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MSK/Derm > SM 246: Inflammatory Myopathies > Flashcards

SM 246: Inflammatory Myopathies Flashcards

1
Q

What is the most common cause of acquired myopathies?

A

Inflammatory/autoimmune myopathy - these are treatable

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2
Q

What is an inflammatory myopathy?

A

An inflammatory response against normal muscle, supporting stroma, or muscle as one of many target tissues

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3
Q

Is all inflammation in muscle an “autoimmune myopathy”?

A

No - just because you see inflammation on biopsy, doesn’t mean you have inflammation

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4
Q

Do you need inflammation for an “autoimmune myopathy”?

A

Also no, might not catch it on muscle biopsy

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5
Q

What are the 4 broad types of inflammatory myopathy?

A

Polymyositis, Deramtomyositis, Inclusion Body Myositis, and Necrotizing Autoimmune Myopathy

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6
Q

What are the two types of Dermatomyositis?

A

Adult with or without skin involvment, as well as Juvenile

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7
Q

How do all myopathies present in general?

A

Proximal muscle weakness, no sensory complaints, myalgias and other sites of inflammation throughout the body

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8
Q

How do myopathies effect patients?

A

Effect day to day activities like sitting, standing, and applying makeup

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9
Q

What is a myalgia?

A

A muscle pain like running a marathon, sore

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10
Q

Is shortness of breath associated with myopathy?

A

Yes

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11
Q

What should you ask about in ROS for a myopathy?

A

Urine color changes (Rhabdo/tea colored urine), swallowing issues (choking issue), medications (statins)

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12
Q

What is the urine color change you should be worried about in a myopathy?

A

Tea colored urine = rhabdomyolysis

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13
Q

Which medications can cause myopathy?

A

Statins

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14
Q

What medical conditions can cause myositis and myopathy?

A

Connective tissue diseases, sarcoidosis, and renal/liver

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15
Q

What should you look for on physical exam in a myopathy?

A

Muscle bulk/atrophy/fasciculations, check proximal and distal muscles (especially head and neck extensors), Deep Tendon Reflexes (DTRs) and sensory exam

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16
Q

What are fasciculations, and what could they suggest?

A

Twitches, ALS

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17
Q

Why should you assess head and neck extensors in a muscle weakness patient?

A

They may go into respiratory failure if they can’t extend their head and neck

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18
Q

What are DTRs?

A

Deep Tendon Reflexes

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19
Q

What labs should you order in a suspected myopathy?

A

CK, aldolase + lactic acid, urine Myoglobin, ESR and CRP Myositis antibodies EMG/MRI Muscle biopsy

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20
Q

What are ESR and CRP related to?

A

Acute phase inflammation, possibly found in myopathy

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21
Q

How is an MRI useful in a myositis?

A

White areas on an MRI correspond to inflamed muscle and guide biopsy

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22
Q

How does polymyositis present?

A

Symmetric proximal muscle weakness, occasional facial weakness + dysphagia, myalgias/achiness, slow onset (except for SRP)

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23
Q

What type of polymyositis is rapid onset?

A

SRP Polymyositis is fast/few weeks, due to antibodies, other things are slow

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24
Q

How does myositis affect the lung?

A

Myositis often cooccurs with fibrotic lung disease, and should be testing for potential lung disease with Anti-Jo1 antibody

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25
What is the Anti-Jo1 antibody used for?
Tests for potential lung involvement/ILD and urgent pulmonology referral in a myositis patient
26
What nonspecific symptoms does polymyositis produce?
Myalgias, seronegative arthritis, and occasional fever
27
What is a normal CK?
Varies by race/gender - african men have the highest at baseline
28
What disease is CK specific for?
It isn't
29
What does a muscle biopsy look like on a myositis?
Inflammation around fibers, scattered necrosis, internal nuclei (should be periopheral in health myocytes), varied sizes of myocyte fibers
30
How should polymyositis be treated?
Corticosteroids, Azathioprine, Methotrexage, IVIG, Rituximab - all take a while to work
31
How does Dermatomyositis present?
Proximal weakness, dysphagia, heliotrope rash and eczema on the extensor surfaces on joints
32
What does Gottron's sign suggest?
Dermatomyositis
33
What is shawl sign?
A rash that appears on sun exposed areas of the skin, suggesting Dermatomyositis
34
What are comborbids with dermatomyositis?
Interstitial Lung Disease (anti-Jo1+), Malignancy, Esophageal disease
35
How does dermatomyositis effect kids and adults differently?
Dermatomyositis suggests cancer in adults, and GI vasculopathy in kids
36
What part of the immune system drives Polymyositis and how does that compare to Dermantomyositis?
Polymyositis = CD8 driven, Dermatomyositis = MAC driven
37
If you see compliment deposition at the edge of a capillary, what should you think and why?
Compliment deposition = MAC = Dermatomyositis Leads to poor oxygen exchange to muscles
38
What is perifascicular atrophy?
Atrophy of muscle around the edges of capillaries in Dermatomyositis due to compliment deposition inside the edges of capillaries and poor oxygenation
39
How do you treat Dermatomyositis?
Corticosteroids + Azathioprine + Methotrexage + IVIg + Rituximab (Same as Polymyositis)
40
Should cancer be surveilled in kids with Dermantomyositis?
Nope, they tend to develop GI vasculopathy instead of cancer; adults with Dermatomyositis develop cancer and should be screened
41
What is Inclusion Body Myositis?
Distal (not proximal) muscle weakness, asymmetrical, more common in 50+ y/o men
42
Which muscles does Inclusion Body Myositis effect?
Effects distal muscles instead of proximal ones like other myopathies, including: Finger flexors, wrist flexors, quadriceps
43
is Inclusion Body Myositis symmetric or asymmetric, and how rapidly does it develop?
Asymmetric distal muscle weakness with years of onset
44
What labs suggest Inclusion Body Myositis?
Elevated CK, Nerve Conduction Study abnormal
45
How does Inclusion Body Myositis appear on H/E stain?
"Rimmed Vacuoles" + low/no inflammation
46
What does "Rimmed Vacuoles" on a stain suggest?
Inclusion Body Myositis
47
What is found in Rimmed Vacuoles and how would you prove it?
Rimmed Vacuoles contain Amyloid which you can prove with a Congo Red stain Inclusion Body Myositis = Alzheimer's Disease
48
Should steroids be used in Inclusion Body Myositis?
No, Inclusion Body Myositis is driven by Amyloid, not inflammation; use IVIg
49
What is necrotizing myopathy?
A type of myopathy with little to inflammation but lots of degeneration and high CK's, presents acutely
50
What drugs cause necrotizing myopathy?
Statins, which lead to an antibody against HMG CoA Reductase, cause statin induced necrotizing myopathy - not the same thing as normal statin myopathy
51
High CK + low inflammation suggests?
Necrotizing Myopathy like statin or SRP induced necrotizing myopathy
52
Besides statins, what can cause necrotizing myopathy?
SRP (signal reduction particle) and paraneoplastic manifestations
53
How should stating induced necrotizing myopathy be treated?
Treat with heavy and long-term immunotherapy
54
What is SRP Myopathy?
A necrotizing myopathy caused by SRP with a high prevalence in african american women
55
How should SRP Myopathy be treated?
Intense immunotherapy
56
How does SRP Myopathy present?
Rapidly (over days to weeks), causing muscle weakness
57
What is this and what would it suggest?
The white area on the MRI suggests Myositis in a muscle, and can be used to guide a biopsy for an inflammatory myopathy
58
What would this pathology suggest?
Inflammatory cells between myofibers suggests Polymyositis
59
What does this suggest and why?
Polymyositis, due to the influx of inlammatory cells, fibers of small size, and occasional internalized nuclei
60
What is this and what does it suggest?
Gottron's Sign due to the rash on the extensor surface of the elbow, suggesting Dermatomyositis
61
What is this and what does it suggest?
Shawl rash since it clearly looks like the rash is only on sun exposed surfaces not covered by clothes Suggets Dermatomyositis
62
What is this and what does it suggest?
Perivascular inflammation, suggesting Dermatomyositis
63
What is this and what does it suggest?
Rimmed vacuoles, suggesting Inclusion Body Myopathy

MSK/Derm (46 decks)

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