Muscle Disease

Question 1

What is polymyalgia rheumatica?

Answer 1

Chronic inflammatory condition characterised by proximal muscle pain and morning stiffness

Question 2

How does polymyalgia rheumatica present?

Answer 2

In over 50s

Ache of shoulder girdle and hip girdle (normal strength)

Prolonged morning stiffness which improves with movement

Constitutional symptoms

Question 3

What muscle disease is closely associated with giant cell arteritis (GCA)?

Answer 3

Polymyalgia rheumatica

Question 4

Which symptoms must you ask about in a patient presenting with shoulder/hip ache and morning stiffness?

Answer 4

Headache, jaw claudication, scalp tenderness, visual disturbance

Question 5

What is the treatment of polymyalgia rheumatica?

Answer 5

Low dose steroids (15mg/day)

symptoms improve dramatically

Question 6

What blood tests would you order in suspected polymyalgia rheumatica and what would they show?

Answer 6

Inflammatory markers

Raised

Question 7

What is polymyositis?

Answer 7

Idiopathic inflammatory condition causing symmetrical proximal muscle weakness

Question 8

How does polymyositis present?

Answer 8

More common in women

Insidious onset proximal muscle weakness

May be associated with dysphagia and interstitial lung disease

Question 9

What autoantibodies are associated with polymyositis?

Answer 9

Anti-Jo-1 (particularly associated with interstitial lung disease)

Anti-SRP

Question 10

What blood tests would you order in a patient with suspected polymyositis and what would they show?

Answer 10

Inflammatory markers - raised

Creatinine kinase - dramatically raised

Autoantibodies - Anti-Jo-1, anti-SRP

Question 11

What tests are diagnostic of polymyositis and what do they show?

Answer 11

Electromyography - abnormal

Muscle biopsy - show muscle inflammation, necrosis and regeneration

Question 12

What is the treatment of polymyosisits?

Answer 12

Prednisolone (40mg, responds slowly)

Question 13

What is associated with polymyositis?

Answer 13

Malignancy (should be screened for at diagnosis)

Question 14

What factors would make inclusion body myositis a more likely diagnosis than polymyositis?

Answer 14

Male

Distal limb weakness

CK raised but not as dramatically as in polymyositis

Poor response to steroids

Question 15

What does muscle biopsy show in inclusion body myositis?

Answer 15

Inculsion bodies (white spaces within myocyte)

Question 16

What condition is dermatomyositis clinically very similar to? How does this condition present?

Answer 16

Polymyositis

Proximal muscle weakness

Question 17

What are the names and appearances of the cutaneous signs seen in dermatomyositis?

Answer 17

Gottron’s papules - purple discolouration of knuckles

Heliotrope rash - purple discolouration around eyelids

Shawl sign - rash over superior back and posterior neck

_____ - V-shaped rash over chest

Question 18

What is fibromyalgia?

Answer 18

Idiopathic inflammatory condition characterised by widespread muscle pain and fatigue

Question 19

How does fibromyalgia present?

Answer 19

Widespread muscle pain

Fatigue

Low pain threshold

Sensitivity to noise, smell, heat

Association with depression, emotional trauma, IBS, chronic fatigue syndrome

Question 20

How do you diagnose fibromyalgia?

Answer 20

No specific diagnostic criteria largely a diagnosis of exclusion

Question 21

What must be ruled out before diagnosing fibromyalgia?

Answer 21

Hypothyroidism

Rheumatoid arthriris

Systemic lupus erythematosus

Question 22

How do you manage fibromyalgia?

Answer 22

Mainstay is helping patients to self-manage

Adjuvant analgesia (amitryptiline, gapapentin, pregabalin)

Cognitive behavioural therapy