Muscle Diseases Flashcards

1
Q

what is polymyalgia rheumatica and who does it affect

A

a chronic inflammatory condition of unknown aetiology that affects elderly individuals (>50)

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2
Q

what are symptoms of polymyalgia rheumatica

A

proximal myalgia of hip and shoulder girdles accompanying morning stiffness that lasts more than 1 hour and improves as day goes on

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3
Q

how is polymyalgia rheumatica diagnosed

A

examination for muscle weakness there is no diagnostic test but it is almost always association with raise CRP and PV/ESR

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4
Q

how is polymyalgia rheumatica treated

A

low dose steroids (prednisolone 15mg daily) used as diagnostic tool dose gradually reduced over 18 months

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5
Q

what are complications of polymyalgia rheumatica

A

15% of patient with PMR develop giant cell arteritis (GCA) and 40-50% of patients with GCA have associated PMR this relationship aint fully established

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6
Q

what is giant cell arteritis

A

the most common form of systemic vasculitis in adults unknown aetiology and occurs in older patients

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7
Q

when should giant cell arteritis be considered

A

new onset headache in patients >50 with elevated ESR, CPR or plasma viscosity

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8
Q

what does giant cell arteritis look like histologically

A

transmural inflammation of intima, media and adventitia of affected arteries as well as patchy infiltration of lymphocytes, macrophages and multinucleate giant cells vessel wall thickening can result in arterial luminal narrowing resulting in distal ischaemia

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9
Q

what are symptoms of giant cell arteritis

A

visual disturbances (swollen optic disc) headaches (temporal or occipital areas) jaw claudication (ischaemia or maxillary artery - discomfort on chewing or speaking) scalp tenderness esp on direct palpation (combing hair?) temporal artery thickening fatigue, malaise, fever

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10
Q

how is giant cell arteritis diagnosed

A

raised inflammatory markers temporal artery biopsy

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11
Q

what is biopsy findings of giant cell arteritis

A

mononuclear infiltration or granulomatous inflammation, usually with multinucleate giant cells

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12
Q

how is giant cell arteritis treated

A

prednisolone 40mg if no visual impairment prednisolone 60mg if visual symptoms steroid decrease vascular inflammation

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13
Q

when should treatment be given in giant cell arteritis and how long for

A

as soon as diagnosis suspected - dont delay for biopsy dose gradually tapered over around 2 years

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14
Q

what is polymyositis

A

idiopathic inflammatory myopathy that causes symmetrical, proximal muscle weakness

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15
Q

what is dermatomyositis

A

clinically similar but also has typical cutaneous manifestations

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16
Q

what is prevalence of polymyositis & dermatomyositis

A

more common in women (2:1) and usually affects adults >20 especially those 45-60

17
Q

what is the pathogenesis of polymyositis & dermatomyositis

A

CD8+ T cells along with macrophages surround muscle fibres and eventually invade and destroy them an autoimmune response to nuclear and cytoplasmic auto antigen is detected in 60-80% of patients

18
Q

what antibodies does polymyositis & dermatomyositis share with other autoimmune diseases

A

ANA, anti-RNP

19
Q

what antibodies is unique to polymyositis & dermatomyositis

A

anti-Jo-1 and anti-SRP

20
Q

what is symptoms of polymyositis

A

insidious onset muscle weakness often with particular activity eg climbing stair myalgia dysphagia (poor prognostic sign) ILD occurs in 5-30% (esp for anti-Jo patients)

21
Q

what is symptoms of dermatomyositis

A

similar to polymyositis with V shaped rash over chest, gottrons papules and heliotrope rash

22
Q

how is polymyositis & dermatomyositis diagnosed

A

raised inflammatory markers and CK antibodies MRI may be useful EMG findings abnormal in 90% muscle biopsy crucial as shows fibres in varying stages of inflammation, necrosis and regeneration

23
Q

what are the abnormal EMG findings in polymyositis & dermatomyositis

A

short duration low voltage polyphasic motor unit action potentials with spontaneous fibrillation activity

24
Q

what is the treatment in polymyositis & dermatomyositis

A

prednisolone 40mg combined with immunosuppressive drugs such as methotrexate or azathioprine

25
Q

does polymyositis & dermatomyositis immediately respond to treatment

A

it responds slowly around 30% patients left with some residual weakness (older patients and those who present late do less well)

26
Q

what is a complication of polymyositis & dermatomyositis

A

higher prevalence of malignancy in patients with this common cancers = breast, ovarian, lung, colon, oesophagus and bladder malignancy should be screened for at time of diagnosis

27
Q

what is said to be reason for this complication of polymyositis & dermatomyositis

A

malignancy activates the immune system, which stimulates the development of the autoimmune disease

28
Q

what is fibromyalgia

A

unexplained condition causing widespread muscle pain and fatigue as well as lower threshold of pain, heat, noise and odours

29
Q

who does fibromyalgia affect more

A

young and middle aged woman but can affect anyone

30
Q

fibromyalgia can occur as primary condition or secondary to what

A

25% of patients with RA 50% of patients with SLE

31
Q

what conditions is fibromyalgia associated with

A

depression IBS migraine

32
Q

what are the symptoms of fibromyalgia

A

persistent >3 months widespread pain fatigue = disrupted sleep cognitive difficulties multiple other explained symptoms = anxiety, depression, impairment of activities of daily living (ADL)

33
Q

how is fibromyalgia diagnosed

A

no specific investigation diagnosis of exclusion of everything else

34
Q

how is fibromyalgia treated

A

self management technique advice regarding exercise and activity typical and atypical analgesia including tricyclics (amitriptyline), gabapentin and pregabalin psychological input with techniques such as CBT may be of some help