Polymyositosis Flashcards

1
Q

what type of myopathy is polymyositosis

A

idiopathic inflammatory

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2
Q

what type of muscle weakness is seen

A

symmetrical and proximal

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3
Q

is it more common in women or men

A

women 2:1

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4
Q

what is the typical age of onset

A

20 and over especially 45-60 year olds

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5
Q

what is the immunology behind this

A

T cell mediated cytotoxic process directed against unidentified muscle antigens
CD8 T cells along with macrophages initially surround healthy nonnectrotic muscle fibres and eventually invade and destroy them

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6
Q

what percentage of patients have an autoimmune response to nuclear and cytoplasmic autoantigens

A

60-80%

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7
Q

what are some of the serum autoantibodies found in polymyositis that are shared with other autoimmune diseases

A

ANA and anti-RNP antibody

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8
Q

what are some of the serum autoantibodies that are unique to polymyositis

A

anti-Jo-1 and anti-SRP antibodies

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9
Q

how do patients with polymyositis usually present

A

symmetrical and proximal weakness in upper and lower extremities of insidious onset commonly noticed as difficulties with particular activities
some patients have myalgia

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10
Q

what are some of the activities polymyositis is noticed with

A

climbing stairs

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11
Q

what can dysphagia occur due to

A

secondary to oropharyngeal and oesophageal involvement

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12
Q

what percentage of patients with polymyositis present with dysphagia

A

33%

poor prognostic sign

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13
Q

what percentage present with interstitial lung disease

A

5-30%

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14
Q

what patients are more likely to present with ILD

A

positive for anti-Jo-1

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15
Q

what investigations are done

A

raised inflammatory markers
creatine kinase raised 10 times the normal level
antibodies including ANA, anti-Jo-1 and anti-SRP, MRI, electromyography, muscle biopsy is crucial

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16
Q

what is MRI useful for

A

localise the extent of muscle involvement

17
Q

what percentage of parents that have abnormal electromyography

A

90%

18
Q

what does muscle biopsy show

A

muscle fibres in varying stages of inflammation, necrosis and regeneration

19
Q

what is the management

A

prednisolone (40mg) combined with immunosuppression drugs (methotrexate or azathioprine)

20
Q

how well does polymyositis respond to treatment

A

well but slowly, 30% have residual weakness, older patients tend too do less well