Inflammatory Myopathies Flashcards

1
Q

Dermatomyositis, polymyositis, and inclusion body myositis are all examples of…

A

inflammatory myopathies

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2
Q

How common are inflammatory myopathies?

A

Uncommon, 1 in 100,000 annually

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3
Q

Are inflammatory myopathies suppurative?

A

Nonsuppurative inflammation

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4
Q

Inflammatory myopathies have what four characteristics?

A
  1. Presence of inflammatory cells
  2. Necrosis and phagocytosis of muscle fibers
  3. A mixture of regenerating and atrophic fibers
  4. Fibrosis
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5
Q

Which muscles experience weakness with inflammatory myopathies?

A

Symmetrical proximal muscles (shoulders, hips, torso) (can eventually go distal)

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6
Q

How do inflammatory myopathy symptoms progress over time?

A

Gradually increasing symptoms over weeks or months
Progressive difficulties with activities of daily living (lifting object, climbing stairs, combing hair, holding head up)

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7
Q

Inflammatory myopathy lab results show increased…

A

serum levels of muscle-derived enzymes, ESR, and maybe C-reactive protein

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8
Q

Inflammatory myopathies are commonly comorbid with…

A

other autoimmune conditions (antibodies tested to determine which)

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9
Q

How are inflammatory myopathies generally treated?

A

Autoimmune treatments:

  • Corticosteroids
  • Immunosuppressant drugs (DMARDs) or IV immunoglobulin
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10
Q

Which inflammatory myopathy has no effective therapy?

A

Inclusion body myositis

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11
Q

Does dermatomyositis more often affect children or adults?

A

Adults

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12
Q

Dermatomyositis is likely comorbid with…

A

systemic sclerosis or other autoimmune disorders

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13
Q

A patient presents with a rash on their upper eyelids, face, trunk, and some other areas. It is known that this patient has systemic sclerosis , but now there is evidence of necrosis of skeletal muscle tissue and fiber atrophy.
What is the likely diagnosis?

A

Dermatomyositis

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14
Q

What is the etiology of dermatomyositis?

A

Autoimmune

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15
Q

With dermatomyositis, compliment mediated (humoral immunity) antibodies attack ___ of skeletal muscle

A

attack microvasculature of skeletal muscle (angiopathy) (microvasculitis)

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16
Q

With dermatomyositis, ___ antibodies attack microvasculature of skeletal muscle (angiopathy)

A

compliment mediated antibodies (humoral immunity)

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17
Q

Dermatomyositis involves ischemia of ___ which leads to ___

A

ischemia of individual muscle fibers which leads to necrosis of skeletal muscle tissue and fiber atrophy

18
Q

Why might infarcts occur with dermatomyositits?

A

Due to involvement of large intramuscular arteries

19
Q

Is dermatomyositis limited to skeletal muscle?

A

No, a similar process will affect other tissues and organs (ie. diaphragm)

20
Q

What is the etiology of polymyositis?

A

Autoimmune

21
Q

Polymyositis almost always begins after age ___

A

20

22
Q

Which sex is predominantly affected by polymyositis?

A

Females

23
Q

Does polymyositis involve a skin rash or microvasculopathy?

A

No

24
Q

Do polymyositis involve angiopathy?

A

No

25
Q

How are T cells related to polymyositis?

A

T cells directly attack muscle fibers

26
Q

What is represented histologically in polymyositis?

A
  • Macrophages in muscle tissue
  • Necrosis
  • Phagocytosis
  • Regeneration
  • Fibrosis
27
Q

Does polymyositis progress systemically or locally?

A

Systemic progression

28
Q

Polymyositis has similar muscle inflammation as that of which is seen in ___

A

HIV infection

29
Q

What is the nature of degeneration associated with polymyositis?

A

Degeneration is scattered, and not always in the areas of inflammation

30
Q

What is the most common inflammatory myopathy in geriatrics?

A

Inclusion body myositis

31
Q

Inclusion body myositis most commonly presents at what age?

A

Over age 50

32
Q

Which sex is predominantly affected by inclusion body myositis?

A

Males

33
Q

Inclusion body myositis involves the presence of specific antigen on ___

A

sarcolemma

34
Q

How are T cells related to inclusion body myositis?
What is this similar to?

A

T cells attack skeletal muscle fiber; similarly to polymyositis

35
Q

What is found histologically in inclusion body myositis?

A

Basophilic granular inclusions in the cytoplasm or nuclei of involved muscle fibers and near vacuoles

36
Q

Inclusion body myositis primarily involves which regions of the body?

A

Distal muscles: hands and feet

37
Q

A 55-year-old male patient presents with atrophy of skeletal muscle tissue in their hands. Upon biopsy, the involved muscle fibers show basophilic granular inclusions containing intracellular B-amyloid plaque throughout the cytoplasm and nuclei.
What is the diagnosis?

A

Inclusion body myositis

38
Q

What is found in the inclusions of muscles affected by inclusion body myositis?

A
  • B-amyloid plaque
  • Tau proteins and other proteins associated with Alzheimer present
  • Parkin (associated with Parkinson disease) and the prion precursor protein have also been found
39
Q

What are the ESR and CRP levels in someone with inclusion body myositis?

A

Elevated

40
Q

How does inclusion body myositis respond to therapy?

A

Poorly