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Medical Finals (High Yield) > [Rheum] Vasculitis > Flashcards

[Rheum] Vasculitis Flashcards

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USMLE® Step 1 flashcards
1
Q

what is vasculitis?

A

a group of autoimmune diseases that affect the vessels

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2
Q

what can vasculitis be classified as?

A
  • large vessel disease
  • medium vessel disease
  • small vessel disease
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3
Q

how does vasculitis present?

A

multiple organ systems are affected so pts present as polysymptomatic

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4
Q

what should polysymptomatic pts have?

A

rheumatology work up with:

  • ANA (anti-nuclear antibodies)
  • ANCA (anti-neutrophilic cytoplasmic antibodies)
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5
Q

what should be done if ANA or ANCA came back +ve?

A

further rheumatological ix:
- other antibodies
+/- biopsy (gold standard) of kidney/skin

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6
Q

what is the ANCA associated with granulomatosis with polyangiitis (GPA)?

A

c-ANCA

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7
Q

what are the defining sx of granulomatosis with polyangiitis (GPA)?

A
  • saddle shaped nose

- recurrent epistaxis / sinusitis

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8
Q

what does histology for granulomatosis with polyangiitis (GPA) show?

A

granulomas

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9
Q

what is the ANCA associated with microscopic polyangiitis?

A

p-ANCA

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10
Q

what are the defining sx of microscopic polyangiitis?

A

similar to GPA but less likely to have nasopharyngeal involvement

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11
Q

what does histology for microscopic polyangiitis show?

A

no granulomas

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12
Q

what is the ANCA associated with eosinophilic granulomatosis with polyangiitis (EGPA)?

A

p-ANCA

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13
Q

what are the defining sx of eosinophilic granulomatosis with polyangiitis (EGPA)?

A

asthmatic, quite often requires intense rx for control

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14
Q

what does histology for eosinophilic granulomatosis with polyangiitis (EGPA)?

A

granulomas

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15
Q

what are the common Sx shared by GPA, microscopic polyangiitis and EGPA?

A
  • renal impairment / rapidly progressive GN (RPGN)
  • lung inflammation and haemoptysis
  • skin ulceration (purpura in lower limbs)
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16
Q

what are the common Rx shared by GPA, microscopic polyangiitis and EGPA?

A
  • rx remission: cyclophosphamide and corticosteroids

- once remission: low dose steroids + methotrexate / mycophenolate / azathoprine

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17
Q

what is the most common large vessel vasculitis?

A

Giant Cell Arteritis (GCA)

18
Q

what arteries does GCA affect?

A

large arteries in the head, most commonly temporal artery

19
Q

in whom is GCA rare in?

A

<50 year olds

20
Q

what are the sx of GCA?

A
  • single sided temporal headaches
  • scalp and jaw tenderness (painful shaving, combing hair)
  • visual disturbance: ophthalmic artery involvement [ophthalmic emergency]
21
Q

what should be given urgently in GCA?

A

prednisolone (40-60mg)

60-100mg if visual sx

22
Q

what is the gold standard dx for GCA?

A

temporal artery biopsy

23
Q

heavy smoker, recurrent digit ischaemia. dx?

A

thromboangiitis obliterans (Buerger’s)

24
Q

haemoptysis, haematuria and anti-GBM. dx?

A

Goodpasture’s syndrome

25
Q

new agitation and hallucinations on prednisolone. dx?

A

steroid induced psychosis

26
Q

fever >5 days, conjunctivitis, strawberry tongue. dx?

A

Kawasaki’s disease

27
Q

transmural inflammation, “beads on a string” angiography. dx?

A

polyarteritis nodosa

28
Q

recurrent mouth and genital ulcers not due to infection. dx?

A

Behcet syndrome

29
Q

what should all small vessel disease be treated with initially?

A

cyclophosphamide and corticosteroids

30
Q

what should be performed if there is renal, lung or skin involvement?

A

biopsy

31
Q

what is the common autoantibody present in the following:

  • rheumatoid arthritis
  • Sjögren’s syndrome?
A

rheumatoid factor (RF)

32
Q

what is the autoantibody present in rheumatoid arthritis?

A

anti-CCP

33
Q

what is the common autoantibody present in the following?

  • SLE
  • systemic sclerosis (limited and diffuse)
  • Sjögren’s syndrome
  • poly-/dermato-myositis
  • autoimmune hepatitis
A

ANA

34
Q

what are the autoantibodies present in SLE?

A

anti ds-DNA and anti-Smith (Sm)

35
Q

what is the autoantibody present in limited systemic sclerosis?

A

anti-centromere

36
Q

what is the autoantibody present in diffuse systemic sclerosis?

A

anti-Scl-70

37
Q

what are the autoantibodies present in Sjögren’s syndrome?

A

anti-Ro and anti-La

38
Q

what are the autoantibodies present in poly-/dermato-myositis?

A

anti-Jo and anti-Mi2

39
Q

what is the autoantibody present in drug induced lupus?

A

anti-histone

40
Q

what is the autoantibody present in vasculitides?

A

ANCA

Medical Finals (High Yield) (57 decks)

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