Muscle Disease

Question 1

Who gets polymyositis and dermatomyositis

Answer 1

F:M 2:1; 40-50yo

Question 2

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

Answer 2

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

Question 3

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

Answer 3

muscle weakness

Question 4

How does muscle weakness present?

Answer 4

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

Question 5

What is also seen with dermatomyositis?

Answer 5

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

Question 6

What other organs are affected polymyositis and dermatomyositis?

Answer 6

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

Question 7

What are the constitutional symptoms seen with polymyostiis or dermatomyositis?

Answer 7

fever; weight loss ; Raynauds; non-erosie polyarthritis

Question 8

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

Answer 8

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

Question 9

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

Answer 9

men over 45 yo

Question 10

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

Answer 10

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

Question 11

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

Answer 11

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

Question 12

What autoantibodies are seen with polymyositis and dermatomyositis?

Answer 12

ANA, anti-Jo-1

Question 13

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

Answer 13

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

Question 14

What is seen on electromyography in polymyositis?

Answer 14

increased fibrillations; abnormal motor potentials; complex repetitive discharges

Question 15

What is the definitive test for polymyositis and dermatomyositis?

Answer 15

muscle biopsy

Question 16

What is seen on muscle biopsy in polymyositis?

Answer 16

perivascular inflammation and muscle necrosis

Question 17

What is seen on MRI in pts with polymyositis

Answer 17

muscle inflammation, oedema; fibrosis and calcification

Question 18

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

Answer 18

40mg prednisolone PO

Question 19

How does rituximab work?

Answer 19

B cell depleting drug

Question 20

How do steroid factor into treatment for polymyositis?

Answer 20

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

Question 21

Who gets inclusion body myosiits?

Answer 21

pts >50 years

M:F 3:1

Question 22

What are the differences between inclusion body myositis and polymyosits?

Answer 22

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

Question 23

What is the problem with treating inclusion body myostiis?

Answer 23

responds poorly to treatmetns

Question 24

What age is polymyalgia rheumatica seen in?

Answer 24

almost exclusively in the over 50s

Question 25

What condition is PMR associated with?

Answer 25

temporal arteritis/giant cell arteritis

Question 26

What are the clinical manifestations in PMR?

Answer 26

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

Question 27

How is muscle strength afffected in PMR?

Answer 27

it isnt- muscle strength is normal

Question 28

What causes the reduced movement of joints?

Answer 28

paint and stiffness- NOT WEAKNESS

Question 29

What are the symptoms seen with giant cell arteritis?

Answer 29

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

Question 30

What is seen on blood with giant cell arteritis?

Answer 30

raised ESR; PV;CRP

Question 31

How is giant cell arteritis diagnosed?

Answer 31

temporal artery biopsy

Question 32

What is the treatment ofr PMR?

Answer 32

steroids which are gradually reduced over 18-24 months

Question 33

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

Answer 33

fibromyalgia

Question 34

What is the gender distribution of fibromyalgia?

Answer 34

F:M 6:1

Question 35

What may precipitate fibromyalgia?

Answer 35

emotional or physical trauma

Question 36

What are the clinical manifestions of fibromyalgia?

Answer 36

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

Question 37

What is seen on exam of fibromyalgia?

Answer 37

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

Question 38

What are the treaments for fibromyalgia?

Answer 38

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