Poly/dermatomyositis Flashcards

1
Q

What is poly/dermatomyositis?

A

rare condition characterised by the insidious onset of progressive symmetrical proximal muscle weakness

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2
Q

What is the cause of poly/dermatomyositis?

A
  1. autoimmune mediated inflammation of striated muscl

2. conditions (especially dermatomyositis) are quite often paraneoplastic phenomena

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3
Q

What is the patho of polymyositis?

A

Endomysial inflammation with CD8+ T cells

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4
Q

What does polymyositis usually involve?

A

shoulders

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5
Q

What is the patho of dermatomyositis?

A
  1. Perimysial inflammation with CD4+ T cells.

2. Membrane attack complex causes destruction

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6
Q

What is the presentation of polymyositis?

A

Progressive symmetrical muscle weakness

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7
Q

What are the signs associated with dermatomyositis?

A
  1. Gottron’s papules
  2. facial erthyema
  3. shawl rash
  4. mechanical hands
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8
Q

What are Gottron’s papules in dermatomyositis?

A

rough red papules across knuckles, knees and elbows

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9
Q

What is facial erythema like in dermatomyositis?

A

Heliotrope rash of eyelids

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10
Q

What is shawl rash like in dermatomyositis?

A

shoulders and chest darkening

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11
Q

What are the mechanical hands like in dermatomyositis?

A

Dark and thick irregular patches

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12
Q

What investigations are done?

A

1, Muscle enzymes

  1. EMG
  2. Muscle biopsy
  3. Autoantibodies
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13
Q

What do muscle enzymes show in poly/dermatomyositis?

A

CK and Aldolase evelated

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14
Q

What does EMG show in poly/dermatomyositis?

A

fibrillation potentials

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15
Q

What do muscle biopsy show in poly/dermatomyositis?

A

can confirm diagnosis by visualising inflammation

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16
Q

What do autoantibodies show in poly/dermatomyositis?

A

Anti-SRP, Anti-Mi2, Anti-Jo1 (tRNA synthetase which is associated with interstitial fibrosis)

17
Q

How do you manage this?

A

START CORTICOSTEROIDS- in both acute and maintenance cases

18
Q

What is dermatocytis assoicated with?

A

ovarian cancer