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Pathology exam two > Connective Tissue Ds > Flashcards

Connective Tissue Ds Flashcards

1
Q

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

A

central tolerance

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2
Q

peripheral tolerance

A

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

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3
Q

Three major systems Peripheral Tolerance

A

clonal deletion, clonal anergy, peripheral suppresion by T cells

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4
Q

what controls uncontrolled T-cell activation

A

Fas-Fas ligand system

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5
Q

how does clonal anergy work

A

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

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6
Q

molecular mimicry hows that work

A

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

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7
Q

An autoantibody against the Fc portion of autologous IgG

A

Rheumatoid factor

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8
Q

What is the actual measured Immunoglobin in Rheumatoid factor

A

IgM

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9
Q

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

A

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

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10
Q

What HLA is assoicated with SLE

A

HLA-DQ

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11
Q

What kills people whom have SLE

A

renal diease

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12
Q

What is diagnostic for SLE

A

antibodies to double stranded DNA and the SM antigen

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13
Q

In Sle high titlers of DS DNA are usually associated with

A

active renal diease

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14
Q

In SLE if anti-SS-B is present

A

low risk for of nephritis

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15
Q

Liquefactive degeneration is seen in

A

the skin in SLE

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16
Q

most common class of lupus

A

class IV diffuce lupus

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17
Q

Focal lupus nephritis

A

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

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18
Q

what percent of glomeruli are involved in diffuse lupus nephritis

A

more than 50% tq

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19
Q

what cells are proliferated in diffuse lupus nephritis

A

endothelial, mesengial, and epithelial cells with crescents

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20
Q

tq most serious form of renal lesions in SLE

A

DIffuse lupus nephritis

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21
Q

Most common lesion encountered in renal biopsy

A

diffuse lupus nephritis

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22
Q

wire loop lesion tq

A

when extensive subendothelial deposits create a thickening of capillary wall

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23
Q

when is wire loop lesion seen

A

lupus in advanced sclerosing lupus nephritis

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24
Q

wire loop lesion indicates what about diease

A

active and poor prognosis

25
Q

CNS symptoms of SLE caused by

A

acute vasculitis

26
Q

libman-sacks endocarditis

A

nonbacterial verrucous endocarditis, caused by sle

27
Q

Are ana’s specfic or senstive for lupus

A

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

28
Q

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

A

4/11

29
Q

1 COD for SLE

A

renal failure

30
Q

tq Chronic discoid lupus

A

only affects skin, if any systemic involvement mild

31
Q

BQ drugs that cause Drug-induced lupus

A

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

32
Q

Sjorgren syndrome pnemonic

A

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

33
Q

describe sjorgren syndrome

A

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

34
Q

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

A

Woman

35
Q

What antiboides are implicated in sjogren syndrome against ribonucleoproteins

A

SS-A (RO) SS-B (La) ……RoLa

36
Q

tq what are those with sjorgren syndrome at high risk for

A

non-hodgkins lymphoma

37
Q

what is most commonly effect in scleroderma

A

skin

38
Q

Crest is associated with

A

localized scleroderma

39
Q

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

A

diffuse scleroderma

40
Q

what do you see in early scleroderma

A

edema

41
Q

what do you see in late scleroderma

A

capillaries with thickened walls

42
Q

where is scleroderma most severe

A

in the esophagus, food can get stuck here

43
Q

why would the kidneys be of any concern in scleroderma

A

as hpertension and malgnant hypertension can result

44
Q

if the lungs are involved in scleroderma what may occur

A

pulmonary hypertension and interstitial fibrosis

45
Q

those with crest will have what assoicated with it tq

A

anticentromere antibody

46
Q

what two lab vaules will you see in scleroderma

A

anticentromere antibodies and antibodies to scl-70

47
Q

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

A

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

48
Q

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

A

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

49
Q

what does CREST stand for

A

calcinosis, raynauds, esophageal dysphagia, sclerodactyly, telangectasia

50
Q

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

A

dermatomyosits

51
Q

Who is affected by Dermatomyositis adults or children

A

both

52
Q

Dermatomyositis what is affected first? later?

A

first proximal muscles, late fine movements

53
Q

adults with dermatomyositis have an increased risk of developing?

A

visceral cancers

54
Q

anti-mi-2

A

seen in dermatomyositis and polymyositis

55
Q

CD4 or CD8 cells are associated with dermatomyositis

A

CD4 T cells

56
Q

CD4 or CD8 T cells are associated wtih polymyositis

A

CD8 T cells

57
Q

Anti-Jo-1 seen in

A

polymyositus

58
Q

inclusion-body myositis begins with

A

DISTAL muscles

Pathology exam two (4 decks)

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