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MSK/Rheum > Sclerosis/Lupus > Flashcards

Sclerosis/Lupus Flashcards

1
Q

white fingers caused by

A

blood flow cut off

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2
Q

blue fingers caused by

A

low oxygen

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3
Q

red fingers caused by

A

blood return

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4
Q

how much of Raynaud’s is primary

A

60%

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5
Q

indications of primary Raynauds

A

normal nail fold capillaries

spares the palms

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6
Q

how much of Raynaud’s is secondary

A

40%

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7
Q

scleroderma diagnosis

A

thickened skin

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8
Q

oral manifestations of systemic sclerosis

A

perioral skin tightening- decreased oral aperture
periodontal disease
telangiectasias on lips and tongue

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9
Q

environmental factors contributing to SS

A 
silica exposure
organic solvents
industrial fumes
CMV, parvo
history of childbearing
cancer
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10
Q

morpheme may indicate

A

abnormalities of underlying mesenchymally-derived tissues, including the skull

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11
Q

CREST- C

A

calcinosis- calcium deposits in skin

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12
Q

CREST- R

A

Raynaud’s phenomenon

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13
Q

CREST-E

A

esophageal dysfunction- acid reflex and decrease in motility of esophagus

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14
Q

CREST-S

A

sclerodactyly- thickening and tightening of skin on fingers and hands

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15
Q

CREST-T

A

telangiectasias-dilation of capillaries causing red marks on surface of skin

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16
Q

how long between Raynaud’s and onset of limited sclerosis

A

10 years

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17
Q

tendon friction rubs are indicative of

A

large joint involvement

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18
Q

serum markers in DSSc

A

anti-Scl70 (anti-topoisomerase

anti-RNA polumerase

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19
Q

how long between Raynaud’s and onset of diffuse sclerosis

A

one year

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20
Q

extensive skin sclerosis

A

proximal to the wrists, often involving trunk but sparing the back

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21
Q

factors favoring ischemia in secondary Raynaud’s

A 
vasoconstriction, endothelial damage
decreased NO, prostacyclin
Decrease in activated VEGF
active alpha-2 receptors
endothelin-1 increase
decreased fibronolysis
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22
Q

treatment of scleroderma renal disease

A

ACE inhibitors

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23
Q

SSc lung disease

A

pulmonary hypertension

interstitial lung disease

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24
Q

SSc cardiac disease

A

conduction system

cardiomyopathy, pericarditis…

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25
Q

SSc upper GI disease

A

GERD, esophageal dysmotility, aperistaltic esophagus, esophageal stricture

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26
Q

SSc lower GI manifestations

A

decreased peristalsis- pseudo-obstruction
bacterial overgrowth, malabsorption
megacolon
wide-mouth diverticuli

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27
Q

SSc musculoskeletal manifestations

A 
arthralgia
tendon friction rubs
inflammatory arthritis
myopathy
calcinosis
acrolysis
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28
Q

anti-centromere autoantibody

A

limited cutaneous disease
digital ischemia
audiovestibular disease
female predominance

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29
Q

anti-Scl-70

A

diffuse disease- interstitial lung disease

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30
Q

anti-RNA pol III

A

diffuse skin disease, renal crisis

31
Q

T cells (CD4+) produce

A

IL4, IL6, IL13

32
Q

B cells (CD20+) secrete

A

IL6, TGF_beta

33
Q

HLA function

A

presentation of antigen

34
Q

ITGAM function

A

adhesion of WBC to endothelium tolerance

35
Q

IRF5 function

A

production of IFN alpha

36
Q

BLK function

A

activation of B cells

37
Q

abnormalities of T cells in lupus

A 
Hyperactivation of helper cells
Recognition of self antigens
Autoreactive clones escape tolerance
T cell repertoire skewed toward help
Defective T cell regulatory circuits
38
Q

abnormalities of B cells in lupus

A

Hyperactivation

Recognition of self antigens (RNA/protein particles

39
Q

what happens to rates of apoptosis in SLE lymphocytes

A

increased

40
Q

what happens to rate of phagocytosis in SLE

A

decreased

41
Q

what must blebs be coated with for clearance

A

C1q

42
Q

Belimumab

A

neutralizing human monoclonal antibody against BLyS

43
Q

what does belimumab do

A

decreases B cell development, proliferation, Ig production

44
Q

definite diagnosis of SLE requires how many features

A

4 of 11

45
Q

specific autoantibodies for lupus

A

anti-dsDNA
anti-Sm
antiphospholipid

46
Q

comorbidities of SLE

A

premature atherosclerosis

47
Q

malar rash

A

Fixed erythema, flat or raised,sparing the nasolabial folds

48
Q

discoid rash

A

Raised patches, adherent keratotic scaling, folicular plugging; older lesions may cause scarring

49
Q

photosensitivity

A

Skin rash from sunlight by patient history or physician observation

50
Q

mouth involvement in SLE

A

oral or naso-pharyngeal ulcers in 50-75%

usually painless

51
Q

arthritis description in SLE

A

non-erosive, non-deforming
morning stiffness
knees, small joints of hands

52
Q

cardiac criteria for SLE

A

pericarditis most prominent

53
Q

pulmonary criteria for SLE

A

pleural effusion

54
Q

class I LN

A

minimal mesangial

55
Q

class II LN

A

mesangial proliferative

56
Q

class III LN

A

focal

57
Q

class IV LN

A

diffuse

58
Q

class V LN

A

membranous

59
Q

class VI LN

A

advanced sclerotic

60
Q

diffuse proliferative LN

A

Extensive immune complex deposition in mesangium and subendothelial side of capillary basement membranes

61
Q

Immune complex deposition confined to intramembranous and subepithelial area of capillary basement membrane

A

membranous lupus nephritis

62
Q

neuro presentations of SLE

A

organic mental syndrome

seizures

63
Q

what type of anemia in SLE

A

hemolytic most frequent

64
Q

pathogenic autoantibodies in SLE

A

IgG
Monospecific
High affinity
Somatically mutated

65
Q

anti-dsDNA

A

diagnostic, may correlate with disease

renal

66
Q

anti-Sm

A

CNS involvement

67
Q

anti-nRNP

A

joints > renal

68
Q

anti-SSA/Ro, SSB/La

A

neonatal lupus, SS, SCLE

69
Q

antiphospholipids

A

thrombosis

70
Q

neonatal lupus 2 forms

A

congenital heart block, neonatal rash

71
Q

complement activation results in production of what

A

chemotactic factor

anaphylotoxins

72
Q

complement activation results in

A

glomerulonephritis, fetal loss

73
Q

moderate disease: add what medication (SLE)

A

belimumab

74
Q

severe disease: add what medication (SLE)

A

mycophenalate mofetil (MMF)

MSK/Rheum (5 decks)

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