Chapter 145 - Raynaud Phenomenon Flashcards by Beatrice Maxine Chan

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

Raynaud phenomenon

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Most common underlying cause of secondary Raynaud phenomenon

Systemic sclerosis

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Triphasic sequence of Raynaud phenomeon

Pallor (vasoconstriction, ischemia)
Cyanosis (venous stasis)
Erythema (compensatory reperfusion)

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

10%

90%

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

Second; F

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Fundamental to diagnosis of RP

Clear description of vasospastic attacks

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

Pallor (ischemic)

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

30 minutes

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Signs of prolonged attacks of RP

Trophic changes of the digits

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Complications of ulcerations of Raynaud phenomenon (3)

Digital osteomyelitis
Gangrene
Autoamputation

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Critical part of evaluating patient with RP

Careful inspection of proximal nailfold with aid of microscopy

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

SSc-specific autoantibodies

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Independent predictor for progression to SSc

Microvascular damage

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

Allen test

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

Adson maneuver

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

15; 1st

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Features predictive of progression include (4)

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

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Most common cause of secondary RP

Connective tissue disease

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

True

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Drugs associated with Raynaud phenomenon (11)

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

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__ manufacturing in polymer industry confers risk for developing RP

Vinyl chloride

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Most common cause of iatrogenic RP

B adrenergic blockers (propranol)

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Pathophysiology of IFN induced RP (4)

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

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Mechanisms involved in pathogenesis of Raynaud phenomenon (3)

Vascular abnormalities
Neural abnormalities
Intravascular abnormalities

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

Nitric oxide | Prostacyclin

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

Endothelin 1 | Angiotensin II

Inhibitor of endothelin receptor 1

Bosentan

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

cGRP

___ abnormalities to SSc RP more than primary RP

Intravascular

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

Neuromuscular Ach receptor Serotonin 1B receptor Serotonin 1E receptor

Criteria for primary Raynaud phenomenon (8)

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

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

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

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

CBC UA ANA ESR

Helpful adjunctive tool predictive of progression to SSC

Nailfold capillaroscopy

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

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

Dynamic nailfold changes are observed in primary RP | True or False

False, not observed

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

Thermal imaging (thermography)

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

Acrocyanosis

Difference of acrocyanosis with raynaud phenomenon

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

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

Pernio

Difference of pernio and Raynaud phenomenon

Benign, self-limiting only lasts 1-3 weeks

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

Erythromelalgia

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

True

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

Erythromelalgia

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

Livedo reticularis

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

Reflex sympathetic dystrophy

Distinguishing features of reflex sympathetic dystrophy (5)

``` Disproportionate levels of pain Hypo-/Hypertrichosis Hyper-/Anhidrosis Nail changes Soft tissue atrophy ```

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

False, in secondary disease

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

Raynaud crisis

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

1. SSc with coexisting macrovascular disease | 2. Positive anticentromere antibodies

Poor prognostic sign for Raynaud

Development of digital ulcers

Validated reliable measure for RP activity

Raynaud condition score

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

Patient Acceptable Symptom State

Effective at reducing frequency and severity of attacks

Behavioral modification Pharmacologic therapy Surgical intervention

Mild RP is treated with

Lifestyle changes/behavioral modifications

Examples of behavioral modification

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

Induces cutaneous vasoconstriction and can provoke attacks of RP

Nicotine

Enhance vasoconstriction by blocking adenosine receptors

Caffeine

Important to minimize vasoconstriction in those with hyperactivity of SNS

Stress modification

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

Calcium channel blockers

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

``` Dihydropyridine class of CCBs Nifedipine 30-90mg XR OD Every evening ```

Adverse effects of CCBs (4)

Headache Flushing Hypotension Lower extremity edema

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

Gastroesophageal reflux symptoms

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

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

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

PDE inhibitors | Sildenafil 20 mg TID (short acting)

ADE of PDE inhibitors

Headache dyspepsia Dizziness Visual disturbances

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

Iloprost 0.5-2ng/kg/min IV

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

Scleroderma renal crisis

Desirable in patients with RP triggered by emotional anxiety

Fluoxetine 20-40mg daily

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

Bosentan 62.5 mg BID for 4 weeks

ADE of bosentan in 10%

Dose dep. elevation of liver transaminases

Side effects of alpha1 adrenergic blockers

Postural hypotension Drowsiness Fatigue

Central role in actin-dep and myosin-dep vasoconstriction

Rho kinases

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

Pentoxifylline 400-800mg TID

Treatment for Raynaud crisis

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

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

40-100; thumb

Complications related to botulinum toxin

Dysesthesia | Transient hand weakness

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

Sympathectomy

Traditional approach of sympathectomy

Thoracocervical

ADE of sympathectomy

Hyperhidrosis Horner syndrome Persistent neuropathic pain

Advantage of digital vs thoracic sympathectomy

Serious complications are infrequent

Cultures of digital ulcers usually demonstrate

Staphylococcus aureus

Treatment for MRSA

TMP-SMX Clindamycin Tetracycline

Important element of ulcer care

Skilled nursing and podiatry care

Chemical sympathectomy with __ may also be used to reverse vasoconstriction

Lidocaine | Bupivacaine