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MSK/Rheum > Sclerosis/Lupus > Flashcards

Sclerosis/Lupus Flashcards

(74 cards)

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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
SSc upper GI disease
GERD, esophageal dysmotility, aperistaltic esophagus, esophageal stricture
26
SSc lower GI manifestations
decreased peristalsis- pseudo-obstruction bacterial overgrowth, malabsorption megacolon wide-mouth diverticuli
27
SSc musculoskeletal manifestations
``` arthralgia tendon friction rubs inflammatory arthritis myopathy calcinosis acrolysis ```
28
anti-centromere autoantibody
limited cutaneous disease digital ischemia audiovestibular disease female predominance
29
anti-Scl-70
diffuse disease- interstitial lung disease
30
anti-RNA pol III
diffuse skin disease, renal crisis
31
T cells (CD4+) produce
IL4, IL6, IL13
32
B cells (CD20+) secrete
IL6, TGF_beta
33
HLA function
presentation of antigen
34
ITGAM function
adhesion of WBC to endothelium tolerance
35
IRF5 function
production of IFN alpha
36
BLK function
activation of B cells
37
abnormalities of T cells in lupus
``` Hyperactivation of helper cells Recognition of self antigens Autoreactive clones escape tolerance T cell repertoire skewed toward help Defective T cell regulatory circuits ```
38
abnormalities of B cells in lupus
Hyperactivation | Recognition of self antigens (RNA/protein particles
39
what happens to rates of apoptosis in SLE lymphocytes
increased
40
what happens to rate of phagocytosis in SLE
decreased
41
what must blebs be coated with for clearance
C1q
42
Belimumab
neutralizing human monoclonal antibody against BLyS
43
what does belimumab do
decreases B cell development, proliferation, Ig production
44
definite diagnosis of SLE requires how many features
4 of 11
45
specific autoantibodies for lupus
anti-dsDNA anti-Sm antiphospholipid
46
comorbidities of SLE
premature atherosclerosis
47
malar rash
Fixed erythema, flat or raised,sparing the nasolabial folds
48
discoid rash
Raised patches, adherent keratotic scaling, folicular plugging; older lesions may cause scarring
49
photosensitivity
Skin rash from sunlight by patient history or physician observation
50
mouth involvement in SLE
oral or naso-pharyngeal ulcers in 50-75% usually painless
51
arthritis description in SLE
non-erosive, non-deforming morning stiffness knees, small joints of hands
52
cardiac criteria for SLE
pericarditis most prominent
53
pulmonary criteria for SLE
pleural effusion
54
class I LN
minimal mesangial
55
class II LN
mesangial proliferative
56
class III LN
focal
57
class IV LN
diffuse
58
class V LN
membranous
59
class VI LN
advanced sclerotic
60
diffuse proliferative LN
Extensive immune complex deposition in mesangium and subendothelial side of capillary basement membranes
61
Immune complex deposition confined to intramembranous and subepithelial area of capillary basement membrane
membranous lupus nephritis
62
neuro presentations of SLE
organic mental syndrome | seizures
63
what type of anemia in SLE
hemolytic most frequent
64
pathogenic autoantibodies in SLE
IgG Monospecific High affinity Somatically mutated
65
anti-dsDNA
diagnostic, may correlate with disease | renal
66
anti-Sm
CNS involvement
67
anti-nRNP
joints > renal
68
anti-SSA/Ro, SSB/La
neonatal lupus, SS, SCLE
69
antiphospholipids
thrombosis
70
neonatal lupus 2 forms
congenital heart block, neonatal rash
71
complement activation results in production of what
chemotactic factor | anaphylotoxins
72
complement activation results in
glomerulonephritis, fetal loss
73
moderate disease: add what medication (SLE)
belimumab
74
severe disease: add what medication (SLE)
mycophenalate mofetil (MMF)

MSK/Rheum (5 decks)

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