Myopathies

Question 1

Polymyositis and dermatomyositis are more common in men/women?

Answer 1

Women (2:1)

Question 2

Myopathies are not associated with a risk of malignancy. True/false?

Answer 2

False (increased risk of paraneoplastic malignancy)

Question 3

Describe the common presenting features of myositis (5)

Answer 3

1) Muscle weakness
2) Insidious onset (worsens over months)
3) Usually symmetrical, proximal muscles
4) Often associated with specific problems e.g. brushing hair
5) Myalgia is in 25-50% but usually mild

Question 4

Gottron’s sign is indicative of what…. Where does it present?

Answer 4

Dermatomyositis

Presents over MCP and PIPs especially

Question 5

Describe the 3 clinical signs of dermatomyositis?

Answer 5

• Shawl sign (on back)
• Gottron’s sign (around MCP and PIP)
• Heliotrope rash (around eyes)

Question 6

Myositis can affect other organs, including: (3)

Answer 6

1) Lung (ILD)
2) Oesophagus (dysphagia/ loss of swallow)
3) Heart (myocarditis)

Question 7

Malignancy is increased in dermatomyositis over polymyositis, true or false?

Answer 7

True

Risk highest in men over 45 years old.

Question 8

Diagnosis of myositis is made primarily following investigations. True/false?

Answer 8

False - mainly by presenting symptoms

Question 9

Describe the 2 types of muscle testing that can be done to investigate myositis?

Answer 9

• Isotonic testing (30 seconds sit to stand test)

- Confrontational testing (direct test the power)

Question 10

Autoantibodies in polymyositis include (2)

Answer 10

• ANA

- Anti-Jo-1

Question 11

Definitive test for polymyositis is

Answer 11

Muscle biopsy

Question 12

Treatment for polymositis is initially….

Answer 12

Glucocorticoids (short onset, move to immunosuppresants later).

Question 13

Inclusion body myositis is a type of polymyositis. True/false?

Answer 13

False it’s often misdiagnosed as polymyositis - it’s also commoner in men.

Question 14

What clinical features distinguish inclusion body myositis from polymyositis?

Answer 14

Inclusion body:

• Weakness asymmetrical
• CK is lower than in polymyositis
• Commoner in men (3:1)
• Responds poorly to corticosteroids

Question 15

Polymyalgia rheumatica occurs almost exclusively in which age group?

Answer 15

> 50 years old

Question 16

PMR is higher in which region?

Answer 16

Northern

Question 17

PMR is associated with which serious condition?

Answer 17

Giant cell arteritis / temporal arteritis (15%)

Question 18

PMR stiffness should last how long?

Answer 18

<30 minutes

Question 19

PMR stiffness presents when?

Answer 19

Morning

Question 20

Jaw claudication and headache are buzzwords for…

Answer 20

Temporal arteritis (claudication is VERY specific for this)

Question 21

The classical visual change in giant cell arteritis is…

Answer 21

Amaurosis fugax (like a black curtain descending around one eye)

Question 22

Is there a specific blood test for GCA?

Answer 22

No - generally inflammatory markers will be raised

Question 23

What’s the “best” test for GCA and its principle drawback?

Answer 23

Temporal artery biopsy. Disease may present with skip lesions.

Question 24

Treatment of PMR (with NO suspicion of GCA) is with what drug & dose?

Answer 24

Prednisolone - 15mg

Question 25

If GCA is suspected in PMR, what dose of steroids is started?

Answer 25

40-60mg

Question 26

Fibromyalgia is an inflammatory disease. True/false?

Answer 26

False

Question 27

Fibromyalgia is commoner in men/women?

Answer 27

Women - around 6x

Question 28

What's a clinical finding for fibromyalgia?

Answer 28

Excessive tenderness on palpation of soft tissue

Question 29

Is there a specific test for fibromyalgia?

Answer 29

No. Patients have excessive tenderness to palpation: there are 18 tender points and patients need to have 11

Question 30

What has been shown to be the most effective mechanism of managing fibromyalgia?

Answer 30

Graded exercise

Question 31

Which drugs can cause myalgia/ muscle wasting?

Answer 31

Statins & steroids

Question 32

Blood tests for myositis (4)

Answer 32

1) CK 2) Inflammatory markers 3) Electrolytes, calcium, PTH, TSH (to rule out other causes) 4) Autoantibodies (ANA and Anti-Jo-1)

Question 33

Non-blood test for myositis?

Answer 33

Electromyography (EMG) showing increased fibrillations.

Question 34

What imaging modality is good in showing response to treatment of myositis?

Answer 34

MRI

Question 35

Non first-line therapies for myositis include

Answer 35

IVIG, IV Rituximab

Question 36

What is the key to making a diagnosis of inclusion body myositis?

Answer 36

Presence of inclusion bodies on muscle biopsy

Question 37

Clinical manifestations of PMR

Answer 37

Ache in shoulder and hip girdle. Morning stiffness. Symmetrical stiffness. Reduced range of movement of shoulders, neck and hips.

Question 38

Clinical sign of GCA

Answer 38

Temporal artery will be enlarged, tender and non-pulsatile.

Question 39

Serious complication of GCA

Answer 39

Irreversible blindness

Question 40

In PMR/GCA, when should steroids begin to be tapered off?

Answer 40

Over 18-24 months

Question 41

What's a "test" for PMR involving a medication?

Answer 41

Give trial 15mg prednisolone. If they have PMR they will have a massive response.

Question 42

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

Answer 42

Fibromyalgia

Question 43

Fibromyalgia may begin after what precipitating factor?

Answer 43

Emotional or Physical Trauma

Question 44

Clinical symptoms of fibromyalgia (7)

Answer 44

1) Pain in neck, shoulders, lower back and chest wall 2) Diffuse and chronic 3) Variable intensity 4) Symptoms worse on exertion 5) Poor sleep and mind fog 6) Pain, parasthesia, IBS. 7) Sensation of swelling

Question 45

What drugs can be used to treat fibromyalgia? (3)

Answer 45

1) Anti-depressants 2) Analgesia 3) Pain modulators (e.g. gabapentin, pregabalin)

Question 46

Non-pharmacological options for fibromyalgia treatment (5)

Answer 46

1) Patient education 2) MDT response 3) Graded exercise programme 4) CBT 5) Complimentary medicine (e.g. acupuncture)

Question 47

A 63 year old man presents with onset of pain and stiffness around his shoulders and hips. Ongoing for around 3 weeks. He now needs help to get out of bed in the morning. Likely diagnosis? Questions and tests needed?

Answer 47

` (morning stiffness, areas of stiffness). Questions: Anything to suggest GCA? Tests: Inflammatory markers, screen autoantibodies (should be negative), temporal biopsy if suggestive of GCA

Question 48

Is muscle power reduced in PMR?

Answer 48

No it is normal

Question 49

A 64 year old man has been noticing a heavy feeling in his arms for about 6 months. He is finding it increasingly tiring climbing the stairs. Likely diagnosis? Questions? Tests needed?

Answer 49

Likely diagnosis: Polymyositis (unless cutaneous signs present then dermatomyositis) due to proximal weakness, weakness in activity (climbing stairs), insidious, gradual onset. Questions: any cutaneous signs? Tests: Antibody screen (Anti-Jo-1 should be positive), inflammatory markers (should be raised), CK (should be raised), EMG on proximal muscles, muscle biopsy.

Question 50

A 26 year old woman is referred with a 2 year history of worsening pain “all over”. She has difficulty performing simple tasks like house work due to fatigue. She finds sleep unrefreshing. Likely diagnosis? Questions? Tests needed? Management?

Answer 50

Fibromyalgia ("all over pain", female sex, difficulty performing simple tasks due to fatigue, unrefreshing sleep). Questions: Any history of emotional or physical trauma Tests: Inflammatory markers (should be normal), autoantibodies (should be none), CK (should be normal). Management: Graded exercise most benefit