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M&M Test 4 > B6-023 Dermatomyositis/Scleroderma > Flashcards

B6-023 Dermatomyositis/Scleroderma Flashcards

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1
Q

proximal muscle weakness with skin involvement

A

dermatomyositis

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2
Q

proximal muscle weakness without skin involvement

A

polymyositis

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3
Q

anti-Jo 1 antibody

A

polymyositis

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4
Q

occurs in older adults with distal extremity involvement
responds poorly to treatment

A

inclusion body myositis

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5
Q

have 1 or all of the following findings:
myositis, ILD, raynaud’s, inflammatory arthropathy, mechanic’s hand

A

antisynthetase syndrome (Anti-Jo-1)

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6
Q

early complement deposition with vascular involvement

A

dermatomyositis

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7
Q

B cells and CD 4+ T cells in perifasicular and perivascular infiltration

A

dermatomyositis

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8
Q

endomysial CD8+ T cells around non-necrotic fibers

A

polymyositis/IBM

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9
Q

vacuoles with rim of eosinophilic granules

A

IBM

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10
Q

used to treat dysphagia caused by dermatomyositis

A

IVIG

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11
Q

1st line steroid sparing agents used with high dose steroid for dermatomyositis [2]

A

methotrexate
azathioprine

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12
Q

used for ILD due to dermatomyositis or resistant dermatomyositis [2]

A

rituximab
mycophenolate

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13
Q

treatment of dermatomyositis

A
  1. prednisone- high dose, taper
  2. immunosuppressant
  3. long term management and reassessment
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14
Q

what antibodies have high risk of malignancy associated with dermatomyositis?

A

anti-p155/140
anti-MJ

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15
Q

only skin and soft tissue involvement
no Raynaud’s
kids > adults

A

localized scleroderma

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16
Q

puffiness of hands
Raynauds
sclerodactly
salt and pepper vitiligo
reduced oral aperture

A

scleroderma

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17
Q

triad of pathophysiology of scleroderma

A
  1. autoantibodies
  2. obliterative vasculopathy
  3. fibrosis
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18
Q

main effector cytokine that drives myofibroblast differentiation

A

TGF-b

**causes fibrosis

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19
Q

late vascular manifestation of limited cutaneous scleroderma

A

pulmonary hypertension

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20
Q

all manifestations of diffuse cutaneous scleroderma occur […] in the timeline

A

early

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21
Q

antibody strongly associated with limited cutaneous systemic sclerosis

A

Anti-centromere Ab

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22
Q

CREST syndrome patients have

A

limited cutaneous systemic sclerosis

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23
Q

increased risk of isolated PAH

A

limited cutaneous systemic sclerosis

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24
Q

what antibody is associated with diffuse cutaneous scleroderma?

A

anti-topoisomerase-1 Ab (Scl-70)

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25
Q

what type of scleroderma is more likely to have severe interstitial lung disease?

A

diffuse cutaneous scleroderma

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26
Q

what antibody is associated with skin thickening that might predate Raynaud’s?

A

anti-RNA polymerase III

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27
Q

which antibody in scleroderma is most strongly associated with cancer?

A

anti-RNA polymerase III

28
Q

Anti-SSA (Ro)
and SSB (La) antibodies

A

Sjogren’s syndrome

29
Q

dry eyes and mouth
arthralgia
fatigue
vaginal dryness
upper airway disease

A

Sjogren’s syndrome

30
Q

risk of lymphoma is elevated in […]

A

Sjogren’s

31
Q

[…] must be positive in mixed connective tissue disease

A

anti-RNP

32
Q

skin thickening
raynaud’s
positive ANA/ACA

A

systemic sclerosis

33
Q

limited vs diffuse cutaneous is defined based on

A

extent of skin involvement

34
Q

systemic sclerosis involving only distal arms and legs, and face

A

limited

35
Q

endomysial inflammation
CD 8+ lymphocytes

A

polymyositis

36
Q

myocyte vacuoles on trichrome stain

A

IBM

37
Q

erythematous or violaceous papules over the MCP/IP joints
may have white scaling

A

gottron’s papules

38
Q

first line treatment for dermatomyositis

A

high dose prednisone tapered over months
coupled with methotrexate or azathioprine

39
Q

used in combination with prednisone/azathioprine for treatment of dermatomyositis if dysphagia is present

A

IVIG

40
Q

used for resistant dermatomyositis or in cases with ILD

A

rituximab

41
Q

what antibody is limited cutaneous systemic sclerosis associated with?

A

anti-centromere (ACA)

42
Q

what antibody is most commonly associated with polymyositis?

A

Anti-Jo-1

43
Q

antibodies associated with diffuse scleroderma [2]

A

anti-Scl-70
anti-RNA polymerase III

44
Q

perimysial inflammation with atrophy
CD 4+ t cells

A

dermatomyositis

45
Q

photodistributed facial erythema and edema of eyelids associated with dermatomyositis

A

heliotrope rash

46
Q

thickening, cracking, irregular marks on hands due to hyperkeratinosis of digital skin
associated with dermatomyositis

A

mechanic’s hands

47
Q

[polymyositis/dermatomyositis]
increased risk of malignancy

A

dermatomyositis

48
Q

violaceous rash on the upper back with areas of hyper/hypo pigmentation
associated with dermatomyositis

A

shawl and face rash

49
Q

erythematous papules with white scale over the IP and MCP joints of both hands
associated with dermatomyositis

A

gottron’s papules

50
Q

what would you expect to see on skin biopsy of dermatomyositis?

A

interface dermatitis

**inflammation of the DEJ

51
Q

perifasicular inflammation and atrophy

A

dermatomyositis

52
Q

prominent vasculopathy including gastrointestinal vasculitis leading to GI bleed/perforation

subtype of dermatomyositis

A

juvenille dermatomyositis

53
Q

absent skin involvement
anti-jo-1 antibody

A

polymyositis

54
Q

older adults
distal extremity involvement
poor Rx response

A

IBM

55
Q

why doesn’t raynaud’s typically cause necrosis of the fingers?

A

thermoregulation is done by A-V shunts in the skin

nutritional flow is provided by a separate capillary network that lies deeper

56
Q

what causes primary Raynaud’s?

A

idiopathic

57
Q

what causes secondary raynaud’s?

A

secondary to mixed connective tissue disease

58
Q

is digital ulceration seen in primary or secondary Raynaud’s?

A

secondary

59
Q

treatment of Raynaud’s

A

calcium channel blockers

60
Q

Raynaud’s occurring in younger patients with symmetric involvement and normal labs

A

primary

61
Q

nailfold capillary changes would be associated with […] Raynaud’s

A

secondary

62
Q

anti-centromere ab is associated with

A

limited cutaneous systemic sclerosis

CREST syndrome

63
Q

anti-topoisomerase-1 ab (Scl-70-ab) is associated with

A

diffuse cutaneous systemic sclerosis

64
Q

what antibody in systemic sclerosis is most heavily associated with increased risk of malignancy?

A

anti-RNA polymerase III

65
Q

what antibody in systemic sclerosis is most heavily associated with rapidly progressive skin thickening that may predate the onset of Raynaud’s?

A

anti-RNA polymerase III

66
Q

systemic sclerosis patients with anti-RNA poly III antibodies require vigilant […] monitoring

A

ambulatory BP

**higher risk of Scleroderma Renal Crisis

67
Q

Anti-RNP must be positive to diagnose

A

mixed connective tissue disease

M&M Test 4 (14 decks)

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