B6-015 Myositis

Question 1

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

Answer 1

similar to normal with lower amplitude waves

Question 2

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

Answer 2

combination therapy

prednisone + azathioprine

Question 3

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

Answer 3

steroid sparing agent (azathioprine)

Question 4

most appropriate treatment for infectious myositis

Answer 4

IV antibiotics and consider surgical drainage

Question 5

[….] is often associated with malignancy

Answer 5

dermatomyositis

Question 6

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

Answer 6

ovarian cancer

Question 7

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

Answer 7

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

Question 8

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

Answer 8

IVIG

Question 9

features consistent with long term steroid use

Answer 9

moon facies
weight gain

Question 10

most common neuromuscular disorder in the world

Answer 10

chronic alcohol related neuropathy

Question 11

2 most common types of painless myopathy

Answer 11

alcohol and steroids

Question 12

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

Answer 12

ingestion of alcohol

Question 13

Answer 13

normal skeletal muscle

**note: uniform connective tissue and nuclei at edges

Question 14

distinguishing feature of myocardium

Answer 14

intercalated disc

Question 15

Answer 15

normal myocardium

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

Question 16

damage to myofibers directly

Answer 16

myopathic injury

Question 17

injury that disrupts muscle innervation

Answer 17

neurogenic injury

Question 18

name some expected skin findings of dermatomyositis

Answer 18

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

Question 19

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

Answer 19

dermatomyositis

Question 20

heliotrope rash and Gottron papules are associated with what autoantibody?

Answer 20

anti-Mi2

Question 21

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

Answer 21

anti-Jo1

Question 22

patients with what autoantibody should not receive methotrexate?

Answer 22

anti-Jo1

Question 23

paraneoplastic and juvenille myositis cases are associated with what autoantibody?

Answer 23

anti-P155/P140

Question 24

CD8 positive T cells are prominent in inflammatory infiltrate

Answer 24

polymyositis

Question 25

on EMG, fewer motor units and increased amplitude indicates

Answer 25

neurogenic lesion

Question 26

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

Answer 26

myopathic lesion

Question 27

most common acquired disorder in patient over 50 years

Answer 27

sporadic inclusion body myositis

Question 28

weakness of distal muscles

Answer 28

sporadic inclusion body myositis

Question 29

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

Answer 29

sporadic inclusion body myositis

Question 30

asymmetrical, non-dominant side affected

Answer 30

sporadic inclusion body myositis

Question 31

dysphagia is more common with

Answer 31

sporadic inclusion body myositis

Question 32

vacuoles in muscle

Answer 32

sporadic inclusion body myositis

Question 33

distribution of weakness for polymyositis

Answer 33

proximal, symmetric

Question 34

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

Answer 34

males

Question 35

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

Answer 35

over 50

Question 36

what is the typical onset of sporadic inclusion body myositis?

Answer 36

insidious

Question 37

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

Answer 37

asymmetric and distal

Question 38

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

Answer 38

normal or less than 10x normal

Question 39

inflammation, rimmed vacuoles, inclusions

Answer 39

sporadic inclusion body myositis

Question 40

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

Answer 40

no

Question 41

is polymyositis more common in males or females?

Answer 41

females

Question 42

what is the typical age of onset of polymyositis?

Answer 42

under 50

Question 43

what is the typical onset of polymyositis?

Answer 43

acute, rapid

Question 44

what is the expected CK levels in polymyositis?

Answer 44

greater than 10x normal

Question 45

biopsy shows inflammation and fiber necrosis

Answer 45

polymyositis

Question 46

does polymyositis generally respond to therapy?

Answer 46

yes

Question 47

associated with anti-SRP

Answer 47

immune mediated necrotizing myopathy

Question 48

associated with malignancy, statin treatment, or viral infection

Answer 48

immune mediated necrotizing myopathy

Question 49

weakness is proximal and severe with CK levels over 3000

Answer 49

immune mediated necrotizing myopathy

Question 50

scattered necrotic fibers with macrophage infiltration and regenerating fibers

Answer 50

immune mediated necrotizing myopathy

Question 51

manifestation of sarcoidosis

Answer 51

granulomatous myositis

Question 52

multinucleated giant cells of the Langhans type with peripherally located nuclei

Answer 52

granulomatous myositis

Question 53

first line treatment for myositis

Answer 53

glucocorticoid

Question 54

inhibits dyhyrofolate reductase

Answer 54

methotrexate

Question 55

advantage of methotrexate over azathioprine

Answer 55

once a week administration

Question 56

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

Answer 56

IVIG

Question 57

targets CD20 positive B cells

Answer 57

rituximab

Question 58

promising treatment for recurrent and resistant dematomyositis and polymyositis

Answer 58

rituximab

Question 59

antimalarial that is 70% effective in controlling skin disease

Answer 59

hydroxychloroquine

Question 60

perifasicular atrophy

Answer 60

dermatomyositis

Question 61

CD 4+ T cells

Answer 61

dermatomyositis

Question 62

CD 8+ T cells

Answer 62

polymyositis

Question 63

endomysial infiltration of lymphocytes

Answer 63

polymyositis

Question 64

perimysial infiltration with perifasicular atrophy on biopsy

Answer 64

dermatomyositis

Question 65

endomysial infiltration with necrotic fibers seen on biopsy

Answer 65

polymyositis