Muscle Diseases and Vasculitis Flashcards

1
Q

what is polymyalgia rheumatica

A

chronic inflammatory condition characterised by pain and stiffness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

who typically gets polymyalgia rheumatica

A

over 50s

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

how does polymyalgia rheumatica present

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

muscle strength is reduced in polymalgia rheumatica true/false

A

false - no weakness just pain and stiffness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

how is polymalgia rheumatica diagnosed

A

clinical diagnosis - CRP and ESR will be raised

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

which condition is associated with polymalgia rheumatica

A

giant cell arteritis, 50% of patients will develop it

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what are the investigations for giant cell arteritis

A

temporal artery biopsy - may show patchy inflammation

inflammatory markers raised

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

how does polymyositis present

A

symmetrical proximal muscle weakness
insidious onset
obvious atrophy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

list some extra-articular manifestations of polymyositis

A

interstitial lung disease
oesophageal muscle involvement causing dysphagia
myocarditis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

dermatomyositis increases risk greatly of what disease

A

solid organ cancers such as breast, bladder and prostate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

which antibodies are present in polymyositis and dermatomyositis

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
describe vasculitis
inflammation of lining of the blood vessel causing stenosis, occlusion and can lead to ischaemia
26
large cell vasculitis affects which vessels
aorta and its main branches - granulomatous inflammation
27
list the two conditions in large cell vasculitis and who typically gets them
giant cell arteritis - middle aged people | Takayasu arteritis - younger and asian populations
28
what is the treatment of large cell vasculitis
40-60mg of prednisolone daily
29
list some symptoms of small cell vasculitis
non-blanching purpuric rash on legs arthritis glomerulonephritis lung opacities on x-ray
30
how is small cell vasculitis divided up
ANCA positive and negative vasculitis
31
list some ANCA positive vasculitis conditions
granulomatosis with polyangiits - ENT symptoms eosinophilic granulomatosis with polyangiitis - late onset asthma with raised peripheral eosinophils microscopic polyangiitis
32
what is Henoch Schonlein Purpura
IgA disorder with vasculitis involving small vessels of skin - causing rash commonly affects children after having URTI and usually self-limiting