Inflammatory Myopathies

Question 1

2 characteristics skin lesions of dermatomyositis?
Increased risk for what and give top 3 examples?
Hallmark biopsy?
Lab markers?

Answer 1

The purple upper eye lid rash is called heliotrope and then shawl sign (redness over neck, shoulders upper chest, back)
Malignancy: ovarian, lung and pancreatic
Perimysial inflammation and perifascicular atrophy
Jo1, mi2, pi140/155, CK, MDA5

Question 2

Big difference between polymyositis and DM?
2 lab markers of poly?
Biopsy?

Answer 2

No skin in ply
CK and jo1
Endomysial inflammation with necrotic fibers

Question 3

Management of DM/PM?

What 4 drugs to give for IBM if this doesn’t work?

Answer 3

Corticosteroids

try IvIG, then ritumab, cyclosporine, or tracolimus

Question 4

What two hallmark areas of the body are affected by inclusion body myositis?
Muscle biopsy hallmark?
1 lab marker?

Answer 4

Quads and distal UE (finger flexion)
Rimmed vacuoles
Anti cN1A

Question 5

How do we best characterize Takayasu Arteritis?
Two image modalities to diagnose?
Treatment?
Another name for it?

Answer 5

Granulomatous inflammation of the aorta and its major branches (subclavian) in young women (most common in Asia)
Long smooth tapered stenosis 
MRI or CT
Corticosteroids 
Pulseless disease

Question 6

3 things to remember about HSP?

What is the mechanism of attack for anti GBM?

Answer 6

Palpable purpura, no thrombocytopenia, IGA deposits

Antibodies against basement membrane

Question 7

What is the triad of Behcet syndrome?
What 3 places in the world is it most commonly found?
Where else can there be ulcers?
What is the lab marker?

Answer 7

Recurrent mouth ulcers, genital ulcers, uveitis
Turkey, Asia, Middle East
Distal ileum or cecum
HLAB51

Question 8

Characterize Polyarteritis Nodosa?
Strong association?
80% have the following symptom?
Which part of the body is spared?
How do we make the diagnosis, 2 things.
Gender?

Answer 8

Inflammation of small and medium vessels.
HBV
Vasculitis neuropathy of peripheral nerve, so foot drop
Lungs
Biopsy shows fibrinogen necrosis and need to do angiogram to see micro aneurysm
Male

Question 9

How do we characterize Kawasaki disease?
Age group?
Most common cause of death?
2 things to treat?

Answer 9

Illness in kids that involve skin, mouth, and LN.
Under 5
CAD
IVIG and High dose ASA
Fever, swollen LN, widespread rash, red tongue (Strawberry tongue), red and swollen hands and feet.

Question 10

How do we characterize Granulomatosis with Polyangiitis?
3 areas of the body most commonly affected?
1 lab marker?
Gender?

Answer 10

Granulomatous inflammation of blood vessels.
Lung, Kidney, Respiratory tract with 90% nasal insolvent with saddle nose.
PR3 ANCA
Male

Question 11

What is the hallmark triad of Eosinophilic Granulomatosis with Polyangiitis?
1 lab marker?

Answer 11

Asthma + eosinophils + vasculitis

MPO ANCA

Question 12

Patient population with Thromboangiitis Obliterans?
Diagnosis is made how?
Treatment?

Answer 12

Young males who smoke. Only occurs in smokers
Angiography seeing corkscrew appearance
Stop smoking

Question 13

4 big differences between primary and second raynauds?

Answer 13

Primary is benign, primary is 15-30, secondary is over 30, nailfold test is normal in primary and abnormal in secondary.

Question 14

What is another name for Giant Cell Arteritis?
What two vessels are most commonly affected?
4 clinical features?
Big time association?
Gold standard diagnosis?
Treatment?
What happens if no treatment?

Answer 14

Temporal Arteritis
Cranial arteries and aortic arch 
HA, jaw clots, visual problems, Super high ESR
PMR
Temporal artery biopsy, need an inch
Corticosteroids
Blindness

Question 15

What is Polymyalgia Rheumatic?
Association?
Identifying feature?
What is normal, 3 things?

Answer 15

Proximal severe, symmetrical morning and all day stiffness, soreness and pain
GCA
Feel weak because of the pain, but it isn’t true pain
No inflammation, EMG are normal, muscle enzymes are normal