Rheumatology: Polymyositis & Dermatomyositis

Question 1

Polymyositis (PM) and dermatomyositis (DM) are characterized by ______ and ______.

Answer 1

chronic muscle weakness; infiltration of muscle tissue by chronic inflammatory cells

Question 2

In DM, the inflammatory muscle disease is accompanied by ____.

Answer 2

typical skin rashes

Question 3

PM and DM occur in association with neoplastic disease or in “overlap” with other autoimmune diseases such
as _____, _____, _____, and _____.

Answer 3

scleroderma, mixed connective tissue disease (MCTD), Sjögren’s syndrome, SLE

Question 4

_____ and _____ are typically the main complaints in patients with PM/DM.

Answer 4

Muscle weakness; low endurance

Question 5

What is an erythematous papular rash over the metacarpal or interphalangeal joints called?

Answer 5

Gottron’s papules

Question 6

What are Gottron’s papules called they occur over the elbows and knees?

Answer 6

Gottron’s sign

Question 7

Blue-purple discoloration of the upper eyelid

Answer 7

Heliotrope rash

Question 8

What is V-sign?

Answer 8

Erythematous V-shape rash over the anterior chest

Question 9

What is a shawl-sign rash?

Answer 9

an erythematous rash over the shoulders and upper back

Question 10

What is cracked, dry-appearing skin over the finger pads, particularly on the radial side of the index fingers called?

Answer 10

mechanic’s hands

Question 11

What is Calcinosis cutis?

Answer 11

Subcutaneous calcification; occurs primarily in juvenile dermatomyositis

Question 12

What are Gottron’s papules?

Answer 12

Erythematous papular rash over the metacarpal or interphalangeal joints; can also occur over the elbows and knees (Gottron’s sign)

Question 13

What is a Heliotrope rash?

Answer 13

Blue-purple discoloration of the upper eyelid

Question 14

This is an erythematous V-shape rash over the anterior chest.

Answer 14

V-sign

Question 15

This is an erythematous rash over the shoulders and upper back.

Answer 15

Shawl-sign rash

Question 16

Periungual erythema, nail-fold telangiectasias, and cuticular overgrowth are more common in ___.

Answer 16

DM

Question 17

What is Holster sign?

Answer 17

poikiloderma (atrophic mottled skin) on the lateral aspects of the thighs

Question 18

What are the 7 extra-muscular manifestations of DM/PM?

Answer 18

1. Constitutional symptoms: Unintentional weight-loss, fever, fatigue.
2. Gastrointestinal: Dysphagia; intestinal perforation (caused by mesenteric vasculitis in juvenile DM)
3. Pulmonary: Interstitial lung disease (pulmonary fibrosis —50 to 60% in those with the anti-Jo-1 antibody; aspiration pneumonia.
4. Cardiac: Myocarditis; conduction defects; arrhythmias.
5. Musculoskeletal: Nonerosive inflammatory arthritis.
6. Vascular: Raynaud’s phenomenon; vasculitis (in juvenile DM)
7. Anti-synthetase Syndrome: Constellation associated with antisynthetase antibodies (e.g. anti-Jo-1 antibody): PM or DM presenting with fever (20%), arthritis (50%), mechanic’s hands (30%), Raynaud’s phenomenon (40%), and interstitial lung disease (ILD, 60%).

Question 19

What is the epidemiology of DM/PM?

Answer 19

10 per million

Question 20

What is the female:male ratio of DM/PM?

Answer 20

2-3:1

Question 21

What is the peak age of incidence of DM/PM?

Answer 21

two: childhood and 5th decade

Question 22

What race is predisposed to DM/PM?

Answer 22

African-Americans by 2-3 fold

Question 23

____ will be elevated in the vast majority of patients with active DM/PM.

Answer 23

CPK

Question 24

What indicators, other than CPK, can be used to check for muscle injury?

Answer 24

aldolase, myoglobinemia, LDH, AST, ALT

Question 25

What are the 3 myositis-specific antibodies (MSAs)?

Answer 25

1. aminoacyl-tRNA synthetase antibodies
2. Anti-Mi-2 antibody
3. Anti-signal recognition particle (SRP) antibodies