Inflammatory Myopathies Flashcards Preview

MS/Rheum > Inflammatory Myopathies > Flashcards

Flashcards in Inflammatory Myopathies Deck (24):
1

Common clinical findings in inflam myopathies

-Symmetric muscle weakness (PM and DM is proximal IBM is distal)
-Elevated levels CK (PM and DM > IBM
-Abnormal EMGs

2

12% occur with malignancy what types?

breast CA, adenocarcinoma

3

heart involvement with....

conduction abnormalities and arrhythmias, myocarditis, and CAD

4

GI involvement with ...

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

5

What are the genetic predisposition to inflamm myopathies

HLA-DRB1 and -DQA1

6

General pathogenesis assc with inflamm myopathies

(1) Cytokines + autoAbs + complement → endothelium damage → hypoxia → capillary loss

(2) Cytokines + CD8 T cells → ER stress → myofiber damage

→ loss of skeletal muscle fibers

7

AutoAbs assc with iflamm myopathies

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

8

Epidemiology difference between
polymyositis
dermatomyositis
inclusion body myositis

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

9

proximal muscle weakness + polyarthritis + Raynaud’s + RASH

Dermatomyositis

10

What part of body does rash in DM typically effect

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

11

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

Inclusion Body myositis

12

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

Dermatomyositis

13

thigh and finger flexors

IBM

14

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

Polymyositis

15

contractures of joints can occur are called and seen with?

= Mechanics hands in DM

16

DM Rash without myositis

these pts are at risk for...

Amyopathic Dermatomyositis

severe ILD

17

purple vasculitic patches over knuckles

Whats dz is this seen with

Gottron’s patches in Dermatomyositis (DM)
*SLE rash spares knuckles

18

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

Juvenile Dermatomyositis

in 6-11 yo

19

basophilic rimmed vacuoles

inclusion body myositis

20

perivascular infiltration

DM

21

muscles affected by IBM

distal (thigh and finger flexors)

22

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

Anti-sythetase syndrome seen in PM and DM

23

genetic predisposition to IBM

HLA-B8, DR52, DRQ2

24

Insidious, slowly progressive weakness of mainly distal muscles

IBM