Icm Rheum 07: Scleroderma

Front 1

2 kinds of localized scleroderma

Back 1

morphea
linear scleroderma

Front 2

in localized scleroderma there are no ___ (2)

Back 2

systemic symptoms
auto Abs

Front 3

4 kinds of systemic sclerosis

Back 3

limited cutaneous SSc
diffuse cutaneous SSc
sine scleroderma
overlap syndrome

Front 4

limited cutaneous SSc affects ___ (2), but diffuse cutaneous SSc affects those and ___ (2)

Back 4

distal extremities
face
proximal extremities
trunk

Front 5

overlap syndrome combines SSc with one of ____ (6)

Back 5

RA
polymyositis
SLE
Sjögren
vasculitis
sarcoidosis

Front 6

limited cutaneous SSc is called CREST syndrome which stands for

Back 6

calcinosis
Raynaud's phenomenon
esophageal dysmotility
sclerodactylyl
telangiectasia

Front 7

male:female ratio for SSc

Back 7

1:8

Front 8

age range for SSc

Back 8

35-55

Front 9

the most important auto Ab for SSc

Back 9

anti SCL-70

Front 10

SCL-70 indicates ___ disease with high likelihood of ___

Back 10

diffuse
interstitial lung disease

Front 11

____ is an Ab associated with LC SSc

Back 11

anti-centromere

Front 12

vascular damage in SSc is caused by excess ___, impaired ___ (2) synthesis, and activation of ___ (2)

Back 12

ET-1 excess
PGI2
NO
vWF
adhesion molecules

Front 13

T/F: Raynaud's phenomenon is present in all forms of SSc

Back 13

true

Front 14

T/F: Raynaud's phenomenon is sufficient for SSc dx

Back 14

false

Front 15

___ are pathognomonic for SSc

Back 15

pitting scars

Front 16

3 kinds of vascular injuries associated with SSc

Back 16

pulmonary HTN (PAH)
scleroderma renal crisis
gastric antrum vascular ectasia (GAVE)

Front 17

patients with GAVE are prone to ___

Back 17

GI bleeds

Front 18

in SSc there is overproduction and accumulation of ___ in ___ (2)

Back 18

collagen
small vessels
tissue

Front 19

collagen deposition is driven by ___, especially ___. fibroblasts respond by producing ___

Back 19

inflammatory cytokines
IL-6
collagen 1, 3, 7

Front 20

___ score grades how stiff a patch of skin is

Back 20

Rodnan

Front 21

___ is the main cause of death in SSc and is a diagnostic criterion

Back 21

pulmonary fibrosis

Front 22

pulmonary fibrosis can cause ___, which can cause ___ (2 cardiac effects)

Back 22

PAH
RHF
VF

Front 23

___ is needed to detect pulmonary fibrosis. in early stage it appears as ___, in late stage it appears as ___.

Back 23

high res CT
ground glass
honeycombing

Front 24

SSc with honeycombing appearance is reversible/irreversible

Back 24

irreversible

Front 25

in LC SSc, you can have PAH without ___. this is called ___.

Back 25

interstitial fibrosis
primary PAH

Front 26

in primary PAH SSc, HRCT is ___

Back 26

almost normal

Front 27

in both primary and secondary PAH, DLCO is ___. in primary PAH, FVC is ___, but in secondary it's ___.

Back 27

low
normal
low

Front 28

T/F: reduced DLCO in primary constitutes a V/Q mismatch

Back 28

true

Front 29

___ anemia is associated with SSc renal disease

Back 29

microangiopathic hemolytic

Front 30

renal disease is primarily associated with ___ SSc with ___ Abs

Back 30

DC
anti SD70

Front 31

GIT is involved in ___% of SSc

Back 31

50-80

Front 32

when liver is involved in SSc it's in the form of ___

Back 32

primary biliary cirrhosis

Front 33

___ is a skeletal criterion for SSc. ___ (2) may be present with it.

Back 33

sclerodactyly
acro-osteolysis (fracture of distal phalanx)
clubbing

Front 34

___ is very common in SSc but is not a criterion. ____ is often superimposed on it.

Back 34

calcinosis
infection

Front 35

the only major SSc criterion

Back 35

DC SSc

Front 36

3 minor SSc criteria

Back 36

pitting scars
sclerodactyly
pulmonary fibrosis

Front 37

to diagnose SSc, you need ___ major criterion or ___ minor criteria

Back 37

1
2

Front 38

2 Abs characteristic of mixed CT disease

Back 38

ANA
anti RNP

Front 39

Tx for raynaud's phenomenon (3)

Back 39

warmth (wax)
prostacyclin
bosentan (ET1 blocker)

Front 40

Tx for calcinosis (2)

Back 40

debridement
antibiotics

Front 41

Tx for GI

Back 41

high dose PPIs

Front 42

steroids and MTX are used in SSc to treat ___ (3)

Back 42

skin
joints
myositis

Front 43

MTX is an example of a ___

Back 43

disease modifying anti rheumatic drug (DMARD)

Front 44

PAH in SSc is treated with

Back 44

bosentan
synthetic PGI2
slidenafil
oxygen

Front 45

10 year survival for DC SSc

Back 45

55%

Front 46

muscle ___ but not muscle ___ is present in polymyositis

Back 46

weakness
pain

Front 47

T/F: fever may be present in polylmyositis

Back 47

true

Front 48

___% of polymyositis/dermatomyositis pts have malignancy

Back 48

20

Front 49

most important diagnostic for DM/PM is ___, specifically ___.

Back 49

muscle enzymes
CK

Front 50

5 muscle enzymes measured in DM/PM

Back 50

AST
ALT
CK
LDH
aldolase

Front 51

main treatment for DM/PM

Back 51

high dose steroids
DMARDS
IVIG

Front 52

DMARDs used for DM/PM

Back 52

MTX
azathioprine

Front 53

IVIG counterindication

Back 53

IgA deficiency