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Chapter 145 - Raynaud Phenomenon Flashcards

1
Q

Vascular disorder characterized by recurrent episodic attacks of digital ischemia provoked by exposure to cold or emotional stress

A

Raynaud phenomenon

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2
Q

Most common underlying cause of secondary Raynaud phenomenon

A

Systemic sclerosis

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3
Q

Triphasic sequence of Raynaud phenomeon

A
  1. Pallor (vasoconstriction, ischemia)
  2. Cyanosis (venous stasis)
  3. Erythema (compensatory reperfusion)
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4
Q

RP affects __% of general population and __% of patients with systemic sclerosis

A

10%

90%

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5
Q

RP develops during the __ decade of life with __ predominance 4:1

A

Second; F

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6
Q

Fundamental to diagnosis of RP

A

Clear description of vasospastic attacks

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7
Q

If pain is prominent during ___ phase, a secondary cause is more likely.

A

Pallor (ischemic)

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8
Q

Episodes of RP usually last for __ but may persist for hours.

A

30 minutes

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9
Q

Signs of prolonged attacks of RP

A

Trophic changes of the digits

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10
Q

Complications of ulcerations of Raynaud phenomenon (3)

A

Digital osteomyelitis
Gangrene
Autoamputation

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11
Q

Critical part of evaluating patient with RP

A

Careful inspection of proximal nailfold with aid of microscopy

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12
Q

Presence of ___ portend a 60 fold risk for progression to systemic sclerosis

A

SSc-specific autoantibodies

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13
Q

Independent predictor for progression to SSc

A

Microvascular damage

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14
Q

Assess arterial and capillary function of the hands by simultaneously compression of radial and ulnar arteries

A

Allen test

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15
Q

Test for diminution of radial pulse with exaggerated maneuver of neck and shoulder to diagnose thoracic outlet syndrome

A

Adson maneuver

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16
Q

Progression of primary to secondary form of RP is as high as __% during the __ decade of onset.

A

15; 1st

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17
Q

Features predictive of progression include (4)

A

Nailfold capillary abnormalities
Hand edema (puffy hands)
Positive Allen’s test
Antinuclear antibodies

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18
Q

Most common cause of secondary RP

A

Connective tissue disease

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19
Q

Incidence of RP in RA patients is equal to that of general population.
True or False

A

True

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20
Q

Drugs associated with Raynaud phenomenon (11)

A 
Amphetamines
Beta blockers
Bleomycin
Clonidine
Cyclosporine
IfN alpha and beta
Imatinib
Vinblastine
Methylsergide
Ergot derivatives
OCPs
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21
Q

__ manufacturing in polymer industry confers risk for developing RP

A

Vinyl chloride

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22
Q

Most common cause of iatrogenic RP

A

B adrenergic blockers (propranol)

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23
Q

Pathophysiology of IFN induced RP (4)

A

Direct vasospastic effect
Increase in blood viscosity
Deposition of immune complexes
Arterial occlusion by thrombi

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24
Q

Mechanisms involved in pathogenesis of Raynaud phenomenon (3)

A

Vascular abnormalities
Neural abnormalities
Intravascular abnormalities

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25
Q

Endothelial dependent vasodilation is reduced in both primary and secondary RP due to inadequate production of __(2)__

A

Nitric oxide

Prostacyclin

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26
Q

High circulating levels of __(2)__ contributes to acral hypoperfusion

A

Endothelin 1

Angiotensin II

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27
Q

Inhibitor of endothelin receptor 1

A

Bosentan

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28
Q

Deficiency of __ in primary and secondary RP leads to altered neurovascular axis contributing to defective vasodilation

A

cGRP

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29
Q

___ abnormalities to SSc RP more than primary RP

A

Intravascular

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30
Q

Polymorphisms in the ff might confer genetic susceptibility to developing primary RP

A

Neuromuscular Ach receptor
Serotonin 1B receptor
Serotonin 1E receptor

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31
Q

Criteria for primary Raynaud phenomenon (8)

A
  1. Vasospastic attacks pptd by exposure to cold or emotional stimuli
  2. Bilateral involvement of extremities
  3. Normal vascular examination with normal nailfold capillary microscopy and symmetric pulses
  4. Absence of gangrene
  5. No evidence of underlying disease, drug, or occupational exposure
  6. (-) ANA
  7. Normal ESR
  8. Hx of symptoms for at least 2 years
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32
Q

Secondary RP is more likely in the ff settings (5)

A
  1. Age at onset of 30 years old and more
  2. Intense, painful, asymmetric episodes associated with ischemic skin lesions
  3. Clinical signs suggestive of CT disease
  4. Specific autoantibodies
  5. Evidence of microvascular disease seen on nailfold microscopy
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33
Q

In all patients with RP the ff should be obtained (4)

A

CBC
UA
ANA
ESR

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34
Q

Helpful adjunctive tool predictive of progression to SSC

A

Nailfold capillaroscopy

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35
Q

Highly specific findings for and can predict progression to SSc (2)

A
  1. Capillary dilation (giant capillaries) with focal hemorrhage
  2. Areas of avascularity (capillary dropout)
36
Q

Dynamic nailfold changes are observed in primary RP

True or False

A

False, not observed

37
Q

Newer diagnostic imaging technique that measures digital and acral temperature and able to distinguish betweeb primary and secondary RP

A

Thermal imaging (thermography)

38
Q

Functional vascular condition characterized by blue violet discoloration of acral skin as a result of diminished oxyhemoglobin

A

Acrocyanosis

39
Q

Difference of acrocyanosis with raynaud phenomenon

A
  1. Persistent, irrespective of exposure to cold temperature or other stimuli
  2. No triphasic color changes
40
Q

Acral, inflammatory dermatosis which develops in response to exposure to cold, wet conditions

41
Q

Difference of pernio and Raynaud phenomenon

A

Benign, self-limiting only lasts 1-3 weeks

42
Q

Rare, functional vascular disease with pain, burning, edema, and erythema of warm extremities

A

Erythromelalgia

43
Q

Attacks of erythromelalgia can occur in evening & at night, and can disrupt sleep. True or False

44
Q

If RP represents excess vasoconstrictive area on a spectrum, ___ represents excess vasodilatory form.

A

Erythromelalgia

45
Q

___ is a very common, localized, physiologic response to cold exposure and appears as mottled, well-formed reticulated vascular pattern that blanched with pressure

A

Livedo reticularis

46
Q

__ is a painful disorder accompanied by vasomotor instability that results from ANS dysfunction due to injury to a limb

A

Reflex sympathetic dystrophy

47
Q

Distinguishing features of reflex sympathetic dystrophy (5)

A 
Disproportionate levels of pain
Hypo-/Hypertrichosis
Hyper-/Anhidrosis
Nail changes
Soft tissue atrophy
48
Q

Complications such as ulceration, soft tissue atrophy, bone loss commonly occur in primary RP.
True or False

A

False, in secondary disease

49
Q

Rapidly progressive form of RP characterized by acute, potentially catastrophic tissue ischemia and is considered a medical emergency

A

Raynaud crisis

50
Q

Susceptibility to Raynaud crisis is highest among patients with (2)

A
  1. SSc with coexisting macrovascular disease

2. Positive anticentromere antibodies

51
Q

Poor prognostic sign for Raynaud

A

Development of digital ulcers

52
Q

Validated reliable measure for RP activity

A

Raynaud condition score

53
Q

Absolute value which objectively evaluate the success of treatments and value which patients consider themselves well

A

Patient Acceptable Symptom State

54
Q

Effective at reducing frequency and severity of attacks

A

Behavioral modification
Pharmacologic therapy
Surgical intervention

55
Q

Mild RP is treated with

A

Lifestyle changes/behavioral modifications

56
Q

Examples of behavioral modification

A

Dress warmly
Smoking cessation
Limit caffeine consumption
Recognize and terminate attacks early

57
Q

Induces cutaneous vasoconstriction and can provoke attacks of RP

58
Q

Enhance vasoconstriction by blocking adenosine receptors

59
Q

Important to minimize vasoconstriction in those with hyperactivity of SNS

A

Stress modification

60
Q

Preferred first line therapy for both primary and SSc associated RP who do not respond to behavioral modifications

A

Calcium channel blockers

61
Q

Most efficacious, least cardioselective, in terms of reducing the frequency and severity of attacks of RP

A 
Dihydropyridine class of CCBs
Nifedipine 30-90mg XR OD Every evening
62
Q

Adverse effects of CCBs (4)

A

Headache
Flushing
Hypotension
Lower extremity edema

63
Q

CCB should be used in caution with SSc patients as it may exacerbate

A

Gastroesophageal reflux symptoms

64
Q

Used to attenuate symptom severity and improved digital ulcer healing times in patients with CT disease-associated RP

A

Topical nitrate or L-arginine 2-8g/day

65
Q

Used for managing patients with CCB- refractory RP by enhancing nitric oxide signaling via inhibition of CGMP degradation

A

PDE inhibitors

Sildenafil 20 mg TID (short acting)

66
Q

ADE of PDE inhibitors

A

Headache
dyspepsia
Dizziness
Visual disturbances

67
Q

Potent vasodilator and inhibits platelet adhesion and aggregation which leads to rapid digital ulcer healing in SSc associated RP

A

Iloprost 0.5-2ng/kg/min IV

68
Q

ARBs is preferred over ACEis in treatment of RP, except in cases of

A

Scleroderma renal crisis

69
Q

Desirable in patients with RP triggered by emotional anxiety

A

Fluoxetine 20-40mg daily

70
Q

Prevents formation of new ulcers and indispensable option for refractory digitl ulcers

A

Bosentan 62.5 mg BID for 4 weeks

71
Q

ADE of bosentan in 10%

A

Dose dep. elevation of liver transaminases

72
Q

Side effects of alpha1 adrenergic blockers

A

Postural hypotension
Drowsiness
Fatigue

73
Q

Central role in actin-dep and myosin-dep vasoconstriction

A

Rho kinases

74
Q

Preferable in RP secondary to cryoglobulinemia, Waldenstrom macroglobulinemia, baseline low BP

A

Pentoxifylline 400-800mg TID

75
Q

Treatment for Raynaud crisis

A
  1. LWMH for 20 weeks
  2. Iloprost 0.5-2ng/kg/min infused over 8 hours on 3 days ff by every 6 week infusions
  3. Intraarterial phentolamine
76
Q

Approximately __ units of onabotulinum toxin A is used per hand, injection of __ is unnecessary

A

40-100; thumb

77
Q

Complications related to botulinum toxin

A

Dysesthesia

Transient hand weakness

78
Q

The most common surgery performed to permanently sever vasoconstrictive signals from SNS innervating the hands

A

Sympathectomy

79
Q

Traditional approach of sympathectomy

A

Thoracocervical

80
Q

ADE of sympathectomy

A

Hyperhidrosis
Horner syndrome
Persistent neuropathic pain

81
Q

Advantage of digital vs thoracic sympathectomy

A

Serious complications are infrequent

82
Q

Cultures of digital ulcers usually demonstrate

A

Staphylococcus aureus

83
Q

Treatment for MRSA

A

TMP-SMX
Clindamycin
Tetracycline

84
Q

Important element of ulcer care

A

Skilled nursing and podiatry care

85
Q

Chemical sympathectomy with __ may also be used to reverse vasoconstriction

A

Lidocaine

Bupivacaine

7th (23 decks)

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