Systemic Autoimmune Disease 2

Question 1

Dermatomyositis, Polymyositis, and Inclusion Body Myositis are grouped together why?

Answer 1

They are inflammatory myopathies

Question 2

Inflammatory myopathies are what?

Answer 2

Group of disorders characterized by immune mediated damage and inflammation of skeletal muscles

Question 3

Classical rash of dermatomyositis (DM)?

Answer 3

Classic rash of upper eyelids with periorbital edema

Question 4

What is Grotton’s lesion in DM?

Answer 4

Erythematous eruption over knuckles, elbows, and knees

Question 5

How do muscles present in DM?

Answer 5

Symmetric weakening of muscles of proximal muscles first

Question 6

What is Polymyositis?

Answer 6

Inflammatory myopathy that involves symmetric proximal muscles and occurs mainly in adults

Question 7

What is Inclusion Body myositis (IBM)?

Answer 7

Distal muscle involvement that can be asymmetric

Question 8

What causes DM?

Answer 8

Immunologic injury to microvasculature mediated by antibodies and complement resulting in myocyte necrosis. B-Cell mediated

Question 9

What causes Polymyositis/IBM?

Answer 9

Cell mediated, CD8 T-cells with increased MHC1 expression

Question 10

Biggest problem with these inflammatory group?

Answer 10

Difficulty moving

Question 11

How do you diagnose these inflammatory problems?

Answer 11

BIOPSY, creatine kinase levels

Question 12

Special pathologic feature for DM?

Answer 12

Inflammatory infiltrates around small blood vessels

Question 13

Special pathologic feature for Polymyositis?

Answer 13

Direct injury to myofibers by CD8 without evidence of vascular injury

Question 14

Special pathologic feature for IBM?

Answer 14

Presence of rimmed vacuoles within myocytes

Question 15

What disease is characterized by widespread musculoskeletal pain/stiffness with multiple tender points, easy fatigability and non-restorative sleep

Answer 15

Fibromyalgia

Question 16

Fibromyalgia involves what in the CSF?

Answer 16

Decreased Serotonin
Low Somatomedin C
Increased Neurotransmitter substance P

Question 17

True or false fibromyalgia is more likely metabolic and neurological than inflammatory or structural?

Answer 17

True

Question 18

What sites can be used as control sites (no tenderness) to test for fibromyalgia?

Answer 18

Dorsum of 3rd digit between PIP and DIP
Medial third of clavicle
Medial malleolus
Forehead

Question 19

What causes inflammation in Serum Sickness?

Answer 19

Immune complexes activate complement which triggers inflammation

Question 20

Kawasaki disease is what type of immune damage reaction and what does it target?

Answer 20

Anti-endothelial cell antibodies

Type 2

Question 21

Goodpoasture’s syndrome is what type of immune reaction and what’s it target?

Answer 21

Anti-basement membrane antibodies

Type 2

Question 22

Antineutrophil Cytoplasmic antibodies (ANCA) signify what type 2 reaction?

Answer 22

Polyarteritis nodosa

Wegener’s

Question 23

Type 3 immune complexes (3)?

Answer 23

DNA anti-DNA immune complexes in SLE
Mixed cryoglobulinemia with IgG, IgM and complement
HBsAg-anti HBsAg immune complexes

Question 24

Type 4 disease involving granulomas?

Answer 24

Granulomatous arteritis

Question 25

What is erythema multiform?

Answer 25

Uncommon self limited lesions involving skin and subcutaneous tissue associated with drugs and infections

Question 26

What is Erythema nodosum?

Answer 26

Panniculitis, inflammation of subcutaneous tissue associated with infections, drugs and no obvious vasculitis

Question 27

Infiltrates of Erythema Nodosum?

Answer 27

Lymphocytes, histiocytes, and multinucleated giant cells

Question 28

What is Erythema induratum?

Answer 28

Primary vasculitis of deep vessels in subcutaneous tissues Necrosis and inflammation within fat Associated with hypersensitivity to TB