Muscle Diseases and Vasculitis Flashcards

1
Q

what is polymyalgia rheumatica

A

chronic inflammatory condition characterised by pain and stiffness

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2
Q

who typically gets polymyalgia rheumatica

A

over 50s

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3
Q

how does polymyalgia rheumatica present

A

myalgia in proximal limb girdles - symmetrical
morning stiffness lasting >1 hour
fatigue, weight loss

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4
Q

muscle strength is reduced in polymalgia rheumatica true/false

A

false - no weakness just pain and stiffness

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5
Q

how is polymalgia rheumatica diagnosed

A

clinical diagnosis - CRP and ESR will be raised

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6
Q

which condition is associated with polymalgia rheumatica

A

giant cell arteritis, 50% of patients will develop it

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7
Q

what is the treatment for polymyalgia rheumatica

A

responds rapidly to low dose steroids - 15mg prednisolone daily and gradually reduce dose and symptoms improve

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8
Q

describe the pathogenesis of giant cell arteritis

A

transmural inflammation of blood vessel layers with patchy infiltration by lymphocytes and macrophages - thickens wall and narrows the lumen of vessel

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9
Q

how does giant cell arteritis present

A
visual disturbances 
headache - in temporal lobes 
jaw claudication 
scalp tenderness 
fatigue 
visible tortuous temporal artery possible
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10
Q

what are the investigations for giant cell arteritis

A

temporal artery biopsy - may show patchy inflammation

inflammatory markers raised

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11
Q

how is giant cell arteritis treated

A

corticosteroids, 40mg if no visual disturbances and 60mg if visual disturbances – usually resolves within 2 years

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12
Q

how does polymyositis present

A

symmetrical proximal muscle weakness
insidious onset
obvious atrophy

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13
Q

describe the cutaneous features of dermatomyositis

A

v-shaped rash over chest
heliotrope rash - erythema and swelling around eyes
Gottrons papules - violorosaceous rash on hands at MCP and PIPs

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14
Q

list some extra-articular manifestations of polymyositis

A

interstitial lung disease
oesophageal muscle involvement causing dysphagia
myocarditis

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15
Q

dermatomyositis increases risk greatly of what disease

A

solid organ cancers such as breast, bladder and prostate

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16
Q

which antibodies are present in polymyositis and dermatomyositis

A

anti-jo-1

17
Q

what is the diagnostic investigation for polymyositis

A

muscle biopsy - showing perivascular inflammation and necrosis

18
Q

other than muscle biopsy what other investigations are useful for polymyositis

A

creatinine kinase raised

electromyography

19
Q

how is polymyositis treated

A

corticosteroids in short term with immunosuppression

20
Q

is fibromyalgia an inflammatory condition

A

no - presents with muscle pain and fatigue

21
Q

who typically gets fibromyalgia

A

middle aged women

22
Q

list the clinical features of fibromyalgia

A

myofascial pain and twitching lasting >3 months
poor sleep
cognitive difficulties
skin hyper-reactivity

23
Q

how is fibromyalgia diagnosed

A

no definitive diagnosis - must rule out similar conditions eg SLE, hypothyroidism and RA

24
Q

how is fibromyalgia managed

A

no cure, patient education and atypical analgesics such as gabapentin and pregabalin

25
Q

describe vasculitis

A

inflammation of lining of the blood vessel causing stenosis, occlusion and can lead to ischaemia

26
Q

large cell vasculitis affects which vessels

A

aorta and its main branches - granulomatous inflammation

27
Q

list the two conditions in large cell vasculitis and who typically gets them

A

giant cell arteritis - middle aged people

Takayasu arteritis - younger and asian populations

28
Q

what is the treatment of large cell vasculitis

A

40-60mg of prednisolone daily

29
Q

list some symptoms of small cell vasculitis

A

non-blanching purpuric rash on legs
arthritis
glomerulonephritis
lung opacities on x-ray

30
Q

how is small cell vasculitis divided up

A

ANCA positive and negative vasculitis

31
Q

list some ANCA positive vasculitis conditions

A

granulomatosis with polyangiits - ENT symptoms
eosinophilic granulomatosis with polyangiitis - late onset asthma with raised peripheral eosinophils
microscopic polyangiitis

32
Q

what is Henoch Schonlein Purpura

A

IgA disorder with vasculitis involving small vessels of skin - causing rash
commonly affects children after having URTI and usually self-limiting