Muscle Diseases

Question 1

name the elatively common chronic inflammatory condition of unknown etiology that affects elderly individuals (very rare under 50 years)

Answer 1

polymyalgia rheumatica

Question 2

what is polymyalgia rheumatica characterised by?

Answer 2

proximal myalgia of the hip and shoulder girdles with accompanying morning stiffness that lasts for more than 1 hour

Question 3

do symptoms of polymyalgia rheumatica improve as the day goes on and with movement?

Answer 3

yes

Question 4

approximately 15% of patients with polymyalgia rheumatica develop what?

Answer 4

giant cell arteritis (GCA)

40-50% of patients with GCA have associated PMR

Question 5

there is no diagnostic test for PMR but what is seen n blood of those with it?

Answer 5

raised CRP and PV

Question 6

symptoms of PMR respond very dramatically to what drug?

Answer 6

low dose steroids -

prednisolone (15mg)

Question 7

in PMR treatment, you reduce the dose of prednisolone over a period of how long?

Answer 7

18 months

Question 8

name the most common form of systemic vasculitis in older patients of unknown aetiology

Answer 8

giant cell arteritis - GCA

Question 9

what layers of the artery are affected in GCA?

Answer 9

intima
media
adventitia

Question 10

name the cells involved in GCA

Answer 10

patchy infiltration by lymphocytes, macrophages and multinucleated giant cells

Question 11

end result of vessel affected in GCA?

Answer 11

wall thickening and arterial luminal narrowing - distal ischemia

Question 12

common signs and symptoms of GCA?

Answer 12

visual disturbances
headache
jaw claudication
scalp tenderness

Question 13

new-onset headache in patients 50 years of age or older with an elevated erythrocyte sedimentation rate (ESR), CRP and plasma viscosity

diagnosis?

Answer 13

GCA

Question 14

headache in GCA is usually ____________ and located in the _________ or occipital areas

Answer 14

headache in GCA is usually CONTINUOUS and located in the TEMPORAL or occipital areas

Question 15

a GCA patient will notice scalp tenderness when what?

Answer 15

hair combing

Question 16

jaw claudication in GCA is a result of schema of what artery?

Answer 16

maxillary artery

Question 17

visual disturbances of GCA?

Answer 17

unilateral visual blurring
vision loss

painless

occasionally diplopia

Question 18

what is seen on ophthalmoscopy of a GCA patient?

Answer 18

swollen optic disc

Question 19

diagnosis of GCA?

Answer 19

raised inflammatory markers

temporal artery biopsy

Question 20

temporal artery biopsy in GCA is high/low specificity/sensitivity

Answer 20

100% specificity

low sensitivity as patchy

mononuclear infiltration or granulomatous inflammation, usually with multinucleated giant cells

Question 21

GCA treatment?

Answer 21

corticosteroids - prednisolone 40mg

60mg if visual impairment

dose is tapered over 2 years to curation

Question 22

idiopathic inflammatory myopathy that causes symmetrical, proximal muscle weakness

diagnosis?

Answer 22

polymyositis

Question 23

idiopathic inflammatory myopathy that causes symmetrical, proximal muscle weakness WITH cutaneous manifestations

diagnosis?

Answer 23

dermatomyositis

Question 24

polymyositis and dermatomyositis are more common in what sex?

Answer 24

women older 20

especially aged 45-60

Question 25

name the common cell involved in polymyositis

Answer 25

CD8+ T cells

Question 26

name the 4 important autoantibodies in polymyositis

Answer 26

ANA and anti-RNP antibody - SHARED anti-Jo-1 and anti-SRP

Question 27

what do patients with polymyositis present with?

Answer 27

symmetrical, proximal muscle weakness in upper and lower extremities

Question 28

what intra-abdominal abnormality occurs in 1/3 of those with polymyositis?

Answer 28

dysphagia secondary to oropharyngeal and esophageal involvement

Question 29

what lung condition occurs in 5-30% of those with polymyositis?

Answer 29

interstitial lung disease especially those positive for anti-Jo-1 antibody

Question 30

investigations into polymyositis?

Answer 30

raised inflammatory markers raised CK ANA, anti-Jo-1 and anti-SRP EMG abnormalities biopsy is crucial

Question 31

polymyositis treatment?

Answer 31

prednisolone 40mg combined with methotrexate or azathioprine

Question 32

typical rash seen in dermatomyositis: (a) chest (b) face

Answer 32

(a) v shaped rash over chest | (b) heliotrope rash

Question 33

name the papules found in dermatomyositis

Answer 33

gottron's papules

Question 34

what is there an increased risk of in those with polymyositis and dermatomyositis?

Answer 34

malignancy

Question 35

name the common cancers caused by polymyositis and dermatomyositis

Answer 35

``` breast ovarian lung colon oesophagus bladder ```

Question 36

name the unexplained condition causing widespread muscle pain and fatigue

Answer 36

fibromyalgia

Question 37

sex and age that fibromyalgia affects?

Answer 37

young females

Question 38

what other conditions is fibromyalgia seen in?

Answer 38

RA and SLE

Question 39

4 signs and symptoms of fibromyalgia?

Answer 39

persistent widespread pain fatigue cognitive difficulties anxiety/depression and impaired ADLs

Question 40

how is fibromyalgia diagnosed?

Answer 40

clinically - rule out others diagnosis of exclusion treatment is with atypical analgesia - amitriptyline, gabapentin and pregabalin