PM_DM

Question 1

PM/DM

Answer 1

Polymositis/dermatoyositis

Question 2

PM

Answer 2

chronic muscle weakness and chronic inflamm infiltrate muscle tissue ? CD8+ T cell-mediated muscle injury

Question 3

DM

Answer 3

chronic muscle weakness and chronic inflamm infiltrate muscle tissue + skin rashes ? complement-mediated vasculopathy

Question 4

PM/DM ? Assoc.

Answer 4

Neoplastic disease, Autoimmune diseases (Scleroderma, mixed connective tissue disease [MCTD], Sjogren’s, SLE)

Question 5

PM/DM ? Group I

Answer 5

Primary idiopathic PM

Question 6

PM/DM ? Group II

Answer 6

Primary idiopathic DM

Question 7

PM/DM ? Group III

Answer 7

PM/DM assoc w/ neoplasia

Question 8

PM/DM ? Group IV

Answer 8

Childhood DM (PM rare)

Question 9

PM/DM ? Group V

Answer 9

PM/DM assoc w/ another rheum/CT disease

Question 10

PM/DM ? S/S, muscle

Answer 10

muscle weakness, low endurance, proximal mm early and severe, (half) pain/tenderness, (severe) upper esophagus + respiratory

Question 11

PM/DM ? S/S, skin

Answer 11

Gottron’s papules, heliotrope rash, V-sign, shawl sign, mechanic’s hands, calcinosis cutis, periungual erythema / nail-fold telangiectasias / cuticular overgrowth (DM), holster sign

Question 12

PM/DM ? S/S, extra-muscular

Answer 12

constitutional, GI, Pulm, Cardiac, MSK, Vascular, Anti-synthetase syndrome

Question 13

PM/DM ? S/S, constitutional

Answer 13

weight loss, fever, fatigue

Question 14

PM/DM ? S/S, GI

Answer 14

dysphagia, intestinal perforation (juvenile DM)

Question 15

PM/DM ? S/S, pulmonary

Answer 15

pulmonary fibrosis, aspiration pneumonia

Question 16

PM/DM ? S/S, cardiac

Answer 16

myocarditis, conduction defects, arrhythmias

Question 17

PM/DM ? S/S, MSK

Answer 17

nonerosive inflamm arthritis

Question 18

PM/DM ? S/S, Vascular

Answer 18

Raynaud’s phenom, vasculitis (juvenile DM)

Question 19

Anti-synthetase Syndrome

Answer 19

Antisynthetase Abs (anti-Jo-1) ? fever, arthritis, mechanic’s hands, Raynaud’s phenom, interstitial lung disease (ILD)

Question 20

PM/DM ? Lab

Answer 20

muscle enzymes (elev CPK + aldolase, myoglobinemia, LDH, AST, ALT), specific Abs (anti-Jo-1, Anti-Mi-2, Anti-SRP), assoc Abs (ANA), inflamm (elev ESR)

Question 21

PM/DM ? EMG

Answer 21

myopathic pattern (inc insertional activity and spontaneous fibrillations), decreased amplitude motor unit Aps

Question 22

PM/DM ? MRI

Answer 22

areas of muscle inflamm, edema w/ active myositis, fibrosis, calcinosis

Question 23

PM ? biopsy

Answer 23

inflamm cells throughout fascicle; CD8+ T cells and macs

Question 24

DM ? biopsy

Answer 24

inflamm cells conc in perivascular, spetal, and periphery; CD4+ T cells, B cells, macs, and Dcs

Question 25

PM/DM ? Incidence

Answer 25

1:100,000 per year, F:M 2-3:1, peaks in childhood and 5th decade, African:White 2-3:1

Question 26

PM/DM ? Pathophysiology, cellular immune

Answer 26

(1) CD8+ T cells in muscle, (2) incr T cells in peripheral blood, (3) mononuclear cell response to muscle, (4) lymphocyte cytotox to muscle, (5) incr IL-1, TNF-a, INF-a

Question 27

PM/DM ? Pathophysiology, humoral immune

Answer 27

(1) CD4+ T cells in muscle, (2) immune complex damage (vasculitis, Ig/comp deposition) (3) MSAs (anti-Jo-1, Mi-2, SRP) and MAAs (ANA)

Question 28

PM/DM ? Pathophysiology, nonimmune

Answer 28

(1) Polymyositis (NF-kB: IL-1 and TNF-a: ER overproduces MHC I), (2) tissue hypoxemia

Question 29

PM/DM ? Pathophysiology, genetic

Answer 29

HLA-DRB1, DQA1, DQB1 (when anti-synthetase Abs in PM)

Question 30

PM/DM ? Pathophysiology, viral infex

Answer 30

eveidence suggests viral infex may trigger PM/DM

Question 31

PM/DM ? Treatment

Answer 31

(1) corticosteroids (prednisone), (2) Immunosuppressive/modulator (methotrexate, cycophosphamide, cyclosporine, IVIG, *rituximab), (3) Physical therapy

Question 32

Inclusion Body Myositis (IBM) ? Incidence

Answer 32

Men > women; age > 50

Question 33

Inclusion Body Myositis (IBM) ? S/S

Answer 33

insidious onset muscle weakness, esp. finger flexors and thigh muscles

Question 34

Inclusion Body Myositis (IBM) ? Pathology

Answer 34

Neg. auto-Ab, *rimmed vacuoles and inclusion, resists immunosuppressive drugs