Chapter 141 - Raynaud phenomenon Flashcards
First description of Raynaud phenomenon
Maurice Raynaud 1862
Definine Raynaud’s
1) exaggeration of normal physiologic response
2) episodic pallor/cyanosis caused by vasoconstriction of small digital arteries or arterioles in response to cold or emotional stress
Cause of the color change stages in Raynauds
1) pallor = vasospastic attack
2) cyanosis = static blood desaturated
3) rubor = hyperemia from post-ischemic vasodilatation
Raynaud’s nomenclature
Raynauds syndrome: all types
Raynauds phenomenon: used to mean secondary now means both
Raynaud’s disease: primary raynauds only
Primary raynaud’s phenomenon = idiopathic and most common
Secondary raynauds phenomenon = underlying disease process
Causes of secondary raynauds phenomenon
1) systemic sclerosis
2) rheumatoid arthritis
3) connective tissue disease
has underlying FIXED occlusive disease
Epidemiology of raynauds phenomenon
1) prevalence 3.3 - 22%
2) women > men 1.6:1
% of people without complete superficial arch
21.5%
% of extremities where all 5 digits are supplied by both deep and superficial arches
86%
The hunting response in hand
With cold exposure regular rhythmic fluctuation in finger flow with periods of vasoconstriction and vasodilatation very 30sec to 10 min
Maximum vasoconstriction in hand occurs at this temperature
10-20 celcius
Pathogenesis theories of Raynauds by Raynaud and Lewis
1) hyperactivity of sympathetic NS (Raynaud) - disproven
2) local vascular fault cause increased sensitivity to cold (Lewis)
Categories of pathogenesis of Raynauds
1) Vascular
2) Humoral
3) Neural
Impaired vasodilatation in raynauds
Decreased NO formation
Endothelial-derived relaxing factors
1) NO
2) prostacyclin
3) ATP
Diseases with decreased NO formation
1) Raynauds
2) systemic sclerosis
Increased vasoconstriction in Raynauds caused by
Increase endothelin 1 concentration and activity
Neural pathway at synaptic cleft in response to cold
Cold –> norepinephrine release –> post-synaptic alpha2 receptor –> vascular vasoconstriction
Abnormal humoral factors in Raynauds
1) estrogen increase expression of alpha2 adrenoceptors
2) increased serotonin
3) increased thromboxane and B-thromboglobulin
4) increased tpa inhibitor
Drugs associated with Raynauds
1) beta-blockers
2) chemotherapeutic agents (vinblastine, bleomycin)
3) bromocriptine
4) amphetamine
5) cocaine
6) ergot
Risk factors for Raynauds
1) family history
2) smoking
3) vibration
Connective tissue disease associated with Raynauds
1) scleroderma
2) SLE
3) RA
4) Sjogren
5) mixed CTD
6) dermatomyositis, polymyositis
7) vasculitis
Occlusive arterial disease associated with Raynauds
1) atherosclerosis
2) Buerger
3) GCA
4) emboli
5) TOS
Occupational arterial disease associated with Raynauds
1) Hypothenar hammer
2) vibration
Myeloproliferative and hematologic disease associated with Raynauds
1) polycythemia rubra vera
2) thrombocytosis
3) cold agglutinins
4) cryoglobulinemia
5) paraproteinemia
Malignancy associated with Raynauds
1) multiple myeloma
2) leukemia
3) adenocarcinoma
4) astrocytoma
Infections associated with Raynauds
1) Hepatitis B and C
2) Parvovirus
3) Purpura fulminans
Thumb involvement in raynauds
Rare
usually means secondary raynauds
Duration of Raynauds attack
10-20 minutes
Other associated diseases with primary raynauds
1) Prinzmetal’s angina
2) migraine
Diagnostic criteria for primary raynauds
1) vasospastic attack precipitated by cold or stress
2) attack of both hands are asynchronous/asymmetric
3) no tissue necrosis/gangrene
4) no other cause noted
5) normal nail fold capillaries
6) negative serology
Most common site of ulnar artery blockade
Hypothenar eminence where it crosses hook of hamate
Segmental pressure cutoff for indication of occlusive disease
10 mmHg drop
Finger systolic pressure measurement cutoff for disease
15 mmHg between fingers
< 70 mmHg absolute
Diagnostic tests for Raynauds
1) vascular lab
2) segmental pressure
3) finger pressure plethysmography
4) cold challenge with temperature measurement
5) nail fold capillary microscopy
6) serology
Cold challenge test description for raynauds
1) baseline digital temperature
2) immerse hand in cold 4C for 20 seconds
3) dry hands and warm hand in room temperature
4) length of time to rewarm measured
Rewarming > 10 min suggest raynauds
Nail fold capillary in raynauds
enlarged, tortuous and deformed in scleroderma and mixed CTD
1st line Treatment for primary raynauds
Prevention and avoid stressors
dihydropyridine Calcium channel blockers in treating raynauds types
1) Nifedepine 10-30 mg po qd-tid (most extensively studied)
2) Nicardipine 20-50 po bid
3) amlodipine 10 mg po daily
4) felodipine 5-20 mg po daily
nisoldipine 5-10 po daily
Non-dihydropyridine calcium channel blockers in raynauds
1) Diltiazem 30-120 mg po tid
2) Verapamil no benefit
less side effect than dihydropyridine but not as effective
Side effect of dihydropyridines
1) hypotension
2) flushing
3) edema
4) palpitations
5) dizziness
Alpha 1 recepto antagonist in raynauds
1) Prazosin 1 mg po tid (useful in secondary raynauds)
2) Terazosin 1 mg po daily
Other classess of drugs in Raynauds
1) ACEi (captopril, enalopril, quinapril)
2) ARB (losartan)
3) SSRI (fluoxetine)
4) PDVi (sildenafil, tadalafil, vardenafil)
5) Nitrate (topical nitroglycerin)
6) prostaglandins (epoprostenol, iloprost)
7) endothelin receptor antagonist (bosentan)
Surgical therapy in raynauds
1) Botulinum toxin
2) Sympathetic block
3) thoracoscopic sympathectomy (high recurrence in primary raynauds but helps in secondary)
4) digital sympathectomy
5) spinal cord stimulators
6) acupuncture
7) laser therapy
