Raynaud's Disease & Raynaud's Phenomenon

Introduction

Essentials of Diagnosis

• Episodic bilateral digital pallor, cyanosis, and rubor.
  
• Precipitated by cold or emotional stress; relieved by warmth.
  
• Seventy to 80 percent of patients are women.

General Considerations

Raynaud's syndrome is an episodic vasospastic disorder characterized by digital color change (white-blue-red) with exposure to cold environment or emotional stress. If idiopathic, it is called Raynaud's disease. If associated with a possible precipitating systemic or regional disorder (autoimmune diseases, myeloproliferative disorders, multiple myeloma, cryoglobulinemia, myxedema, macroglobulinemia, or arterial occlusive disease), it is called Raynaud's phenomenon.

The incidence of disease is estimated to be as high as 10% in the general population. Other vasospastic disorders, such as variant angina and migraine headache, are common in patients with Raynaud's syndrome. An abnormality of the sympathetic nervous system has long been implicated in the etiology of Raynaud's disease; recently, research has focused on the theory of up-regulation of vascular smooth muscle a2-adrenergic receptors.

Clinical Findings

Classically, Raynaud's disease and Raynaud's phenomenon are characterized by intermittent attacks of pallor of the hands or fingers brought on by cold or emotional stress, progressing to cyanosis and then rubor on rewarming. Mild discomfort, paresthesias, numbness, and trace edema often accompany the color changes. In Raynaud's disease, the disease is symmetric, by rule; in Raynaud's phenomenon, the changes may be most noticeable in one hand or even in one or two fingers only. Infrequently, the feet and toes are involved. Between attacks, the affected extremities may be entirely normal.

The distinction between Raynaud's disease and Raynaud's phenomenon is meant to reflect a difference in prognosis. Whereas Raynaud's disease is benign and often controllable, Raynaud's phenomenon may progress to atrophy of the terminal fat pads and development of fingertip gangrene. However, because many inciting diseases may not be clinically suspected at the time the diagnosis of Raynaud's disease is made, there is a large degree of crossover between groups. The diagnosis is based on clinical criteria. Raynaud's disease appears first between ages 15 and 45, almost always in women. A patient with suggestive symptoms that persist for over 3 years without evidence of an associated disease is given the diagnosis of Raynaud's disease.

Differential Diagnosis

A patient with Raynaud's syndrome must be evaluated for possible inciting systemic disorders. Directed history and physical examination and serologic testing may be helpful in excluding the collagen-vascular disorders, which include scleroderma, systemic lupus erythematosus, dermatomyositis, and rheumatoid arthritis. It is estimated that 80% of patients with scleroderma ultimately develop Raynaud's phenomenon. Likewise, cryoglobulinemia can be excluded by appropriate testing. Raynaud's phenomenon is also occasionally seen in patients with neurogenic thoracic outlet syndrome or carpal tunnel syndrome. Frostbite, ergotamine toxicity, and use of chemotherapeutic agents, which can also be associated with Raynaud's phenomenon, can usually be excluded by a careful history; up to one-third of patients receiving combined bleomycin and vincristine (such as for testicular cancer) develop symptomatic vasospastic disease. An abnormal or asymmetric pulse examination, differential blood pressure cuff measurements, gangrene, or a positive Allen test are suggestive of arterial occlusive disease and should prompt upper extremity angiography to exclude stenosis or occlusion from atherosclerosis, Buerger's disease, arterial thoracic outlet syndrome, embolic disease, or repetitive motion injury of the small vessels of the hand. Even undiseased upper extremity vessels often show intense vasospasm with contrast injection, so the arm is wrapped for warmth and prophylactic use of a vasodilator (papaverine or nitroglycerin, 30 mg, injected through the angiography catheter) is advised; both arms are imaged for side-by-side comparison.