Muscle Disease Flashcards by Beth Mitchell

Who gets polymyositis and dermatomyositis

F:M 2:1; 40-50yo

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What are the deatures seen on histology of polymyositis and dermatomyositis?

muscle fibre necrosis; degenration and regenration adn an inflam cell infiltrate

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What is the most common presenting feature in polymyositis and dermatomyositis?

muscle weakness

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How does muscle weakness present?

insidiously; usually symmetrial and affects the proximal muscles; specific problems eg brushing hair or climbing hte stairs ; mayb have myalgia (usually mild)

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What is also seen with dermatomyositis?

Gottrons sign- pink macules over PIP and DIP
Heliotrope rash- redness round the eyes
Shawl sign- pink pur;le rash around back and making a V shape over the chest

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What other organs are affected polymyositis and dermatomyositis?

lung- ILD; resp muscles weakness
GI-dysphagia
cardiac- myocarditis

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What are the constitutional symptoms seen with polymyostiis or dermatomyositis?

fever; weight loss ; Raynauds; non-erosie polyarthritis

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What are pts with polymyositis or dermatomyositis also at increased risk of??

cancer- ovarian, breast, stomahc, lung, bladder and colon

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What pts are at greatest risk of cancer with polymyositis and dermatomyositis?

men over 45 yo

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What medical conditions should be asked about with pts with suspected polymyositis and dermatomyositis?

DM and thyroid disease- as can also cause muscle weakness and pain

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What tests can be done on exam with pts wtih muscle pain or weakness?

confrontational testing-direct testing of power and isotonic testing-30s sit to stand test

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What autoantibodies are seen with polymyositis and dermatomyositis?

ANA, anti-Jo-1

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What blood tests are done in pts with suspected polymyositis or dermatomyositis?

muscle enzymes- CK
inflam markers
to exclude other causes- electrlytes, PTH,TSH

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What is seen on electromyography in polymyositis?

increased fibrillations; abnormal motor potentials; complex repetitive discharges

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What is the definitive test for polymyositis and dermatomyositis?

muscle biopsy

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What is seen on muscle biopsy in polymyositis?

perivascular inflammation and muscle necrosis

What is seen on MRI in pts with polymyositis

muscle inflammation, oedema; fibrosis and calcification

What is the dose of steroids given for polymyositis and dermatomyositis?

40mg prednisolone PO

How does rituximab work?

B cell depleting drug

How do steroid factor into treatment for polymyositis?

start along with an immunosuppressant as they work quickly, but are gradullay decreased and the immunosuppressive drug is kept goin

Who gets inclusion body myosiits?

pts >50 years

M:F 3:1

What are the differences between inclusion body myositis and polymyosits?

patients are older; affects men more than women(whereas polymyositis affects females more) IBM has a more insidious onset and the muscles affected tend to be more distal (polymyositis tends to be proximal muscles) and the muscles weakness is ften asymmetrical with IBM; CK levels are lower than in PM

What is the problem with treating inclusion body myostiis?

responds poorly to treatmetns

What age is polymyalgia rheumatica seen in?

almost exclusively in the over 50s

What condition is PMR associated with?

temporal arteritis/giant cell arteritis

What are the clinical manifestations in PMR?

ache in shoulder and hip girdle; morning stiffness; symmetrical; fatigue; anorexia; wt loss and fever; reduced movement

How is muscle strength afffected in PMR?

it isnt- muscle strength is normal

What causes the reduced movement of joints?

paint and stiffness- NOT WEAKNESS

What are the symptoms seen with giant cell arteritis?

granulomatous arteritis of lareg vessles; headaches; scalp tenderness; jaw claudication; visual loss; tender, enlarged, non-pulsatile temporal arteries

What is seen on blood with giant cell arteritis?

raised ESR; PV;CRP

How is giant cell arteritis diagnosed?

temporal artery biopsy

What is the treatment ofr PMR?

steroids which are gradually reduced over 18-24 months

What is the commonest cause of MSK pain in women 22-50 years?

fibromyalgia

What is the gender distribution of fibromyalgia?

F:M 6:1

What may precipitate fibromyalgia?

emotional or physical trauma

What are the clinical manifestions of fibromyalgia?

pain in the neck;shoulders; lower back; chest wall- often diffuse (hurts everywhere- cant place as joitn or bone or muscle pain); worse with ertion, fatiuge and stress; sensation of swelling; fatigue and poor, unrefreshing sleep; headhches; IBS; depression

What is seen on exam of fibromyalgia?

excessive tenderness on palpation of soft tissue - needs to be tenderness in at least 11/18 tender points

What are the treaments for fibromyalgia?

patient education; graded exercise programme; CBT; anti-depressants- tricyclics; analgesia; gabapentin