Muscle Disease Flashcards

1
Q

what are the 4 main inflammatory causes of primary muscle disease?

A

polymyositis
dermatomyositis
inclusion body myositis
polymyalgia rheumatica

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2
Q

How do muscle diseases present?

A

muscle pain
muscle weakness
muscle stiffness
muscle wasting

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3
Q

which inflammatory cause of primary muscle disease has a noticeable increased incidence of malignancy?

A

dermatositis

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4
Q

what muscles are usually affected by dermatomyositis or polymyositis?

A

proximal muscles

eg muscles around pectoral girdle and pevlic girdle

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5
Q

what are the features of cutaneous disease of dermatomyositis?

A

Gottrons sign
Heliotrope rash
Shawl sign

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6
Q

what is Gottrons sign?

A

papules over metacarple phalangeal (MCP) joints and proximal interphalangeal joints

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7
Q

what is a heliotrope rash?

A

a purpley-reddish rash around the eyes

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8
Q

what is a shawl sign?

A

a purpley-reddish rash around the neck

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9
Q

what drugs used in the treatment of patients with a certain cardiovascular risk factor can cause muscle pain and inflammation?

A

statins

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10
Q

what illict drug is known for causing muscle pain and inflammation?

A

cocaine

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11
Q

what 2 types of muscle testing are important features of examining a patient with suspected inflammatory muscle disease?

A
  1. confrontational testing- direct test of power

2. isotonic testing- muscle power with repeated exercise

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12
Q

how might you do a confrontational muscle test?

A

by applying force in the opposite direction to the patient

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13
Q

how might you do a isotonic muscle test?

A

30s sit to stand test

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14
Q

what enzyme is usually raised in polymyositis?

A

creatine kinase

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15
Q

what autoantibodies can be present in polymyositis and dermatomyositis?

A

ANA

anti-Jo-1

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16
Q

what is the gold standard definitive test for polymyositis and dermatomyositis?

A

muscle biopsy

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17
Q

why are MRI’s useful in the diagnosis of polymyositis and dermatomyositis?

A

help pinpoint an affected muscle to biopsy

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18
Q

what is the treatment for polymyositis and dermatomyositis?

A

steroids (only used acutely)
immunosuppressant drugs
IV immunoglobulin
biologic (rituximab)

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19
Q

compare the onset of weakness between polymyositis, dermatomyositis and inclusional body myositis?

A

polymyositis- months
dermatomyositis- months
inclusional body myositis- years

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20
Q

compare the location of weakness between polymyositis, dermatomyositis and inclusional body myositis?

A

polymyositis- proximal muscles
dermatoymyositis- proximal muscles
inclusion body myositis- distal muscles

21
Q

compare the distribution of muscle weakness between polymyositis, dermatomyositis and inclusional body myositis?

A

polymyositis- symmetrical
dermatomyositis- symmetrical
inclusional body myositis- asymmetrical

22
Q

compare the creatine kinase levels between polymyositis, dermatomyositis and inclusional body myositis?

A

polymyositis- elevated
dermatomyositis- normal or elevated
inclusional body myositis- normal or slighly elevated

23
Q

what is the classic muscle biopsy findings of polymyositis?

A

endomysial inflammation with CD8+ T cells

24
Q

what is the classic muscle biopsy findings of dermatomyositis?

A

perimysial inflammation with CD4+ T cells
+
perifascular atrophy

25
Q

what is the classic muscle biopsy findings of inclusional body myositis?

A

rimmed vacules

26
Q

compare the response of treatment between polymyositis, dermatomyositis and inclusional body myositis?

A

polymyositis- good response
dermatomyositis- good response
inclusional body myositis- poor response

27
Q

why is polymyalgia rheumatica an unusual inflammatory muscle disease?

A

biopsy looks normal

only pain and stiffness- no muscle weakness

28
Q

what muscles are affected in polymyalgia rheumatica? (distribution and location)

A

symmetrical proximal muscles around shoulder and hip girdle

29
Q

what is temporal arteritis?

giant cell arteritis

A

inflammation of the lining of the temporal artery

30
Q

what is the major serious consequence of temporal arteritis?

giant cell arteritis

A

permanent blindness

temporal artery suplies the optic nerve

31
Q

what happens to the jaw in temporal arteritis?

giant cell arteritis

A

jaw claudication- aching in muscles when chewing

32
Q

what kind of headache presents in temporal arteritis?

giant cell arteritis

A

unilateral, able to localie

33
Q

what happens to the temporal arteries in temporal arteritis?

(giant cell arteritis?

A

granulomatous inflammation of the temporal artery

artery becomes tender, enlarged (but with lumen narrowing), and non-pulsatile

34
Q

why is the temporal artery non-pulsative in temporal arteritis?

A

vessel wall is so thick due to inflammation:

pulse isn’t conducted through it

35
Q

why is biopsy not totally effective in suspected temporal arteritis?

A

skip lesions

there are patches where there is no granulomatous inflammation

36
Q

what is the treatment of polymyalgia rheumatica? (with no temporal arteritis)

A

low dose steroids

rapid and dramatic response

37
Q

what is the treatment of polymyalgia rheumatica with temporal arteritis?

A

high dose steroids, gradually decreasing dose for 18 -24 months

38
Q

what is the most common cause of chronic musculoskeletal pain?

A

fibromyalgia

39
Q

what may trigger fibromyalgia?

A

emotional or physical trauma

40
Q

how do you diagnose fibromyalgia?

A

absence of abnormal features

inflammatory markers also normal

41
Q

what is the treatment for fibromyalgia?

A
education
graded exercise
complementary medicine (eg acupuncture)
anti-depressants
anti-convulsants
analgesia
42
Q

who tends to get polymyalgia rheumatica?

A

elderly individuals

very rare under 50

43
Q

how do the symptoms of polymyalgia change over the day?

A

morning stiffness which lasts for more than an hour

symptoms improve as the day goes on (and with movement)

44
Q

why can dysphagia occur in polymyositis?

A

secondary to oroparyngeal and esophageal muscle involvement

-poor prognostic sign

45
Q

what is crucial to diagnose polymyositis and dermatomyositis?

A

muscle biopsy

46
Q

what is seen on muscle biopsy of polymyositis?

A

inflammation, necrosis and regeneration

chronic inflammatory infiltrate with cytotoxic T cell

47
Q

what should additionally be screened for in dermatomyositis that isn’t screened for on polymyositis?

A

malignancy

48
Q

what is fibromyalgia thought to be a disorder of?

A

the central pain processing

syndrome of central sensitivity