Raynaud's Syndrome

Introduction

Raynaud's is an exaggerated vasocontriction of the digital arteries in response to a variety of stimuli. It takes two forms Raynaud's Phenomenon or Raynaud's Syndrome vasospastic disease with a known underlying cause Raynaud's Disease  vasospastic disease with no known cause (idiopathic) Pathophysiology  periodic digital ischemia due vasoconstriction induced by cold temperature or  sympathetic stimuli including pain or emotional stress triphasic color change (white-blue-red progression)  digits turn white from vasospasm and interruption of blood flow  blue discoloration follows from cyanosis and venous stasis  finally digits turn red as a result of rebound hyperemia dysesthesias often follow color changes

Raynaud's Phenomenon

Vasospastic disease with a known underlying disease epidemiology demographics occasional female predominance age >40 years (generally older than patients with Raynaud's disease) location affects the distal aspect of digits associated conditions connective tissue disease scleroderma (80-90% incidence of Raynaud's phenomenon) SLE (18-26%) dermatomyositis (30%) RA (11%) CREST syndrome calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias neurovascular compression (thoracic outlet syndrome) Presentation symptoms asymmetric findings rapid progression physical exam peripheral pulses often absent frequent trophic skin changes (including ulceration and gangrene)   abnormal Allen test Imaging radiographs normal and not indicated  Studies labs blood chemistry - often abnormal invasive studies microangiology - often abnormal angiography - often abnormal

Raynaud's Disease

Vasospastic disease with no known cause (idiopathic) epidemiology seen in young premenopausal women (age <40 years) Presentation symptoms  often bilateral slow progression physical exam peripheral pulses usually present trophic skin changes are uncommon normal Allen test  Imaging radiographs normal and not indicated  Studies labs usually normal invasive studies usually normal diagnosis  based on Allen and Brown criteria

Allen and Brown Criteria for Raynaud's Disease Intermittent attacks with discoloration of acral parts Bilateral involvement Absence of clinical arterial occlusion Gangrene and trophic changes are rare Symptoms present for >2 years Absence of other disease to explain findings Predominance in women

Differential Diagnosis

Factors to differentiate Raynaud's Phenomenon or Raynaud's Disease   age gender time frame of symptom progression severity of exam findings

Treatment

Nonoperative medical management & lifestyle modifications indications first line of treatment modalities smoking cessation and avoidance of cold exposure is critical thermal biofeedback techniques medications include  topical nitrates  calcium channel blockers ASA intra-arterial reserpine dipyridamole (Persantine) pentoxifylline (Trental) Sildenafil Botulinum toxin A injections  indications  medical management has failed  presence of ischemia and pain ulcerations not a contraindication note this is "off-label" use   Shown to improve digital perfusion  Operative thorascopic sympathectomy indications fallen out of favor due to rebound hypersympathetic response digital sympathectomy  indications severe cases that fail conservative treatment (impending gangrene) periarterial sympathectomy most common method removal of adventitia and sympathetic nerve fibers may extend to forearm sympathectomy is not indicated calcific arterial disease  microvascular reconstruction indications may be indicated in rare situations with occlusion of small segment of one major artery while other is spared/minimal involvement

Complications

digital ischemia leading to ulcerations and/or gangrene may necessitate amputation of digit(s)

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