B6-015 Myositis Flashcards

1
Q

what would you expect to see on EMG in a case of inflammatory myopathy?

A

similar to normal with lower amplitude waves

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2
Q

at three years post diagnosis, patients treated with [….] had better functional outcomes and required less prednisone maintenance therapy

A

combination therapy

prednisone + azathioprine

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3
Q

because young patients with myositis require long term steroid therapy, […] should be added to their regime

A

steroid sparing agent (azathioprine)

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4
Q

most appropriate treatment for infectious myositis

A

IV antibiotics and consider surgical drainage

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5
Q

[….] is often associated with malignancy

A

dermatomyositis

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6
Q

in women, there is an association of dermatomyositis and […]

A

ovarian cancer

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7
Q

what would you expect to see on muscle biopsy in a patient with dermatomyositis?

A

-increased complement activation -> complement deposition on capillaries
-microvasulopathy
-perifasicular atrophy

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8
Q

patients presenting with inflammatory myopathies and severe complications should receive […]

A

IVIG

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9
Q

features consistent with long term steroid use

A

moon facies
weight gain

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10
Q

most common neuromuscular disorder in the world

A

chronic alcohol related neuropathy

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11
Q

2 most common types of painless myopathy

A

alcohol and steroids

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12
Q

in chronic alcohol related neuropathy, CK levels are typically normal, but
[…] will result in a rapid increase

A

ingestion of alcohol

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13
Q
A

normal skeletal muscle

**note: uniform connective tissue and nuclei at edges

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14
Q

distinguishing feature of myocardium

A

intercalated disc

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15
Q
A

normal myocardium

**note:1-2 centrally located nuclei, multiple mitochondria, intercalated discs

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16
Q

damage to myofibers directly

A

myopathic injury

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17
Q

injury that disrupts muscle innervation

A

neurogenic injury

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18
Q

name some expected skin findings of dermatomyositis

A

-telangiectasias
-heliotrope rash
-erythema around nail beds, elbows, knees
-tight, shiny, red skin
-maybe pressure point ulcers

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19
Q

upregulation of MHC-1 in myofibers causes an increased expression of ICAM1

A

dermatomyositis

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20
Q

heliotrope rash and Gottron papules are associated with what autoantibody?

A

anti-Mi2

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21
Q

interstitial lung disease and nonerosive arthritis are associated with what autoantibody?

A

anti-Jo1

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22
Q

patients with what autoantibody should not receive methotrexate?

A

anti-Jo1

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23
Q

paraneoplastic and juvenille myositis cases are associated with what autoantibody?

A

anti-P155/P140

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24
Q

CD8 positive T cells are prominent in inflammatory infiltrate

A

polymyositis

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25
Q

on EMG, fewer motor units and increased amplitude indicates

A

neurogenic lesion

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26
Q

on EMG, normal number of motor units with decreased amplitude indicates

A

myopathic lesion

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27
Q

most common acquired disorder in patient over 50 years

A

sporadic inclusion body myositis

28
Q

weakness of distal muscles

A

sporadic inclusion body myositis

29
Q

weakness of wrist, finger flexor muscle, and ankle are most common features

A

sporadic inclusion body myositis

30
Q

asymmetrical, non-dominant side affected

A

sporadic inclusion body myositis

31
Q

dysphagia is more common with

A

sporadic inclusion body myositis

32
Q

vacuoles in muscle

A

sporadic inclusion body myositis

33
Q

distribution of weakness for polymyositis

A

proximal, symmetric

34
Q

sporadic inclusion body myositis is more common in [.males/females]

A

males

35
Q

what is the typical age of onset of sporadic inclusion body myositis?

A

over 50

36
Q

what is the typical onset of sporadic inclusion body myositis?

A

insidious

37
Q

what is the distribution of weakness associated with sporadic inclusion body myositis?

A

asymmetric and distal

38
Q

what is the expected CK levels in sporadic inclusion body myositis?

A

normal or less than 10x normal

39
Q

inflammation, rimmed vacuoles, inclusions

A

sporadic inclusion body myositis

40
Q

is sporadic inclusion body myositis associated with a good response to therapy?

A

no

41
Q

is polymyositis more common in males or females?

A

females

42
Q

what is the typical age of onset of polymyositis?

A

under 50

43
Q

what is the typical onset of polymyositis?

A

acute, rapid

44
Q

what is the expected CK levels in polymyositis?

A

greater than 10x normal

45
Q

biopsy shows inflammation and fiber necrosis

A

polymyositis

46
Q

does polymyositis generally respond to therapy?

A

yes

47
Q

associated with anti-SRP

A

immune mediated necrotizing myopathy

48
Q

associated with malignancy, statin treatment, or viral infection

A

immune mediated necrotizing myopathy

49
Q

weakness is proximal and severe with CK levels over 3000

A

immune mediated necrotizing myopathy

50
Q

scattered necrotic fibers with macrophage infiltration and regenerating fibers

A

immune mediated necrotizing myopathy

51
Q

manifestation of sarcoidosis

A

granulomatous myositis

52
Q

multinucleated giant cells of the Langhans type with peripherally located nuclei

A

granulomatous myositis

53
Q

first line treatment for myositis

A

glucocorticoid

54
Q

inhibits dyhyrofolate reductase

A

methotrexate

55
Q

advantage of methotrexate over azathioprine

A

once a week administration

56
Q

treatment for patients with severe life-threatening weakness such as dysphagia

A

IVIG

57
Q

targets CD20 positive B cells

A

rituximab

58
Q

promising treatment for recurrent and resistant dematomyositis and polymyositis

A

rituximab

59
Q

antimalarial that is 70% effective in controlling skin disease

A

hydroxychloroquine

60
Q

perifasicular atrophy

A

dermatomyositis

61
Q

CD 4+ T cells

A

dermatomyositis

62
Q

CD 8+ T cells

A

polymyositis

63
Q

endomysial infiltration of lymphocytes

A

polymyositis

64
Q

perimysial infiltration with perifasicular atrophy on biopsy

A

dermatomyositis

65
Q

endomysial infiltration with necrotic fibers seen on biopsy

A

polymyositis