June5 M2-Inflammatory Myopathies Flashcards

1
Q

differences in what can make you unable to go up the stairs or do a task

A

may be one of these 2 different** things

  • muscle fatigue (weakness)
  • pain
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2
Q

Oxford grade of power testing

A
  • grade 0: no muscle fct
  • grade 1: some visible mvmt
  • grade 2: full ROM NOT against gravity (can move but it’s hard)
  • grade 3: can do the mvmt against gravity
  • grade 4: can do the mvmt against gravity, but if put resistance, will overcome them (can say ‘‘mvmt against SOME resistance, but not against full resistance)
  • grade 5: full force and full ROM (can resist full resistance)
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3
Q

list of the different idiopathic inflammatory myopathies

A
  • polymyositis
  • dermatomyositis most common (including juvenile dermatomyositis)
  • inclusion body myositis
  • myositis associated with neoplasia
  • myositis associated with CTD
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4
Q

cause of PM and DM

A

autoimmune disease

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5
Q

PM and DM sx in common

A
  • symmetric proximal muscle weakness
  • truncal weakness
  • both develop slowly
  • myalgia
  • swallowing and breathing problems
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6
Q

other muscles that can be affected in PM and DM

A

respiratory and pharyngeal muscles (swallowing and breathing problems)

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7
Q

muscles that are never affected in PM and DM

A

eyes, facial

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8
Q

DM: when does the myositis occur

A

often occurs after onset of cutaneous manifestations, rarely before

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9
Q

diff kinds of skin manifestations in DM (from most common to least common)

A
  • pathognomonic
  • highly characteristic
  • characteristic
  • more common in juvenile DM
  • rare in DM
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10
Q

pathognomonic lesion in DM

A

Gottron’s papules (telegenctasias of SKIN over the joint in hand but spares phalanges. joints not affected)

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11
Q

highly charact lesion of DM

A

heliotrophe rash (periorbital (eyes) violaceous rash, edema in eyelids)

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12
Q

charact lesions in DM

A
  • Shawl sign and V sign: macular violaceous red rash upper chest post and ant.
  • mechanic hands (hyperkeratosis, scaling and horizontal fissuring of palms and fingers bilaterally)
  • erythroderma (like shaw sign but more extensive, may go to face, malar region and forehead)
  • nailfold telengectasias (periungal erythema), cuticular overgrowth, nailfold capillaries on magnifying glass
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13
Q

manif more common in juvenile DM

A

cutaneous calcinosis. black and white skin bumps on extensors of hands, elbows, buttocks

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14
Q

some extra-muscular manifestations of idiopathic inflam myopathies in general and important ones

A
  • pulmonary (IMP): hypoventilation, aspiration pneumonia, ILD
  • joints: arthralgias and arthritis
  • heart: arrhythmia, conduction prob, CHF, myocarditis, pericarditis
  • GI: dysphagia** (COMMON)
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15
Q

myositis associated with neoplasma type of IIM is what usually

A

dermatomyositis assoc with malignancy

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16
Q

risk of malignancy in DM

A

greatest risk at first year at dx, risk remains at 3-5 years, risk may go back to normal later.

17
Q

diff types of malignancies in DM assoc with malignancy

A
  • lung
  • ovary
  • breast
  • colon
  • rarely hemato
18
Q

other name for myositis assoc with CTD

A

overlap myositis

19
Q

CTDs seen with myositis sometimes

A
  • scleroderma
  • SLE
  • MCTD
  • RA
  • Sjogren’s
  • are all autoimmune*
20
Q

most important thing for diagnosis of an IIM (the rest is not specific). the thing that can let you make a dx

A

biopsy

21
Q

Abs that are positive in DM and PM (auto-Abs)

A
  • myositis specific autoAbs exist

- also some myositis assoc autoAbs (seen in other diseases)

22
Q

EMG looks at what

A
  • nerve conduction (velocity diminished in disease)
  • response of muscle to needle (stronger in disease)
  • tells if prob is muscle vs nerve*
23
Q

MRI use in IIM

A
  • to find areas of edema, muscle inflammation (where to do the bx)
  • also for fibrosis, calcification
24
Q

IIM (DM) affects what region of the muscles

A

areas between fasicles (groups of muscle fibers) where arteries and veins travel

  • PERIfascicular area* NOT THE MUSCLE FIBERS
  • the Abs are against the blood vessels there*
25
Q

pathology of DM

A
  • Abs attack blood vessels
  • MAC, cytokines, immune cells come
  • blod vessels make ICAMs and DCAMs which help immune cells adhere
  • blood vessel destruction
  • perivascular inflammation
  • vasculopathy, not vasculitis*
26
Q

what is done in pathology on the biopsy to dx DM specifically

A

stain specifically for MAC elements (C5 to C9)

27
Q

polymyositis and inclusion body myositis pathology

A

affects the muscle fibers (not perifascicular area)

  • CD8 cells, cytokines released, adhesion molecules on vessels, muscle fibers release cytokines too
  • CD8 recognize MAC (all caused by autoAbs), MHC1 involved
28
Q

(imp?) most common IIMs

A
  • dermatomyositis

- myositis associated with CTD

29
Q

inclusion body myositis charact and symptoms

A
  • in males more
  • muscle weakness, DISTAL
  • very insidious
  • CAN affect facial muscles (still ocular are spared)
  • so different from DM and PM classical location*
30
Q

(imp?) tx in DM and PM

A

steroids (even though long term steroids give toxic myopathy, you determine what’s best for pt)