Immunopathology II Systemic- Friedlander

Question 1

what is autoimmunity thought to deal with?

Answer 1

failure of central tolerance

Question 2

name some of the 11 things needed for Dx of Lupus? YOU NEED 4!

Answer 2

1. malar rash
2. discoid rash
3. photosensitivity
4. oral ulcers
5. arthritis
6. serositis
7. renal disorder
8. neuro disorder
9. hematological disorder
10. immune (anti-dsDNA, anti-smith, APL ab, false + VDRL for sphilis)
11. Antinuclear Abs (+ANA)

Question 3

ANA patterns: rim

Answer 3

THINK classic systemic lupus, anti-dsDNA

Question 4

ANA patterns: homogenous

Answer 4

fluorescent green all over; THINK drug-induced lupus

Question 5

ANA patterns: speckled

Answer 5

any damn thing

Question 6

ANA patterns: centromere

Answer 6

THINK CREST

Question 7

ANA patterns: nucleolar

Answer 7

Think Sceleroderma

Question 8

how is lupus vasculitis mediated?

Answer 8

immune-complexes

Question 9

what is seen in the spleen with lupus?

Answer 9

adventitial onionskinning appearance

Question 10

what will a lupus band test show you?

Answer 10

granular immunoglobulin and complement at dermal-epidermal jxn

Question 11

what are mouth ulcers in lupus due to?

Answer 11

infarcts from vasculitis

Question 12

what does lupus cause in regards to the heart?

Answer 12

Libman-Sacks endocarditis: sterile thrombi

Question 13

subacute cutaneous lupus is seen when?

Answer 13

anti-Ro, pts have lupus, sjogren’s or RA

Question 14

what does discoid lupus look like?

Answer 14

patches with scales and lost hair

Question 15

today, what kind of drug will cause drug induced lupus?

Answer 15

anti-aTNF Rx; develop rash and anti-dsDNA Ab

Question 16

what occurs in Sjogren’s?

Answer 16

autoimmune disorder with destruction of exocrine glands; lacrimal and salivary especially: dry eyes and mouth

Question 17

what kind of lab findings will be observed with Sjogren’s?

Answer 17

Anti-SSA (anti-Ro), Anti-SSB (anti-La)

Question 18

what is the worst complication that can occur due to Sjogren’s?

Answer 18

malignant lymphoma

Question 19

what is sceleroderma?

Answer 19

systemic sclerosis: excessive fibrosis and collagen deposition throughout body

Question 20

what does organs most affected by scerloderma?

Answer 20

skin, kidneys, lungs (COD), CV, GI

Question 21

what are the 2 kinds of sceleroderma?

Answer 21

1. Diffuse: widespread skin involvement, rapid, early visceral involvement
2. limited: limited skin involvement, slow, limited systemic involvement

Question 22

what is CREST associated with? stand for?

Answer 22

limited scleroderma;
C: calcinosis (in skin)
R: Raynaud’s
E: esophageal dysmotility
S: Sclerodactyly (scarring fingers and toes)
T: telangiectasis (blood vessel breakage)

Question 23

scleroderma variants: morphea

Answer 23

scleroderma-like changes

Question 24

scleroderma variants: linear localized

Answer 24

line of scarring

Question 25

scleroderma variants: eosinophilic fasciitis?

Answer 25

tight skin + lots of eosinophils

Question 26

polymyositis what is it? what will be seen?

Answer 26

progressive, symmetric proximal m. weakness, skeletal m. injury only; CD8 cells in endomysium

Question 27

dermatomyosistis: is? seen?

Answer 27

progressive, symetric proximal m. weakness AND malar rash; skeletal m. AND skin; CD4 cells in perimysium

Question 28

what features are associated with dermatomyositis?

Answer 28

``` Gottron papules (reddish, scaly bumps on knuckles) Heliotropic rash (under/around eyes) ```

Question 29

what is mixed connective tissue disease?

Answer 29

a big clusterfuck: lupus, polymositis, scleroderma, RA features togther

Question 30

what test is positive for MCTD?

Answer 30

Anti-U RNP-ANA

Question 31

onset of dermatomyositis in adulthood can warn of?

Answer 31

occult CA