Lecture 11 - Myalgias and Myositis Flashcards

1
Q

myalgia

A

symptom of muscle-discomfort: muscle aches, soreness, tenderness, cramps with a normal CK

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2
Q

myopathy

A

muscle weakness (not due to pain), with or without an elevated CK

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3
Q

myositis

A

muscle inflammation
inflammatory disease of muscle
characterized by weakness

elevated muscle enzymes 
CPK (creatine phophokinase) 
AST (aspartate aminotransferase) 
LDH (lactate dehydrogenase) 
Aldolase 

abnormal electromyogram (EMG)

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4
Q

myonecrosis

A

elevated CK due to death of striated muscle cells

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5
Q

rhabdomyolysis

A

rapid death of striated muscle cells often with myoglobinuria or acute renal failure

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6
Q

What are the 5 different groups of myositis?

A

adult polymyositis (AP)
Adult dermatomyositis (DM)
Juvenile Dermatomyositis
associated with malignancy

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7
Q

Polymositis/dermatomysositis

A

subtypes of myositis

autoimmune inflammatory muscle disease

PM: 50-60yo
DM: bimodal 5-15yo and 45-65yo
F > M

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8
Q

How do pts present with polymyositis/dermatomyositis?

A

proximal symmetric m muscle weakness

insidious onset (3-6 months)

minimal or NO pain

can cause weakness of other striated muscles:
+/- dysphonia
+/- dysphagia
+/- respiratory muscle weakness

Dermatomyositis has cutaneous manifestations (including nail changes and subcutaneous calcification)
but other than that these two disease present the same

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9
Q

Gottrons papules

A

rash seen with dermatomyositis

purple erythematous lesions over MCP and IP joints

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10
Q

Heliotrope rash

A

rash seen with dermatomyositis

purpilish rash on eyelids, malar region, forehead, nasolabial folds

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11
Q

V-sign

A

rash seen with dermatomyositis

confluent erythemaotus rash on anterior chest and neck

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12
Q

Shawl sign

A

rash seen with dermatomyositis

erythematous rash over shoulders and upper arms

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13
Q

Holster sign

A

rash seen with dermatomyositis

erythematous rash over lateral aspect of proximal thighs

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14
Q

Mechanics hands

A

rash seen with dermatomyositis

cracking and fissuring of skin over sides of digits

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15
Q

What is the gold standard for polymyositis and dermatomyositis dx?

A

muscle biopsy

but you will also be checking muscle enzymes and autoantibodies

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16
Q

Myositis specific antibodies

A

present in only 30% of PM/DM pts
Jo-1
SRP
Mi-2

80% will have positive ANA (antinuclear antibody) - not myositis specific

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17
Q

Which muscle enzymes will be increased in PM/DM?

A

CPK - can be >10,000 in PM/DM
aldolase elevated
AST
LDH

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18
Q

What will an EMG show for PM/DM?

A

electromyogram

will always be abnormal d/t active inflammation

increased insertional activity with spontaneous fibrillations

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19
Q

Why might you get an MRI for myositis?

A

show sites of edema/inflammation

helpful in guiding muscle biopsy

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20
Q

DM muscle biopsy

A

microvasculature
Perivascular inflammatory infiltrate
perifasicular
CD4 > CD8

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21
Q

Polymyositis muscle biopsy

A

T cell mediated process with myocyte

endomysial CD8

22
Q

Anti synthetase syndrome

A

subgroup of DM/PM with antibodies to RNA synthetase which are responsible for aminoacid binding to tRNA
most commonly associated with Jo-1

mechanics hands
interstitial lung disease

rarely associated with cancer

23
Q

Anti-SRP

A

anti signal recognition peptide

acute severe polymyositis
PAINFUL due to severe myonecrosis
increase risk of heart involvement

poor response to therapy
5 year survival 30%

24
Q

What is the treatment of myositis?

A

Induction:
IV steroids
PO prednisone 1 mg/kg per day

Maintenance: 
methotrexate or Imuran (esp for ILD)
Myocophenolate Mofetil 
Tacrolimus (esp for ILD)
IVIG 
Rituximab
25
Q

Inclusion body myositis

A
white men >50yo
insidious: progressive over months to years 
asymmetric 
DISTAL and proximal muscle weakness 
PAINLESS weakness 
atrophy is prominent 
CPK not very high: 600-800 
negative myositis antibodies
26
Q

Statin myopathy

A

HMG -CoA reductase inhibitor can cause varying degrees of myotoxicity

myalgias associated with muscle cramps and normal CPK
myopathy associated with weakness and normal/mild CPK elevation

statin associated autoimmune myopathy (immune mediated nectrotizing myopathy)

27
Q

What are the risk factors for statin myopathy?

A

higher doses of statin
lipophilic&raquo_space;> hydrophilic (pravastatin is lowest risk)
CYP3A4: cyclosporine, daptomycin, HAART, clarithromycin

28
Q

Steroid induced myopathy

A

steroids have direct catabolic effect on skeletal muscles

increased risk in older pts and critically ill
prednisone >20mg/day
occurs weeks to months after starting steroids
gradual proximal muscle weakness
muscle enzymes are NORMAL
EMG is NORMAL

treatment: discontinue steroids or reduce dose

29
Q

Rhabdomyolysis definition

A

muscle necrosis

30
Q

Rhabdomyolysis triad

A

muscle pain
weakness
dark urine

31
Q

Which labs will be elevated in rhabdomyolysis?

A

CPK

ALT/AST

32
Q

What is the treatment for rhabdomyolysis?

A

IV hydration

33
Q

What causes rhabdomyolysis?

A
muscle trauma/compression
drugs/toxins
infections
electrolyte disorders
inflammatory myositis 
metabolic myopathies
endocrine disorders
malignant hyperthermia
neuroleptic malignant syndrome
34
Q

PMR

A

polymyalgia rheumatica

COMMON
does NOT occur <50yo
Caucasian disease

systemic inflammatory syndrome of elderly characterized by pain/stiffness of shoulder and/or pelvic girdles

35
Q

Core Criteria of PMR

A

Polymyalgia rhuematica

age >50yp (pak 70-80) 
shoulder and hip girdle involvement 
-bilateral aching of shoulder > 2 weeks 
morning stiffness >45 mins ABSENT rheumatoid factor and CCP 
rapid response to corticosteroids
36
Q

Fibromyalgia

A

chronic, non-inflammatory, non-autoimmune, disorder of central pain processing

sxs:
>3 months
multifocal pain (above and below waist and on both sides of the body)
pain induced by palpation >11/18 “tender points”
cognitive problems
psychological distress

37
Q

What are associated sxs seen with fibromyalgia?

A
fatigue 
non restorative sleep 
arthraligas 
cognitive dysfunction 
muscle spasm 
parasthesias 
depression/anxiety
38
Q

How is fibromyalgia dx?

A

dx of exclusion

39
Q

Who gets fibromyalgia?

A

3-4% prevalence

F»>M
usually present in young adult/middle age
rarely present for the first time >50

if >55 then its NOT fibro

40
Q

16 year old female who has muscle pain, shortness of breath and trouble climbing stairs, what is the most likely diagnosis?

A

dermatomyositis

41
Q

65 year old female who has muscle pain, shortness of breath and trouble climbing stairs, what is the most likely diagnosis?

A

dermatomysositis OR polymyositis

42
Q

What age group gets dermatomyositis?

A

biomodal 5-16yo
45 - 60 yo

F>M

43
Q

What age group gets polymyositis?

A

> 50 yo

F > M

44
Q

What age group gets inclusion body myositis?

A

> 50yo

M > F

45
Q

How do we know that fibromyalgia is real?

A

MRI is normal

but fMRI and SPECT show abnormal flow to thalamus and caudate nucleus (signal noxious stimuli) compared to healthy controls and insula, anterior cingulate cortex, and somatosensory cortices in response to painful stimuli

46
Q

What is the management of fibromyalgia?

A
pt education 
analgesia (acetaminophen) 
correct sleep disturbance 
aerobic exercise: Level A evidence 
PT 
CBT
47
Q

Treatment for fibromyalgia

A

SNRI
-duloxetine

TCAs
-amitriptyline

Anticonvulsants
-gabapentin

Muscle relaxant
-cyclobenxaprine

48
Q

What is the prognosis of fibromyalgia?

A

majority of pts continue to experience sxs despite specific treatment

30-40% of pts will experience 40-50% relief from pain

sympathetic pt-physician interaction and organized approach will lead to substantial improvement in many pts

49
Q

A 27 year old female who has muscle pain, and HA for several years, what is the likely diagnosis?

A

fibromyalgia (not GCA d/t the length of this presentation)

50
Q

A pt who reports poorly restorative sleep, with pain above and below the waist, what is the most likely diagnosis?

A

fibromyalgia

51
Q

A pt who wakes his wife in the morning to help him put his shirt on, what is the most likely dx?

A

polymylagia rheumatica (PMR)