Muscle Diseases Flashcards

1
Q

What are two endocrine conditions which can cause myopathy?

A

Hypothyroidism, Cushing’s

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What common medication can cause myopathy?

A

Statins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What causes myositis?

A

No cause, idiopathic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Are polymyositis and dermatomyositis inflammatory conditions?

A

Yes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Which sex are polymyositis and dermatomyositis most common in and by how much?

A

Twice as common in women

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the age of peak incidence of polymyositis and dermatomyositis?

A

45-60

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the difference between polymyositis and dermatomyositis?

A

The same muscle disease, dermatomyositis causes cutaneous features

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What should all patients diagnosed with poly/dermatomyositis be screened for?

A

Malignancy, these conditions increase risk

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Who is dermatomyositis most likely to be a paraneoplastic syndrome in?

A

Males over 50

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What mediates the process of myositis?

A

Cytotoxic T cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What cells surround healthy non-necrotic muscle fibres but then eventually invade and destroy them in myositis?

A

CD8+ T cells and macrophages

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are 4 features of myositis on a biopsy?

A
  • Muscle fibre necrosis - Degeneration - Regeneration - Inflammatory cell infiltrate
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is the main symptom of myositis?

A

Muscle weakness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Is the muscle weakness in myositis of sudden or gradual onset?

A

Gradual, worsening over months

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Which of the following describes the muscle weakness in myositis? a) Symmetrical or Asymmetrical b) Proximal or Distal

A

Symmetrical and proximal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Which muscles are generally affected by myositis?

A

Hip and shoulder girdle

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What do the symptoms of myositis relate to?

A

The functional weakness of the muscles, i.e. difficulty doing particular tasks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Do myositis patients get myalgia?

A

Mild, in some patients

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is Gottron’s Sign and when is it seen?

A

Papules over the joints in the hands- dermatomyositis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What are respiratory features of myositis?

A

ILD/pulmonary fibrosis (5-30% of patients), respiratory muscle weakness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What makes myositis patients more prone to ILD?

A

Being positive for anti-Jo-1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What GI symptom occurs in a third of myositis patients? What does its presence indicate?

A

Dysphagia- a poor prognostic sign

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What cardiac feature can myositis cause?

A

Myocarditis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What are some general symptoms of myositis?

A

Fever, weight loss, Raynaud’s, non-erosive polyarthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Which has a higher risk of malignancy, polymyositis or dermatomyositis?

A

Dermatomyositis (15%) (As opposed to polymyositis at 9%)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What are the cancers which myositis predisposes to?

A

Ovarian, breast, lung, stomach, oesophageal, bladder, colon

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What age/sex is the risk of malignancy in myositis highest?

A

Males > 45

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What % of myositis patients have an increased risk of malignancy? When is this risk highest?

A

25%- highest in the first 5 years following diagnosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What past medical history may be of relevance in myositis?

A

Diabetes, thyroid disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What drugs may be of relevance in myositis?

A

Steroids, statins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What is an example of isotonic testing used for myositis?

A

Sit to stand test- how many times can they do this in 30 seconds

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

What are you looking for in blood tests for myositis?

A
  • Raised CK (sometimes by 10x) - Raised inflammatory markers - Also check electrolytes, Ca++, PTH, TSH
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

What is the main antibody which is specific for myositis?

A

Anti-Jo-1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Auto-antibodies are detected in how many patients with myositis?

A

60-80%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

What other antibody is found in myositis which is sensitive but not specific?

A

ANA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

What is another antibody which is said to be specific for myositis?

A

Anti-SRP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

What is an electromyography?

A

A fine needle is inserted into 2 areas of muscle and a low electric current is passed through to see if it conducts or not

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

What is the definitive, gold standard test for myositis?

A

Muscle biopsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Is a muscle biopsy the same for poly and dermatomyositis?

A

No, they have different patterns

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

MRI can be used for myositis to assess the degree of muscle involvement. What are some things it can show?

A
  • Inflammation - Oedema - Fibrosis - Calcification
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

What is the main treatment for myositis?

A

Start with a high dose of prednisone and then reduce the dose. To reduce the amount of steroids, start an immunosuppressant such as methotrexate or azathioprine but make sure the steroid is on until these have time to work.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Will patients with myositis be on immunosuppressants lifelong?

A

Yes

43
Q

What should be used if myositis patients don’t respond to immunosuppressants?

A

IV immunoglobulin Rituximab

44
Q

What % of myositis patients are left with residual muscle weakness even after treatment?

A

Around 30%

45
Q

Which groups of myositis patients do not respond as well to treatment?

A

Older people and those who presented late

46
Q

What is a problem which can commonly be misdiagnosed as polymyositis?

A

Inclusion body myositis

47
Q

Who does inclusion body myositis occur in? Which sex is most common?

A

Patients > 50, more common in men

48
Q

How is inclusion body myositis different in terms of symptom onset than polymyositis?

A

More insidious onset than polymyositis

49
Q

Which muscles does inclusion body myositis affect?

A

Distal muscles (wrist and finger flexors, quadriceps and anterior tibial muscles)

50
Q

Is the weakness in inclusion body myositis symmetrical or asymmetrical?

A

Asymmetrical

51
Q

Is inclusion body myositis an inflammatory condition?

A

Yes, but not to the degree of polymyositis

52
Q

Is inclusion body myositis an autoimmune condition?

A

No

53
Q

What will CK levels be in inclusion body myositis?

A

Raised, but not as much as in polymyositis

54
Q

What will a muscle biopsy show in inclusion body myositis?

A

Inclusion bodies

55
Q

Does inclusion body myositis respond to treatment?

A

No

56
Q

When is this rash seen?

It continues at the back, what is this known as?

A

Dermatomyositis

Shawl sign

57
Q

When is this sign seen?

What is it known as?

A

Dermatomyositis

Gottron’s Sign

58
Q

When is this rash seen?

What is it known as?

A

Dermatomyositis

Heliotrope rash

59
Q

Is polymyalgia rheumatica inflammatory?

A

Yes

60
Q

What is the cause of polymyalgia rheumatica?

A

Uknown

61
Q

Who does polymyalgia rheumatic occur in?

A

Almost exclusively the over 50s

62
Q

Polymyalgia rheumatica has a higher incidence where?

A

Northern countries

63
Q

What other condition is polymyalgia rheumatica assicated with?

Describe this association.

A

Giant cell arteritis

15% of patients with PMR have GCA

40-50% of patients with GCA have PMR

64
Q

What is polymyalgia rheumatic characterised by?

A

Muscle pain and stiffness

65
Q

Where are the symptoms of polymyalgia rheumatica usually?

Are they symmetrical or asymmetrical?

A

Shoulder and hip girdle

Symmetrical

66
Q

Describe the morning stiffness experienced in polymyalgia rheumatica?

A

Can last up to a few hours and then gets better- improves with movement

67
Q

What are some general features of polymyalgia rheumatica, which can also be caused by GCA?

A

Fatigue, anorexia, weight loss and fever

68
Q

What are some symptoms of GCA?

A

Headache, scalp tenderness, jaw claudication, tender and pulsatile arteries, vision changes

69
Q

Vision loss is permanent in what % of patients with GCA?

A

20%

70
Q

Why does vision loss occur in GCA?

A

The temporal artery supplies the optic nerve

71
Q

How do you diagnose polymyalgia rheumatica?

A

Exclude other diagnoses

Raised inflammatory markers

72
Q

What test should be done ASAP if there are signs of GCA?

A

Temporal artery biopsy

73
Q

Will polymyalgia rheumatica show a change on muscle biopsy?

A

No

74
Q

How long will polymyalgia rheumatica last for?

A

18 months - 2 years

75
Q

What aspect of treatment for polymyalgia rheumatica can actually be used as a diagnostic test?

A

It has a very rapid and dramatic response to treatment with low dose steroids

76
Q

Is the dose of prednisolone kept the same through the whole time a patient has polymyalgia rheumatic/GCA?

A

No, it is tapered down

77
Q

What is the normal starting dose of prednisolone for polymyalgia rheumatica?

A

15mg

78
Q

What dose of prednisolone should be given for polymyalgia rheumatica with a headache and no visual changes?

A

40mg

79
Q

What dose of prednisolone should be given for polymyalgia rheumatica with vision changes?

A

60mg

80
Q

When should you start treatment with high dose prednisolone when there is suspected GCA?

A

Straight away- do not wait for the results of a biopsy

81
Q

GCA involves transmural inflammation of where?

A

The tunica intima, media and adventitia of affected arteries

82
Q

GCA involves infiltration by what cells?

A

Lymphocytes, macrophages and multinucleated giant cells

83
Q

Vessel wall thickening in GCA causes lumen narrowing which results in what?

A

Distal ischaemia, particularly of the temporal artery

84
Q

How might the temporal artery look in GCA?

A

Thickened, prominent and tender to touch

85
Q

When should GCA always be considered as a differential diagnosis?

A

New-onset headahce in over 50s with raised inflammatory markers

86
Q

Describe the headache of GCA and where is it located?

A

Continuous and located in the temporal/occipital areas

87
Q

What is jaw claudication? What causes it?

A

Fatigue or discomfort of the jaw muscles during chewing or prolonged speaking

Caused by ischaemia of the maxillary artery

88
Q

What is the most definitive test for GCA?

A

Temporal artery biopsy

89
Q

What is the sensitivity and specificity of a temporal artery biopsy?

A

100% specificity

15-40% sensitivity

90
Q

Why does a temporal artery biopsy have a low sensitivity for GCA?

A

There is only patchy involvement of the artery i.e. some segments may be normal

91
Q

Is fibromyalgia inflammatory?

A

No

92
Q

What does fibromyalgia cause?

A

Widespread muscle pain and fatigue

93
Q

Who does fibromyalgia occur in?

A

Mostly in young and middle aged women but can affect anyone

94
Q

The cause of fibromyalgia is unknown. What are some potential triggers?

A

Emotional or physical trauma

Poor sleep pattern (can predate the onset of pain)

95
Q

Fibromyalgia patients tend to have a low threshold to what things?

A

Pain, heat, noise and strong odours

96
Q

What is the theory behind the pain of fibromyalgia?

A

Abnormal pain signalling as the pathways become oversensitive

97
Q

Is fibromyalgia a primary or secondary condition or both?

A

Can be primary or secondary (common links with RA and SLE)

98
Q

What are thmain features of fibromyalgia?

A

Persistent (3+ months) of widespread pain

Fatigue and poor, unrefreshing sleep

Cognitive difficulties

Anxiety, depression, impaired activities of daily living

99
Q

What effect does exercise have on fibromyalgia pain?

A

Makes it worse

100
Q

What other conditions can fibromyalgia be associated with?

A

Depression, migraines, IBS

101
Q

How do you diagnose fibromyalgia?

A

Normal inflammatory markers and exclusion of other causes

102
Q

What are the main management options for fibromyalgia?

A

Self-management and understanding of the condition

Advice about graded exercise and activity pacing

CBT

Complementary medicines

Physio/occupational therapy

103
Q

If medications are going to be used for fibromyalgia, what will be used?

A

Amitriptylline, gapapentin, pregabalin

104
Q
A