disorders secondary Raynaud’s is associated with?
A
  • white, blue (vessels dilate to retain blood), then red (reperfusion)
  • SLE, scleroderma, Buerger’s disease, atherosclerosis
30
Q

Takaysu arteritis

-treatment

A

corticosteroids

31
Q

Takaysu arteritis

-classic population

A

-esp young Asian females

33
Q

polyarteritis nodosa

-affected population

A

-classically youg adults

34
Q

Kawasaki’s disease

-classic population affected

A

-asian children

35
Q

Takaysu arteritis

-it’s pretty much the same as giant cell arteritis, with what 2 big exceptions?

A
  1. population: 50 in giant cell arteritis
  2. location: aortic arch branches, instead of carotid a branches
37
Q

polyarteritis nodosa

-describe pattern of lesions

A

“string of pearls”

  • lesions of varying stages at once:
  • early lesions of transmural inflammation with fibrinoid necrosis–this causes stretching, aneurysm of vessel (‘pearls’)
  • late lesions that are healed, with fibrosis (‘string’)
38
Q

temporal giant cell arteritis:

treatment

A
  • corticosteroids
  • treat immediately if suspected–don’t wait for biopsy results. Can lead to blindness!
39
Q

Buerger’s disease

-what risk factor associated with?

A
  • heavy smoking. highly associated
  • Tx is to stop smoking
40
Q

“pulseless disease”

-what is this referring to?

A

Takaysu arteritis.

-weak/absent pulses in upper extremity

42
Q

temporal giant cell arteritis:

-symptoms (classic 3)

A
  1. headache (temporal a)
  2. visual disturbances (ophthalmic a)–can lead to blindness!
  3. jaw claudication
    - flu like symptoms often present, ESR elevated
43
Q

microscopic polyangiitis

-tx

A

-corticosteroids, cyclophosphamide

7 Vascular Pathology (5 decks)

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