Icm: Scleroderma

Front 1

What does scleroderma mean?

Back 1

skleros- hard
derma- skin

Front 2

What vascular abnormalities scleroderma cause?
-large or small blood vessels

Back 2

functional and structural abnormalities of SMALL blood vessels

Front 3

What kind of dz is scerloderma?

Back 3

autoimmunity
-causes small blood vessels changes, fibrosis of skin and internal organs

Front 4

Peak onset?
Male or f/m?

Back 4

35-65 years of age
Similar to Lupus in that more Females than males
-w/ childbirth, fm incidence even greater

Front 5

Is there a genetic component to scleroderma?

Back 5

no
-may be environmental relation
ie: silica dusts, organic solvents

Front 6

Is scleroderma associated with inflammation?

Back 6

NO way !!! remember this, will see scarring, but NO inflammation

Front 7

Histologically, what do you see with blood vessels?

Back 7

scarred blood vessels but no inflammation

Front 8

What kind of vessels does systemic sclerosis affect?

Back 8

widespread small-vessel narrowing and fibrosis

small arteries, arterioles, capillaries

Front 9

What is Raynaud's phenomena

Back 9

-White->Blue->red
*hypersensitivity of alpha-2-alpha adrencergic receptors on vascular smooth-muscle cells to vasoconstricting stimuli

Front 10

What causes luminal narrowing in small blood vessels?

Back 10

smooth muscle cells in intima of small vessels

Front 11

Lack of replacement of damaged blood vessels (neoangiogenesis) believed to be caused by

Back 11

ENDOSTATIN, an angiogenesis inhibitor

Front 12

What changes might observe in systemic sclerosis?

Back 12

Loop capillary beds

Front 13

What causes fibrosis of skin and internal organs in systemic sclerosis?

Back 13

-increased production of collagen, fibronectin, and glycosaminoglycans
**Excessive production of ECM causes THICKENING of skin and FIBROSIS of internal organs

Front 14

blood vessel changes scleroderma occur most commonly w/?

Back 14

Small blood vessels (small arteries, arterioles, capillaries)

Front 15

blood vessel changes scleroderma are associated w/ what levels of ENDOSTATIN

Back 15

increased levels

Front 16

blood vessel changes scleroderma: which cells in the blood vessel proliferate and cause narrowing blood vessels?

Back 16

SMOOTH MUSCLE CELLS in intima of small vessels

Front 17

How is regular angiogenesis affected in scleroderma?

Back 17

Failure to replace damaged vessels (no angiogenesis)

Front 18

What are the 2 types of systemic scleroderma we talked in class?

Back 18

Diffuse cutaneous scleroderma (organs)

Limited Cutaneous Scleroderma (distal to elbow, knees, etc.)

Front 19

Crest is a subtype of which scleroderma?
-diffuse cutaneous?
-limited cutaneous?

Back 19

limited cutaneous scleroderma

Front 20

Skin changes limited to fingers, distal arms, and legs, face and neck?

Back 20

Limited cutaneous

Front 21

Proximal skin thickening involving face/neck/TRUNK/proximal arms and legs?
-diffuse cutaneous?
-limited cutaneous?

Back 21

diffuse cutaneous

Front 22

-diffuse cutaneous?
-limited cutaneous?
*Rapid onset dz

Back 22

Diffuse cutaneous

Front 23

-diffuse cutaneous?
-limited cutaneous?
*Progression of dz after long-standing Raynaud's

Back 23

Limited cutaneous

Front 24

-diffuse cutaneous?
-limited cutaneous?
**Late visceral dz w/ prominent PULMONARY HYPERTENSION and digital amputation from severe Raynaud's

Back 24

Limited cutaneous
*vs. diffuse cutaneous where visceral damage not just lungs

Front 25

-diffuse cutaneous?
-limited cutaneous?
Significant visceral DZ of lUNG, HEART, GI, and KIDNEY

Back 25

Diffuse Cutaneous

Front 26

-diffuse cutaneous?
-limited cutaneous?
CREST Syndrome is a subtype

Back 26

Limited cutaneous
-recall, crest subtype associated w/ anti-centromere ab

Front 27

-diffuse cutaneous?
-limited cutaneous?
*overall poor prognosis survival 40-60% at 10 years

Back 27

diffuse cutaneous
*has significant visceral dz
-LUNG, HEART, GI, KIDNEY

Front 28

-diffuse cutaneous?
-limited cutaneous?
*good prognosis >70% survival at 10 years

Back 28

Limited cutaneous

Front 29

-diffuse cutaneous?
-limited cutaneous?
*+ANA but usually negative anit-centromere

Back 29

Diffuse cutaneous
-recall, diffuse NOT involved w/ CREST usually

Front 30

-diffuse cutaneous?
-limited cutaneous?
*+anti-centromere ab

Back 30

Limited cutaneous
*recall, limited cutaneous and anti-centromere ab associated w/ CREST (centromere C, for Crest)

Front 31

What does CREST stand for?

Back 31

C = calcinosis
R = Raynauds
E = Esophageal involvement
S = Sclerodactyly (fingers)
T = Telangectasias (spidery red spots)
*frequently have anti-centromere ab

Front 32

Definition Raynaud's phenomena?

Back 32

Episodic color changes from Pallor - white
Cyanoisis - blue
Reactive erythmea - red
*enviro. response to cold and/or emotional stress
**numbness and pain associated w/ pallor and cyanosis

Front 33

Raynaud's is initial complaint in who?

Back 33

75% w/ SS
100% w/ Crest

Front 34

How can Raynaud's affect internal organs?

Back 34

transiet cold-induced perfusion decreases blood flow to the lungs of 1/2 of systemic sclerosis patients

Front 35

Where do scleroderma skin changes typically begin?

Back 35

fingers and hand - moves to face and neck

Front 36

What is an early change of SS?

Back 36

edematous change
-painless swelling of the fingers an hands

Front 37

Subcutaneous clacification (calcinosis) and telangectasias common in what types SSS?

Back 37

diffuse, limited, and CREST

Front 38

What happens to the joints in SSS?

Back 38

tendon friction rubs common
-inflammation and fibrinous involvement of tendon sheaths may mimic arthrits
-many have arthralgia and am stiffness

Front 39

In patients w/ scleroderma:
fibrotic changes limited to skin?

Back 39

false

Front 40

In patients w/ scleroderma:
-patients w/ diffuse do not have thickening of skin on calves?

Back 40

flase

Front 41

Which types of scleroderma are associated w/ calcinosis?

Back 41

all

Front 42

greater risk for progressive interstiital lung dz w/ inflammation and then diffuse fibrosis?

Back 42

Diffuse scleroderma

Front 43

What does scleroderma mean?

Back 43

skleros- hard
derma- skin

Front 44

Greater risk for pulmonary hypertension w/o fibrosis

Back 44

limited scleroderma

Front 45

What will see w/ pulmonary function test indicating fibrosis?

Back 45

-restriction w/ reduced vital capacity

Front 46

What will see w/ pulmonary function test indicating pulmonary hypertension?

Back 46

isolated decreased diffusion capacity of oxygen

Front 47

What vascular abnormalities scleroderma cause?
-large or small blood vessels

Back 47

functional and structural abnormalities of SMALL blood vessels

Front 48

What kind of dz is scerloderma?

Back 48

autoimmunity
-causes small blood vessels changes, fibrosis of skin and internal organs

Front 49

Peak onset?
Male or f/m?

Back 49

35-65 years of age
Similar to Lupus in that more Females than males
-w/ childbirth, fm incidence even greater

Front 50

Is there a genetic component to scleroderma?

Back 50

no
-may be environmental relation
ie: silica dusts, organic solvents

Front 51

Is scleroderma associated with inflammation?

Back 51

NO way !!! remember this, will see scarring, but NO inflammation

Front 52

Histologically, what do you see with blood vessels?

Back 52

scarred blood vessels but no inflammation

Front 53

What kind of vessels does systemic sclerosis affect?

Back 53

widespread small-vessel narrowing and fibrosis

small arteries, arterioles, capillaries

Front 54

What is Raynaud's phenomena

Back 54

-White->Blue->red
*hypersensitivity of alpha-2-alpha adrencergic receptors on vascular smooth-muscle cells to vasoconstricting stimuli

Front 55

What causes luminal narrowing in small blood vessels?

Back 55

smooth muscle cells in intima of small vessels

Front 56

In patients w/ scerloderma, which types are associated with calcinosis?

Back 56

diffuse, limited, and CREST

Front 57

What ab common to CREST subtype?

Back 57

anti-centromere

Front 58

What is normally noticeable w/ SS w/ regard to joint involvement?

Back 58

Inflammation and fibrinous involvement of tendon sheaths may mimic arthritis
-tendon friction rubs common

Front 59

What is the leading cause of mortality and morbidity in later stages of SS?

Back 59

Pulmonary involvement
*vascular obliteration, inflammation and then fibrosis of lung tissue can be present
-exertional dyspnea, exercise intolerance, cough

Front 60

Diffuse or limited scleroderma?
-greater risk for progressive interstitial lung dz w/ inflammation and then diffuse fibrosis

Back 60

Diffuse

Front 61

Diffuse or limited scleroderma?
-greater risk for pulmonary HTN w/o fibrosis

Back 61

Limited

Front 62

How dx pulmonary function w/ regard to fibrosis (what observe)?

Back 62

restriciton w/ reduced vital capacity

Front 63

How dx pulmonary function w/ regard to pulmonary hypertension (what observe)?

Back 63

isolated decreased diffusion capacity of oxygen

Front 64

If pulmonary function tests are normal, what is the gold standard for iding pressures?

Back 64

Right Heart Catheterization (echocardiogram can be insensitive)

Front 65

Pulmonary hypertension is a disease of the?

Back 65

small arteries and arterioles of the pulmonary circulation

Front 66

What does pulmonry hypertension look like as the dz progresses?

Back 66

-marked endothelium proliferation occurs in the intimal layer of the pulmonary artery and the muscular layer hypertophies
-small arterioles develop a muscle layer as the dz progresses. In situ thrombosis and formation of plexiform lesions are also characteristic

Front 67

what is associated w/ no angiogenesis?

Back 67

Endothelin-1

Front 68

Is Esophageal involvement common in diffuse or limited scleroderma?

Back 68

both
-impaired conractility of the esophagus presents as dysphagia and odynophagia for solids

Front 69

what antibody usually present in both limited or diffuse?

Back 69

+ANA in 90% of patients, both

Front 70

what antibody assoicated w/ Limited?

Back 70

Anti-centromere 70-80% of time

Front 71

what antibody associated with diffuse?

Back 71

+ScL-70 in 50-70% of diffuse (anti-DNA topoisomerase I)

Front 72

How is scleroderma dx made?

Back 72

clinical grounds and confirmed w/ laboratory testing

Front 73

All patients need a baseline assessment of?

Back 73

internal organ invovlement: pulmonary, esophageal, myocardial, renal, thyroid

Front 74

Are there any reliable tests to track scleroderma dz progression?

Back 74

no

Front 75

Diffuse, limited, or both:
-abnormal peristalsis w/ delayed emptying?

Back 75

abnormal peristalsis of esophagus associated w/ both, but GI more typical of diffuse

Front 76

limited, diffuse, or both?
-interstitial lung dz and fibrosis

Back 76

lungs can be involved in either
**fibrosis more associated w/ diffuse
*pulmonary hypertension more associated w/ limited

Front 77

Raynaud's digital pits?

Back 77

I think both, i know Raynaud's can occur with both, but not certain about digital pits

Front 78

Pulmonary hypertension w/o fibrosis?

Back 78

def. limited

Front 79

Renal crisis w/ hypertension

Back 79

diffuse
-only organ limited typically affects is lungs