Connective Tissue Ds

Question 1

Clonal deletion of self-reactive T and B lymphocyte during their maturation in lymphoid organs, these undergo apoptosis

Answer 1

central tolerance

Question 2

peripheral tolerance

Answer 2

how we destroy or control any self-react T-cells that escape central clonal deletion

Question 3

Three major systems Peripheral Tolerance

Answer 3

clonal deletion, clonal anergy, peripheral suppresion by T cells

Question 4

what controls uncontrolled T-cell activation

Answer 4

Fas-Fas ligand system

Question 5

how does clonal anergy work

Answer 5

activation of CD4+ cells requires two signals, if second signal is not delivered- anergy occurs

Question 6

molecular mimicry hows that work

Answer 6

bacteria antigens that look like self antigen and cause you to have cross reactivity

Question 7

An autoantibody against the Fc portion of autologous IgG

Answer 7

Rheumatoid factor

Question 8

What is the actual measured Immunoglobin in Rheumatoid factor

Answer 8

IgM

Question 9

Anti-Nuclear Antibodies are what titer in normal versus in connective tissue diease

Answer 9

in normal is low titer, high titer in connective tissue diease

Question 10

What HLA is assoicated with SLE

Answer 10

HLA-DQ

Question 11

What kills people whom have SLE

Answer 11

renal diease

Question 12

What is diagnostic for SLE

Answer 12

antibodies to double stranded DNA and the SM antigen

Question 13

In Sle high titlers of DS DNA are usually associated with

Answer 13

active renal diease

Question 14

In SLE if anti-SS-B is present

Answer 14

low risk for of nephritis

Question 15

Liquefactive degeneration is seen in

Answer 15

the skin in SLE

Question 16

most common class of lupus

Answer 16

class IV diffuce lupus

Question 17

Focal lupus nephritis

Answer 17

focal lessions, active lession are characterizes by swelling, proteinuria and hematuria common

Question 18

what percent of glomeruli are involved in diffuse lupus nephritis

Answer 18

more than 50% tq

Question 19

what cells are proliferated in diffuse lupus nephritis

Answer 19

endothelial, mesengial, and epithelial cells with crescents

Question 20

tq most serious form of renal lesions in SLE

Answer 20

DIffuse lupus nephritis

Question 21

Most common lesion encountered in renal biopsy

Answer 21

diffuse lupus nephritis

Question 22

wire loop lesion tq

Answer 22

when extensive subendothelial deposits create a thickening of capillary wall

Question 23

when is wire loop lesion seen

Answer 23

lupus in advanced sclerosing lupus nephritis

Question 24

wire loop lesion indicates what about diease

Answer 24

active and poor prognosis

Question 25

CNS symptoms of SLE caused by

Answer 25

acute vasculitis

Question 26

libman-sacks endocarditis

Answer 26

nonbacterial verrucous endocarditis, caused by sle

Question 27

Are ana's specfic or senstive for lupus

Answer 27

senstive as 100% of those with it will have it however shows up in other ds so not specfic at all

Question 28

of the 11 diagnostic criteria required for SLE how many must be present for lupus to be diagnoised

Answer 28

4/11

Question 29

#1 COD for SLE

Answer 29

renal failure

Question 30

tq Chronic discoid lupus

Answer 30

only affects skin, if any systemic involvement mild

Question 31

BQ drugs that cause Drug-induced lupus

Answer 31

hydralize, procainamide, isoniazide, penicillamine, sulfa, phytonin (SHIPPP)

Question 32

Sjorgren syndrome pnemonic

Answer 32

can't see, can't spit, can't climb up shit

Question 33

describe sjorgren syndrome

Answer 33

you are very dry and you have autoimmune destruction of lacrimal and salivary flands

Question 34

if a ds is autoimmune who is gonna have it more likely

Answer 34

Woman

Question 35

What antiboides are implicated in sjogren syndrome against ribonucleoproteins

Answer 35

SS-A (RO) SS-B (La) ......RoLa

Question 36

tq what are those with sjorgren syndrome at high risk for

Answer 36

non-hodgkins lymphoma

Question 37

what is most commonly effect in scleroderma

Answer 37

skin

Question 38

Crest is associated with

Answer 38

localized scleroderma

Question 39

widespread skin involvement with rapid progression and early visceral involvement seen in

Answer 39

diffuse scleroderma

Question 40

what do you see in early scleroderma

Answer 40

edema

Question 41

what do you see in late scleroderma

Answer 41

capillaries with thickened walls

Question 42

where is scleroderma most severe

Answer 42

in the esophagus, food can get stuck here

Question 43

why would the kidneys be of any concern in scleroderma

Answer 43

as hpertension and malgnant hypertension can result

Question 44

if the lungs are involved in scleroderma what may occur

Answer 44

pulmonary hypertension and interstitial fibrosis

Question 45

those with crest will have what assoicated with it tq

Answer 45

anticentromere antibody

Question 46

what two lab vaules will you see in scleroderma

Answer 46

anticentromere antibodies and antibodies to scl-70

Question 47

Pro's and con's of anticentromere antibodies in scleroderma

Answer 47

pro's - lower freq of pulmonary fibrosis and mortality Con's - higher risk of pulmonary HTN

Question 48

If you see antibodies to SCL-70 elevated in someone with scleroderma this means they will have

Answer 48

high mortality, increase risk of pulmonary fibrosis, and rapid cutanous involvement

Question 49

what does CREST stand for

Answer 49

calcinosis, raynauds, esophageal dysphagia, sclerodactyly, telangectasia

Question 50

Lilac or heliotrope discoloration of upper eyelif with periorbital edema key for TQ

Answer 50

dermatomyosits

Question 51

Who is affected by Dermatomyositis adults or children

Answer 51

both

Question 52

Dermatomyositis what is affected first? later?

Answer 52

first proximal muscles, late fine movements

Question 53

adults with dermatomyositis have an increased risk of developing?

Answer 53

visceral cancers

Question 54

anti-mi-2

Answer 54

seen in dermatomyositis and polymyositis

Question 55

CD4 or CD8 cells are associated with dermatomyositis

Answer 55

CD4 T cells

Question 56

CD4 or CD8 T cells are associated wtih polymyositis

Answer 56

CD8 T cells

Question 57

Anti-Jo-1 seen in

Answer 57

polymyositus

Question 58

inclusion-body myositis begins with

Answer 58

DISTAL muscles