Muscle Diseases Flashcards

1
Q

What is polymyalgia rheumatica

A

chronic inflammatory condition characteriested by proximal hip and shoulder girdle myalgia with accompanying morning stiffness that lasts >1 hr.

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2
Q

Who gets polymyalgia rheumatica

A

Elderly (v rare under 50 years)

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3
Q

What condition is associated with polymyalgia rhuematica

A

Giant Cell/Temporal arteritis

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4
Q

Is weakness a feature of polymyalgia rheumatica?

A

No! only stiffness

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5
Q

Speed of onset of polymyalgia rheumatica

A

Subacute (<2 weeks)

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6
Q

Ix for polymyalgia rheumatica

A

no specific diagnostic test

ass. with raised CRP and ESR

N.B CK and EMG are NORMAL

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7
Q

Tx of polymyalgia rheumatica

A

15mg prednisolone daily - symptoms dramatically improve

SOMETIMES USED AS DIAGNOSTIC TOOL

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8
Q

Usual course length of Tx for polymyalgia rheumatica

A

around 18 months - symptoms will have resolved in majority of cases

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9
Q

what is polymyositis

A

idiopathic inflammatory myopathy that causes symmetrical proximal muscle weakness

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10
Q

what is dermatomyositis

A

similar to polymyositis but has the addition of cutaneous signs

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11
Q

is polymyositis more common in men or women

A

women > men (2:1)

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12
Q

peak incidence of polymyositis

A

40-60 years

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13
Q

autoantibodies associated with myositis

A

ANA
Anti-Jo-1 antibodies
Anti-SRP antibodies

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14
Q

causes of polymyositis

A
  • autoimmune pathogenesis

- paraneoplastic syndrome
from lung, pancreatic, ovarian, bowel malignancy

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15
Q

is polymositis or dermatomyositis more common as a paraneoplastic syndrome

A

dermatomyositis (15%)

- polymyositis 9%

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16
Q

presentation of polymyositis

A

insidious onset of symmetrical proximal muscle weakness (shoulders and hips)

dysphagia and dysphonia secondary to oesophageal and oropharyngeal involvement

respiratory weakness

17
Q

extra-muscular signs that can present in polymyositis

A
interstitial lung fibrosis 
fever 
arthralgia 
Raynauds 
myocardial involvement (arrhythmias, myocarditis)
18
Q

Cutaneous signs of dermatomyositis

A

Gottron’s papules - red and purple areas over MCP and PIP joints

Heliotrope rash - rash around eyes

Shawl sign - rash over back and V down front of chest

19
Q

diagnostic test for polymyositis

A

muscle biopsy

20
Q

Ix for polymyositis

A

inflammatory markers - raised

serum CK - raised

autoantibodies

MRI (shoes extent of muscle involvement)

EMG (abnormal in 90% )

21
Q

Mx of ongoing polymyositis

A

Oral Corticosteroids - pred 40mg daily
+
Immunosuppression - azothioprine or methotrexate

22
Q

Mx of polymyositis with acute muscle weakness or respiratory depression

A

IV Corticosteroids + IVIG

23
Q

Mx of cutaneous signs in dermatomyositis

A

Hydroxychloroquine (DMARD)

24
Q

what is fibromyalgia

A

unexplained condition causing widespread muscle pain and fatigue

25
Q

conditions associated with fibromyalgia

A
RA 
SLE 
depression 
IBS 
migraine
26
Q

presentation of fibromyalgia

A

> 3 months widespread pain on both sides of the body, above and below the waist

fatigue - disturbed and unrefreshing sleep

cognitive difficulties

anxiety and/or depression, functional impairment of daily living activities

27
Q

Ix for fibromyalgia

A

clinical Dx - no specific Ix

rule out other conditions (RA, SLE, polymyalgia rheumatica)

28
Q

Mx of fibromyalgia

A

self-management, advice

neuropathic analgesia (amitriptylline, gabapentin, pregabalin)

counselling, CBT

29
Q

What is inclusion body myositis

A

idiopathic condition characterised as progressive muscle weakness, which is distal and often asymmetrical

30
Q

what is inclusion body myositis often misdiagnosed as

A

polymyositis

31
Q

inclusion body myositis - more common in M or F?

A

M > F (3:1)

32
Q

causes of inclusion body myositis

A

autoimmune pathogenesis
AND
degenerative pathogenesis

33
Q

Ix for inclusion body myositis

A

CK levels
LOWER THAN POLYMYOSITIS

Muscle biopsy - shows inclusion bodies

34
Q

Tx for inclusion body myositis

A

same as for polymyositis - but nothing is consistently effective

Oral corticosteroids + Immunosuppression (azothioprine)