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