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BL Unit 3 > PM_DM > Flashcards

PM_DM Flashcards

1
Q

PM/DM

A

Polymositis/dermatoyositis

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2
Q

PM

A

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

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3
Q

DM

A

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

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4
Q

PM/DM ? Assoc.

A

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

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5
Q

PM/DM ? Group I

A

Primary idiopathic PM

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6
Q

PM/DM ? Group II

A

Primary idiopathic DM

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7
Q

PM/DM ? Group III

A

PM/DM assoc w/ neoplasia

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8
Q

PM/DM ? Group IV

A

Childhood DM (PM rare)

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9
Q

PM/DM ? Group V

A

PM/DM assoc w/ another rheum/CT disease

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10
Q

PM/DM ? S/S, muscle

A

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

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11
Q

PM/DM ? S/S, skin

A

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

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12
Q

PM/DM ? S/S, extra-muscular

A

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

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13
Q

PM/DM ? S/S, constitutional

A

weight loss, fever, fatigue

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14
Q

PM/DM ? S/S, GI

A

dysphagia, intestinal perforation (juvenile DM)

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15
Q

PM/DM ? S/S, pulmonary

A

pulmonary fibrosis, aspiration pneumonia

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16
Q

PM/DM ? S/S, cardiac

A

myocarditis, conduction defects, arrhythmias

17
Q

PM/DM ? S/S, MSK

A

nonerosive inflamm arthritis

18
Q

PM/DM ? S/S, Vascular

A

Raynaud’s phenom, vasculitis (juvenile DM)

19
Q

Anti-synthetase Syndrome

A

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

20
Q

PM/DM ? Lab

A

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)

21
Q

PM/DM ? EMG

A

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

22
Q

PM/DM ? MRI

A

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

23
Q

PM ? biopsy

A

inflamm cells throughout fascicle; CD8+ T cells and macs

24
Q

DM ? biopsy

A

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

25
Q

PM/DM ? Incidence

A

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

26
Q

PM/DM ? Pathophysiology, cellular immune

A

(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

27
Q

PM/DM ? Pathophysiology, humoral immune

A

(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)

28
Q

PM/DM ? Pathophysiology, nonimmune

A

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

29
Q

PM/DM ? Pathophysiology, genetic

A

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

30
Q

PM/DM ? Pathophysiology, viral infex

A

eveidence suggests viral infex may trigger PM/DM

31
Q

PM/DM ? Treatment

A

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

32
Q

Inclusion Body Myositis (IBM) ? Incidence

A

Men > women; age > 50

33
Q

Inclusion Body Myositis (IBM) ? S/S

A

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

34
Q

Inclusion Body Myositis (IBM) ? Pathology

A

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

BL Unit 3 (9 decks)

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