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MSK Patrick Stenhouse > Muscle Diseases > Flashcards

Muscle Diseases Flashcards

1
Q

What is polymyalgia rheumatica?

A

A relatively common chronic inflammatory condition causing myalgia at the hip and shoulder girdles

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2
Q

How is polymyalgia rheumatic characterised?

A
  • Proximal myalgia of the hip and shoulder girdles
  • Mornign stiffness lasting > 1 hour
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3
Q

Polymyalgia rheumatica is strongly related to which other condition?

A

Giant cell arteritis

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4
Q

In what age is polymyalgia rheumatica most common?

A

> 50

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5
Q

Which blood tests can be done for polymyalgia rheumatica?

A

There are no specific tests

  • CRP (will be raised)
  • PV/ESR (will be raised)
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6
Q

Symptoms of polymyalgia rheumatic respond very well to which treatment?

A

Low dose steroids

(prenisolone 15mg)

The response is so dramatic that this can be used as a diagnostic tool

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7
Q

What is the treatment oplan for polymyalgia rheumatica involving low dosease steroids?

A

The steroid dose is gradually reduced over around 18 months

By this time the condition will have resolved in most patients

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8
Q

What is the most common form of systemic vasculitis?

A

Giant cell arteritis

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9
Q

Giant cell arteritis commony affects older/younger patients?

A

Older

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10
Q

How is giant cell arteritis characterised histologically?

A
  1. Transmural inflammation of the intima, media and adventitia
  2. Patchy infiltration by lymphocytes, macrophages and multinucleated giant cells (granulomas)
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11
Q

Vessel wall thickening in giant cell arteritis can result in what?

A

Subsequent distal ischaemia

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12
Q

What are the common signs and symptoms of giant cell arteritis?

A
  • Visual disturbaces
  • Headaches
  • Jaw claudication
  • Scalp tenderness
  • Fatigue, malaise and fever may be present
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13
Q

Which condition should always be considered in the differential diagnosis of new-onset headaches in patients >50 with raised inflammatory markers?

A

Giant cell arteritis

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14
Q

Describe the headache patients may experience in giant cell arteritis

A

Continuous

Located in the temporal or occipital areas

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15
Q

Why does jaw cladication occur in giant cell arteritis?

A

There is temporal arteritis which causes subsequent ischaemia of the maxillary artery

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16
Q

Diplopia may be experienced in patients with giant cell arteritis, what is this?

A

Double vision

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17
Q

What is the most definitive test for giant cell arteritis?

A

Temporal artery biopsy

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18
Q

When should a temporal artery biopsy be done for a patient?

A

As soon as giant cell arteritis is suspected

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19
Q

Why is a positive temporal artery biopsy 100% specific, but only 15-40% sensitive for giant cell arteritis?

A

Giant cell arteritis is a diffuse condition and has patchy involvement

This means some segments of the artery will be normal

20
Q

What are the typical biopsy findings in an artery with giant cell arteritis?

A
  1. Mononuclear infiltration
  2. Granulomatous inflammation (multinucleated giant cells)
21
Q

What is the treatment for giant cell arteritis?

A

Corticosteroids

  • Prednisolone 40mg (no visual impairment)
  • Prednisolone 60mg (visual impairment)
22
Q

When should treatment be started for giant cell arteritis?

A

As soon as the condition is clincially suspected

23
Q

Describe the treatment program involving corticosteroids for giant cell arteritis

A

The prednisolone dose is gradually tapered over 2 years

After this time, the condition will have resolved for most patients

24
Q

What is polymyositis?

A

An idiopathic inflammatory myopathy that caused symmetrical proximal muscle weakness

25
Q

How is dermatomyositis different from polymyositis?

A

It has typical cutaneous manifestations as well as the other symptoms

26
Q

Both polymyositis and dermatomyositis are more common in which sex, and which age group?

A

Females

Adults > 20, especially 45-60

27
Q

What is the basic pathogenesis of polymyositis?

A

It is thought that CD8 T cells and macrophages surround healthy muscle tissue and initiate a cytotoxic response

(an autoimmune response to both nuclear and cytoplasmic antibodies in seen in most patients)

28
Q

Which antibodies are associated with polymyositis and which of these are specific for polymositis/dermatomyositis?

A
  1. ANA
  2. Anti-RNP
  3. ANti-Jo-1 (specific)
  4. Anti-SRP (specific)
29
Q

How do patients with polymyositis present and what is the onset of the condition?

A

Symmetrical, proximal muscle weakness in the upper and lower extremities

Some patients also have myalgia

(insidious in onset)

30
Q

Why may dysphagia occur in polymyositis?

A

Secondary to oropharyngeal and oesphageal involvement

(a poor prognostic sign)

31
Q

Which lung condition is commonly found in those with polymyositis that are postive for anti-Jo-1 antibody?

A

Interstitial lung disease

32
Q

Which investigations are suitable for polymyositis/dermatomyositis?

A
  • Inflammatory markers
  • Serum creatinine kinase
  • Autoantibodies
  • MRI
  • Electromyography
  • Muscle biopsy
33
Q

Why is muscle biopsy crucial in polymyositis/dermatomyositis?

A

It can exclude other rare muscle diseases

34
Q

What does a muscle biopsy classically show in polymyositis/dermatomyositis?

A

Muscle fibres in varying stages of inflammation, necrosis and regeneration

35
Q

What is the management of polymyositis/dermatomyositis?

A

Prednisolone (40mg)

Immunosuppressive drugs e.g. azathioprine or methotrexate

36
Q

What are the classical cutaneous features of dermatomyositis?

A
  1. V-shaped rash over chest
  2. Gottron’s papules
  3. Heliotrope rash
37
Q

There is an associated risk of malignancy with dermatomyositis patients, which types of cancer are most common?

A
  • Breast
  • Ovarian
  • Lung
  • Colon
  • Oesophagus
  • Bladder
38
Q

What is fibromyalgia?

A

An unexplained condition causing widespread muscle pain and fatigue

39
Q

Fibromylagia is most common in which sex and age?

A

Females

Young and middle aged

40
Q

What is fibromyalgia thought to be?

A

Disorder of central pain processing

or

Syndrome of central sensitivity

41
Q

Patients with fibromyalgia tend to have a lower threshold of which things?

A
  • Pain
  • Heat
  • Noise
  • Strong odours
42
Q

Fibromyalgia can occur as a primary or secondary condition. When it occurs as a secondary condition, which conditions is it associated with?

A
  1. RA
  2. SLE
43
Q

How does fibromyalgia clinically present?

A
  1. Persistant widespread pain (> 3 months) on bopth sides of the body, above and below the waist and including the axial spine
  2. Fatigue and unrefreshing sleep
  3. Cognitive difficulties
  4. Anxiety, depression, IBS and migraine
44
Q

Which conditions may present similiarly to fibromyalgia?

A
  • Hypothyroidism
  • RA
  • SLE
  • Polymyalgia rheumatica
  • Other inflammatory autoimmune conditions
45
Q

What is the management of fibromyalgia like?

A
  1. Education of self-management techniques
  2. Graded exercise
  3. Atypical analgesia (amitriptyline)
  4. Gapapentin
  5. Pregabalin
  6. Cognitive behavioural therapy

MSK Patrick Stenhouse (60 decks)

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