Chapter 145 - Raynaud Phenomenon Flashcards
Vascular disorder characterized by recurrent episodic attacks of digital ischemia provoked by exposure to cold or emotional stress
Raynaud phenomenon
Most common underlying cause of secondary Raynaud phenomenon
Systemic sclerosis
Triphasic sequence of Raynaud phenomeon
- Pallor (vasoconstriction, ischemia)
- Cyanosis (venous stasis)
- Erythema (compensatory reperfusion)
RP affects __% of general population and __% of patients with systemic sclerosis
10%
90%
RP develops during the __ decade of life with __ predominance 4:1
Second; F
Fundamental to diagnosis of RP
Clear description of vasospastic attacks
If pain is prominent during ___ phase, a secondary cause is more likely.
Pallor (ischemic)
Episodes of RP usually last for __ but may persist for hours.
30 minutes
Signs of prolonged attacks of RP
Trophic changes of the digits
Complications of ulcerations of Raynaud phenomenon (3)
Digital osteomyelitis
Gangrene
Autoamputation
Critical part of evaluating patient with RP
Careful inspection of proximal nailfold with aid of microscopy
Presence of ___ portend a 60 fold risk for progression to systemic sclerosis
SSc-specific autoantibodies
Independent predictor for progression to SSc
Microvascular damage
Assess arterial and capillary function of the hands by simultaneously compression of radial and ulnar arteries
Allen test
Test for diminution of radial pulse with exaggerated maneuver of neck and shoulder to diagnose thoracic outlet syndrome
Adson maneuver
Progression of primary to secondary form of RP is as high as __% during the __ decade of onset.
15; 1st
Features predictive of progression include (4)
Nailfold capillary abnormalities
Hand edema (puffy hands)
Positive Allen’s test
Antinuclear antibodies
Most common cause of secondary RP
Connective tissue disease
Incidence of RP in RA patients is equal to that of general population.
True or False
True
Drugs associated with Raynaud phenomenon (11)
Amphetamines Beta blockers Bleomycin Clonidine Cyclosporine IfN alpha and beta Imatinib Vinblastine Methylsergide Ergot derivatives OCPs
__ manufacturing in polymer industry confers risk for developing RP
Vinyl chloride
Most common cause of iatrogenic RP
B adrenergic blockers (propranol)
Pathophysiology of IFN induced RP (4)
Direct vasospastic effect
Increase in blood viscosity
Deposition of immune complexes
Arterial occlusion by thrombi
Mechanisms involved in pathogenesis of Raynaud phenomenon (3)
Vascular abnormalities
Neural abnormalities
Intravascular abnormalities
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)
- Vasospastic attacks pptd by exposure to cold or emotional stimuli
- Bilateral involvement of extremities
- Normal vascular examination with normal nailfold capillary microscopy and symmetric pulses
- Absence of gangrene
- No evidence of underlying disease, drug, or occupational exposure
- (-) ANA
- Normal ESR
- Hx of symptoms for at least 2 years
Secondary RP is more likely in the ff settings (5)
- Age at onset of 30 years old and more
- Intense, painful, asymmetric episodes associated with ischemic skin lesions
- Clinical signs suggestive of CT disease
- Specific autoantibodies
- 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)
- Capillary dilation (giant capillaries) with focal hemorrhage
- 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
- Persistent, irrespective of exposure to cold temperature or other stimuli
- 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)
- 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
- LWMH for 20 weeks
- Iloprost 0.5-2ng/kg/min infused over 8 hours on 3 days ff by every 6 week infusions
- 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