idiopathic inflammatory myopathies Flashcards

1
Q

definition of idiopathic primary inflammatory myopathies

A

myopathies characterised by chronic inflammation of striated muscle (polymyositis and inclusion body myositis) and skin (dermatomyositis)

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2
Q

aetiology of idiopathic inflammatory myopathies

A

unknown

maybe autoimmune

maybe infective or malignancy trigger in genetically predisposed individual

inflammation around blood vessels

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3
Q

RF of idiopathic inflammatory myopathies

A

polymyositosis - autoimmune connective tissue disorders eg scleroderma

dermatomyositis - bronchial, stomach, breast and ovarian malignancy and auto-Ab anti-Jo-1, anti-Scl, anti-Mi2, HLA linkage to DRW52

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4
Q

epidemiology of idiopathic inflammatory myopathies

A

rare

0.2-1/100000

5-15yrs and 40-60yrs

inclusion body myositis most common >50yrs

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5
Q

symptoms of idiopathic inflammatory myopathies

A

fatigue

malaise

dyspnoea

myalgia and arthralgia can occur

skin rash, raynauds

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6
Q

symptoms of polymyositis and dermatomyositis

A

gradual onset 3-6mo of progressive painless prox muscle weakness (difficulty raising objects above head, rising from chair, climbing stairs)

can hvae dysphagia and dysphonia

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7
Q

symptoms of inclusion body myositis

A

insidious onset (mo-yr)

affectes rising from chair and dexterity of hands

may be dysphagia and neck droop

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8
Q

signs of polymyositis and dermatomyositis

A
  • prox muscle weakness and atrophy affecting upper and lower limbs
  • skin lesions in dermatomyositis
  • associated with myalgia +- arthralgia, or resp weakness
  • fever
  • raynauds
  • interstitial lung fibrosis
  • myocardial involvement - myocarditis, arrhythmias
  • calcinosis in skin
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9
Q

describe the skin lesions in dermatomyositis

A

macular lilac heliotrope rash on upper eyelids and forehead with periorbital oedema

macular rash on chest wall, neck, elbows or knees - shawl sign positive if over back and shoulders

gottrens papules - scaly erythematous raised plaques on finger joints elbows and knees, periungal telangiectasia, ragged cuticles - pathonomonic if high CK and muscle weakness

mechanics hands - fissuring dermatitis of finger pads

nail fold erythema (dialted capillary loops)

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10
Q
A

grotton papules

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11
Q

signs of inclusion body myositis

A

prox and distal muscle weakness and atrophy - wrist and deep finger flexors, quadriceps

weakness of erector spinae and dysphagia is common

starts with quadriceps, finger flexors or pharyngeal muscles

ventral extremity muscle groups are more affected than dorsal or girdle groups

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12
Q

pathogenesis of polymyositis

A

cytotoxic CD8+ T cell infiltrate which appear to recognise an ag on muscle fibre surface

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13
Q

pathology of dermatomyositis

A

probably humorally mediated disorder with perivascular and perifascicular infiltrate in skeletal muscle

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14
Q

pathology of inclusion body myositis

A

T cell inflammatory infiltrate into the skeletal msucle suggests an immune basis

non-inflammed skeletal muscle fibres may contain rimmed vacuoles and amyloid deposits - suggesting a degenerative process

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15
Q

investigations for idiopathic inflammatory myositis

A

careful evaluation for underlying malignancy

blood:

  • FBC - anaemia of chronic disease
  • high ESR (can be normal)
  • high CK, ALT, AST, LDH and aldolase (ie muscle enzymes)
  • auto-Ab titres - anti-Mi2 and anti-Jo1 associated with acute onset and interstitial lung fibrosis that should be treated aggresively

EMG

  • high insertional activity
  • high spontaneous fibrillations
  • abnormal low amplitude short duration polyphasic motor potentials and bozarre high frequency discharges indicative of myopathy

muscle biopsy

  • required for definitive dx
  • inclusion body myositis has typical features of inflammation as well as vacuolated uninflammed muscle fibres and amyloid deposits

CT or MRI

  • look for malignancies
  • MRI - muscle oedema in acute
  • see muscle is white = inflammed
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16
Q

histology for inclusion body myositis

A

ringed vacuoles and intranuclear inclusions

17
Q

ddx for idiopathic inflammatory myositis

A

carcinomatous myopathy

inclusion body myositis

musclar dystrophy

PMR

endocrine/metabolic myopathy eg steroids

rhabdomyolysis

infection eg HIV

drugs - penicillamine, colchicine, statins or chloroquine

18
Q

mx of acute flares of dermatomyositis

A

IV steroids - methylprednisolone
consider IVIG / immunosuppressant (methotrexate) / rituximab

19
Q

chronic mx for combined muscle and skin dermatomyositis

A

First line
1. oral steroid
2. photoprotection (skin lesions worsened by sun)
3. consider rx for skin disease and malignancy

second line
1. methotrexate/azathioprine

20
Q

mx of chronic dermatomyositis with no muscle involvement

A

first line
1. topical steroid - hydrocortisone
2. antipuritic
3. photoprotection

second line
1. antimalarial

3rd line
1. immunosuppressants

21
Q

monitoring for dermatomyositis

A
  1. review for muscle strength and cardiac/pulmonary involvement
  2. monitor for malignancy - at presentation and annually for 3yrs
  3. monitor for drug toxicity
    * steroids
    * antimalarials - vision within 4mo of starting treatmet
    * methotrexate - FBC, LFTs, prescribe folic acid
    * azathioprine - FBCs, LFTs
    * ciclosporin - monitor renal function and BP
    * Anti-inflammatory agents (thalidomide and dapsone): peripheral neuropathy
22
Q

complications of dermatomyositis

A
  • drug toxity
  • cutaneous calcinosis
  • resp tract infections
  • ILD
  • cardiac disease - conduction defects and dysrhythmias
  • dysphasgia - oropharyngeal or oesophageal muscle weakness
  • malignancy
22
Q

px of dermatomyositis

A

good 5 yr survival
risk of severe disability
relapsing remitting
factors associated with worse outcome:
* Delay in treatment for >6 months after symptom onset
* Greater muscle weakness at presentation
* Respiratory muscle involvement
* Interstitial lung disease
* Malignancy
* Dysphagia
* Cardiac involvement.

23
Q

induction of remission of idiopathic inflammatory myopathies

A

IV steroid
methotrexate/azathiorpine/mycophenalate
IVIG
2nd line - ciclosporin/tacrolimus
3rd line - rituximab or cyclophosphamide

if no risk of severe muscle weakness/life threatening complications
* oral steroid
* immunosuppressant
* IVIG

24
Q

maintenance of idiopathic inflammatory myopathies

A
  1. immunosuppressant eg methotrexate
  2. immunosuppressant combination therapy
  3. consider IVIG