Inflammatory Myopathies Flashcards Preview

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Flashcards in Inflammatory Myopathies Deck (24)
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1
Q

Common clinical findings in inflam myopathies

A
  • Symmetric muscle weakness (PM and DM is proximal IBM is distal)
  • Elevated levels CK (PM and DM > IBM
  • Abnormal EMGs
2
Q

12% occur with malignancy what types?

A

breast CA, adenocarcinoma

3
Q

heart involvement with….

A

conduction abnormalities and arrhythmias, myocarditis, and CAD

4
Q

GI involvement with …

A

Decreased the motility

-weakness of tongue, pharyngeal muscles, esophagus, constipation, diarrhea and stomach pain

5
Q

What are the genetic predisposition to inflamm myopathies

A

HLA-DRB1 and -DQA1

6
Q

General pathogenesis assc with inflamm myopathies

A

(1) Cytokines + autoAbs + complement → endothelium damage → hypoxia → capillary loss
(2) Cytokines + CD8 T cells → ER stress → myofiber damage

→ loss of skeletal muscle fibers

7
Q

AutoAbs assc with iflamm myopathies

A

Anti-synthetase/Jo1
Anti-Signal recognition particle
Anti-Chromodomain helicase DN binding protein 3 and 4

8
Q

Epidemiology difference between
polymyositis
dermatomyositis
inclusion body myositis

A

polymyositis = late teen and 50-60
dermatomyositis = 5-10 and 45-56
inclusion body myositis = 50+ (rare in young people)

9
Q

proximal muscle weakness + polyarthritis + Raynaud’s + RASH

A

Dermatomyositis

10
Q

What part of body does rash in DM typically effect

A

eye lids + periorbital edema
grotton’s patches on knuckles > knees and elbow
scalp

11
Q

Predominantly infiltrate of CD8 T cells and macrophages in muscle fibers + inclusion of vacuolated fiibers (tubulofilamentous inclusions)

CD8 T cells recognize muscle fiber Ag on MHC class I → damage

A

Inclusion Body myositis

12
Q

predominantly peri-vascualr infiltrate of CD4 T cells, macrophages, and DC assc with B cells

A

Dermatomyositis

13
Q

thigh and finger flexors

A

IBM

14
Q

Predominantly infiltrate of CD8 T cells and macrophages into non-necrotic muscle fibers

CD8 T cells recognize muscle fiber Ag on MHC class I → damage

A

Polymyositis

15
Q

contractures of joints can occur are called and seen with?

A

= Mechanics hands in DM

16
Q

DM Rash without myositis

these pts are at risk for…

A

Amyopathic Dermatomyositis

severe ILD

17
Q

purple vasculitic patches over knuckles

Whats dz is this seen with

A

Gottron’s patches in Dermatomyositis (DM)

*SLE rash spares knuckles

18
Q

have calcinosis, cutaneous ulceration and lipodystrophy + proximal muscle weakness + polyarthritis + Raynaud’s + RASH

A

Juvenile Dermatomyositis

in 6-11 yo

19
Q

basophilic rimmed vacuoles

A

inclusion body myositis

20
Q

perivascular infiltration

A

DM

21
Q

muscles affected by IBM

A

distal (thigh and finger flexors)

22
Q

pulm interstitial fibrosis + hardening and fissuring of skin over fingers (Mechanics Hands) + myositis

A

Anti-sythetase syndrome seen in PM and DM

23
Q

genetic predisposition to IBM

A

HLA-B8, DR52, DRQ2

24
Q

Insidious, slowly progressive weakness of mainly distal muscles

A

IBM