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MOD > The Systemic Autoimmune Diseases > Flashcards

The Systemic Autoimmune Diseases Flashcards

1
Q

Central Tolerance

A

testing B/T cells for self affinity

  • autoreactive T-cells: killed
  • autoreactive B-cells: modified
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2
Q

Function of AIRE protein

A

key protein in deleting autoreactive T-cells before birth

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3
Q

Peripheral Tolerance

A

rendering of autoreactive lymphocytes after birth

  • self reactive lymphocytes rendered by CTLA-4/PD-1
  • regulatory t-cells (CD25, IL-2, FOXP3) suppress self-immunity
  • autoreactive T-cells undergo apoptosis when they activate
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4
Q

Most-often-mutated gene in autoimmunity

A

PTPN22

others – NOD2, IL-2, IL-7

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5
Q

Molecular mimicry

A

antigens on microbes mimic self-Ag

- immune response can cross-react to normal tissue

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6
Q

Microchimerism

A

increased number cells from mother in those with AI diseases

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7
Q

ANA: rim pattern

A

Systemic lupus

- anti ds-DNA

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8
Q

ANA: homogenous pattern

A

Drug-lupus

- anti-ssDNA, anti-histone

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9
Q

ANA: speckled pattern

A

think anything….

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10
Q

ANA: centromere pattern

A

CREST

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11
Q

ANA: nucleolar pattern

A

scleroderma

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12
Q

Anti-dsDNA

A

systemic lupus

- rim pattern on ANA

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13
Q

Anti-ssDNA

A

drug-induced lupus

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14
Q

Anti-histone

A

drug-induced lupus

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15
Q

Anti-Sm

A

systemic lupus (1/3 of patients with SLE)

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16
Q

Anti-Ro/SSA

A

Sjogren’s patients, neonatal lupus (heart block)

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17
Q

Anti-LA/SSB

A

Sjogren’s patients

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18
Q

Anti-U1RNP

A

mixed CT disease

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19
Q

Anti-Scl70

A

scleroderma

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20
Q

Anti-RNA polymerase III

A

scleroderma

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21
Q

Anti-centromere

A

CREST (mild scleroderma)

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22
Q

Anti-Jo

A

polymyositis/dermatomyositis

- severe disease, lung damage, arthritis

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23
Q

Anti-Mi2

A

dermatomyositis

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24
Q

Anti-SRP

A

severe polymyositis variant w/ severe necrotizing myopathy

25
Q

Anti-P155/TIF1g and Anti-P140/MJ

A

juvenile and paraneoplasic dermatomyositis

26
Q

Cardinal sign of SLE

A

butterfly rash

27
Q

Systemic Lupus Erythematosus

A

antinuclear antibodies

  • UV light triggers flareups
  • females/kleinfelters more common
  • Tissue injury from Type III, cell destruction from antibodies (Type II)
28
Q

Models for SLE

A
  • Drug reactions (hydralaine/procainamide/anti-aTNF, isoniazid)
  • Genetic deficiences (C1q is used for removing apoptotic bodies)
29
Q

How many criteria must a patient present with to be deemed SLE?

A

4/11 (now 4/17)

30
Q

Hematoxylin bodies

A

stripped nuclei acted-on by ANA’s in lupus

- homogenized nuclei

31
Q

LE cell

A

stripped nucleus opsonized by ANA

- devoured by phagocytes

32
Q

What mediates lupus vasculitis?

A

immune-complex mediated

33
Q

What is the appearance of the spleen in lupus?

A

adventitial onionskinning

34
Q

What is an immune complex deposit in the glomerulus called?

A

wire loop

35
Q

Lupus band test

A

granular Ig and complement at dermal-epidermal junction

36
Q

What causes mouth ulcers in lupus?

A

infarcts from vasculitis

37
Q

What are Libman-Sacks endocarditis?

A

In lupus, sterile thrombi on the lines of closure of valves

- can cause infarcts

38
Q

Neonatal Lupus

A

develop rash, heart block (AV node) by mom’s auto-Ab (anti-Ro)

39
Q

Blood in SLE

A

hypercoaguable
-polyclonal gammopathy (high Ig), lysis of RBC, destruction of neutrophils/platelets, low lymphocyte count, lupus anticoagulants, DVT (w/out lupus anticoagulants), thrombotic thyrombocytopenic purpura

40
Q

Subacute cutaneous lupus

A

Anti-Ro is seen

- have rash with lupus (or sjorgren’s or RA)

41
Q

Discoid lupus

A

rash with patches of scales and lost hairs

  • hyperkeratosis as plugs (with hydropic changes)
  • lymphocytes in patches
42
Q

Drug-induced lupus

A

hydralazine, procainamide, isoniazid

- anti-histone Ab, and anti-dsDNA Ab (patients receiving anti-aTNF tx)

43
Q

What glands does Sjogren’s effect primarily?

A

Salivary, lacrimal, pancreatic glands

- fill with lymphocytes

44
Q

Symptoms of Sjorgren’s

A

autoimmunity against glands

  • dry eyes and teeth decay (no healthy saliva)
  • Mikulicz’s syndrome (enlarged glands)
  • test for using schirmer test
  • Potentially malignant lymphoma
45
Q

Scleroderma

A

“systemic sclerosis”

  • inflammation –> dense fibrosis (seen in hands that cannot flex/extend)
  • abundant CD4+ T-cells, calcium deposits
  • begins with Raynaud’s
  • causes telangiectasias (vessels become dilated tufts)
  • fibrosis of esophagus, small bowel, “onionskinning” of small renal artery
46
Q

Raynaud’s

A

fingers begin to hurt and change color (red, blue, white)

- seen in cold

47
Q

Common cause of death in Scleroderma

A

pulmonary fibrosis/pulmonary vascular narrowing

- pulmonary HTN

48
Q

Why does scleroderma of small renal artery present a problem?

A

Kidney thinks pt is in shock –> releases renin –> increase blood pressure –> stroke/heart problems

49
Q

Limited Scleroderma

A

not as rapid of onset/generalized distribution as systemic sclerosis
- CREST syndrome

50
Q

CREST syndrome

A 
- Limited scleroderma
Calcinosis (deposits on skin)
Raynaud's
Esophageal fibrosis
Sclerodactyly
Telangiectasias
51
Q

Morphea

A

Scleroderma-like changes in a patch

- CD4 rich –> fibrosis

52
Q

Parry-Romberg

A

scar contracts over 1/2 of face and underlying skull is deformed

53
Q

Eosinophilic fasciitis

A

tight skin with many eosinophils in subcutaneous and fascia

54
Q

Polymyositis

A

CD8 lymphocytes into muscle (in the endomysium)

  • sk. rather than sm. m
  • high CK and aldolase
55
Q

Dermatomyositis

A

CD4 T-cells, B-cells, plasma cells (within perimysium/around the blood vessels)

  • MAC often seen in vessel wall
  • muscle fibers at edges of fascicles are most likely atrophied
  • Heliotrope rash (eyelashes turn purple)
  • Gottron’s rash (reddish-purple) on knuckles (characteristic rash)
56
Q

What is mechanic’s hands a sign of ?

A

Polymyositis/dermatomyositis/anti-synthetase (Jo1) syndrome

- dark keratin

57
Q

Mixed Connective Tissue Disease (MCTD)

A

overlapping syndromes (lupus, scleroderma, RA, polymyositis)

  • Anti-U1-RNP is high-titer Ab
  • starts as synovitis of fingers plus Raynaud’s
  • death is usually due to pulmonary vascular sclerosis/fibrosis (rash–> calcification)
58
Q

IgG4-related disease

A

fibroinflammatory diseases
(Riedel’s fibrosing thyroiditis, AI pancreatitis, retroperitoneal fibrosis/periaortitis, fibrosis of salivary/lacrimal glands)
-features: fibrosis w/ cartwheel pattern, IgG4 lesions (high levels)

MOD (7 decks)

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