30 Raynaud Phenomenon and Vasomotor Syndromes

Question 1

MC reported initiators of RP

Answer 1

1) Cold
2) Emotional stress
3) Exercise

Question 2

Relative shifts from cool to warmer temp can trigger RP

Answer 2

T

Question 3

Primary vs Secondary RP: Raynaud sign

Answer 3

Primary

Question 4

Primary vs Secondary RP: Raynaud syndrome

Answer 4

Secondary

Question 5

Generally, RP is more common among

Answer 5

1) Women
2) Younger age groups
3) Family members of patients with RP

Question 6

T/F Majority of patients with RP do not have, nor will have, an associated rheumatologic or vascular disorder

Answer 6

T

Question 7

RP occurs at high freq in these rheumatic conditions and is often the initial symptom of these disorders

Answer 7

SSc or MCTD

Question 8

Primary vs Secondary RP: Older age of onset

Answer 8

Secondary

Question 9

Primary vs Secondary RP: Male gender

Answer 9

Secondary

Question 10

Primary vs Secondary RP: Symmetrical involvement

Answer 10

Primary

Question 11

Primary vs Secondary RP: Asymmetrical

Answer 11

Secondary

Question 12

Primary vs Secondary RP: Absence of tissue necrosis, digital pitting, gangrene

Answer 12

Primary

Question 13

Primary vs Secondary RP: Absence of dilated loops on nailfold capillary exam

Answer 13

Primary

Question 14

Primary vs Secondary RP: Signs and symptoms of ischemia proximal to fingers or toes

Answer 14

Secondary

Question 15

Primary vs Secondary RP: Abnormal lab parameters

Answer 15

Secondary

Question 16

Factors influencing vascular reactivity

Answer 16

Box 30.2 (p.429)
1) Arterial smooth muscle cells
2) Endothelial cell products
3) Sympathetic nervous system
4) Neuropeptides
5) Other

Question 17

T/F Primary RP is fully reversible

Answer 17

T

Question 18

Effect of decreased temp to blood vessels

Answer 18

Selectively increases response of arterial smooth muscle cells to norepinephrine

Question 19

Endothelial cell products that influences vascular reactivity and their effects

Answer 19

1) NO (vasodilation)
2) Prostacyclin (vasodilation)
3) Endothelin-1 (vasoconstriction)

Question 20

Effect of sympathetic nervous system to blood vessels

Answer 20

NE causes vasoconstriction

Question 21

Neuropeptide-effect to blood vessel: Substance P

Answer 21

Vasodilation

Question 22

Neuropeptide-effect to blood vessel: Vasoactive intestinal peptide

Answer 22

Vasodilation

Question 23

Neuropeptide-effect to blood vessel: Calcitonin gene-related peptide

Answer 23

Vasodilation

Question 24

Neuropeptide-effect to blood vessel: Neurokinin A

Answer 24

Vasodilation

Question 25

Neuropeptide-effect to blood vessel: Somatostatin

Answer 25

Vasoconstriction

Question 26

Neuropeptide-effect to blood vessel: Neuropeptide Y

Answer 26

Vasoconstriction

Question 27

Effect of angiotensin to blood vessel

Answer 27

Vasoconstriction

Question 28

SSc-associated RP fundamentally differs from primary RP because of its associated vasculopathy involving

Answer 28

1) Fibrous intimal proliferation 2) Intrvascular thrombi

Question 29

Potent vasoconstrictors with profibrotic activities that have been shown to be overexpressed in the skin of patients with SSc and other forms of secondary RP

Answer 29

1) Endothelin-1 2) Angiotensin

Question 30

3 main classes of neurons that can innervate blood vessels

Answer 30

1) Sympathetic vasoconstrictor neurons 2) Sympathetic or parasympathetic vasodilator neurons 3) Sensory neurons that can also mediate vasodilation

Question 31

Local nerve endings can also sense the microenvironment and release these vasodilating substances

Answer 31

1) Substance P 2) VIP 3) Calcitonin gene-related peptide 4) Neurokinin A

Question 32

Local nerve endings can also sense the microenvironment and release these vasoconstricting substances

Answer 32

1) Somatostatin 2) Neuropeptide Y

Question 33

These receptors in digital and cutaneous vesselsare abnormal with enhanced response to stimulation in patients with primary and secondary RP

Answer 33

Alpha adrenergic receptors

Question 34

How cold can cause vasoconstriction

Answer 34

Selectively amplifies vascular smooth muscle constriction in response to NE mediated through alpha adrenergic receptors

Question 35

How estrogen can cause vasoconstriction

Answer 35

Increase alpha-2 adrenergic receptors in vascular smooth muscle

Question 36

Conditions associated with secondary RP in children

Answer 36

Box 30.3 (p.430)

Question 37

T/F History alone is accepted as diagnostic for RP in general practice

Answer 37

T

Question 38

Diagnostic modality that is associated with danger of precipitating acute catastrophic arteriolar spasm

Answer 38

Arteriography

Question 39

T/F Tri-phasic color changes occur in a majority of patients with RP

Answer 39

F

Question 40

Frequently involved digits

Answer 40

Index, middle, and ring finger

Question 41

Digit often spared in RP

Answer 41

Thumb

Question 42

Tender red SC nodules seen in RP

Answer 42

Pernio

Question 43

In general RP is more common among: M or F

Answer 43

F

Question 44

In general RP is more common among: Young or old

Answer 44

Young

Question 45

T/F RP is more common among family members of patients with RP

Answer 45

T

Question 46

T/F A large majority of patients with RP do not have and will not develop an associated rheumatic disease

Answer 46

T

Question 47

RP occurs at a high frequency for these 2 disorders and is often the initiating symptoms

Answer 47

1) SSc 2) MCTD

Question 48

T/F ESR is necessary in the workup of RP

Answer 48

F, unnecessary and can be misleading

Question 49

T/F ANA is necessary in the workup of RP

Answer 49

F, unnecessary and can be misleading

Question 50

Autoantibodies that can be done for patients with RP that are most sensitive for predicting evolution to SSc

Answer 50

Anti-centromere, antitopoisomerase (anti-Scl70)

Question 51

T/F most pediatric patients with secondary RP have a (+) ANA

Answer 51

T, 85-100%

Question 52

T/F a significant number of patients with primary RP have a (+) ANA without evidence for assoc with a rheumatic disorder

Answer 52

T

Question 53

Compared to RP, perniosis

Answer 53

lacks blanching

Question 54

Compared to RP, acrocyanosis

Answer 54

does not present with a sharp demarcation

Question 55

General measures for mgt of RP

Answer 55

Pg 433, box 30.4

Question 56

First-line in the treatment of RP

Answer 56

Non-pharmacologic measures

Question 57

TReatment algorithm for uncomplicated RP

Answer 57

Pg 434 Fig 30.4

Question 58

TReatment algorithm for uncomplicated RP

Answer 58

Pg 434 Fig 30.5

Question 59

T/F Secondary RP is less likely to show a complete response to CCB compared to primary RP

Answer 59

T

Question 60

T/F ACEi are effective for RP

Answer 60

F

Question 61

First-line pharma agents for uncomplicated RP

Answer 61

CCB

Question 62

Second-line pharma agents for uncomplicated RP

Answer 62

Direct vasodilators: NTG, Hydralazine, Minoxidil Indirect vasodilators: Sympatholytics (prazosin), SSRI (fluoxetine), ACEi (captopril), ARB (losartan), PDEi (sildenafil), Endothelin-1 antagonist (bosentan)

Question 63

First-line pharma agents for complicated RP

Answer 63

CCB, aspirin low dose, pain ctrl

Question 64

2nd-line pharma agents for complicated RP

Answer 64

Topical NTG (1%) Na nitroprusside LMW heparin Local or regional sympathetic block

Question 65

Major goals in secondary RP

Answer 65

Reduce frequency and severity of attacks Prevent new digital ulcerations NOT to completely terminate attacks (unrealistic)

Question 66

Key to success in the setting of critical digital ischemia in RP

Answer 66

Early intervention