Raynaud's Phenomenon: A Common Sense Approach to Diagnosis and Management

Raynaud's Phenomenon: A Common Sense Approach to Diagnosis and Management Posted By:
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Raynaud's phenomenon (RP) is all too common in New England during the colder months of the year. In October, we often see patients return who have been previously diagnosed with RP secondary to another rheumatic disease. Rheumatic disorders associated with secondary RP include systemic sclerosis, limited scleroderma, dermatomyositis, mixed connective tissue disease, systemic lupus erythematosus, rheumatoid arthritis, Buerger's disease, and vasculitis.

Classically, RP changes occur in three color phases. White is caused by blanching of the digital arterioles during vasospasm (see image below); cyanosis occurs as static venous blood deoxygenates; and rubor is caused by reactive hyperemia. Not all patients have three-phase Raynaud's, but the most prominent phase is pallor of a finger or toe followed by either cyanosis or redness. Cold is the most common stimulus, and activities as simple as passing by the refrigerator section in a grocery store or holding a can of cold soda can stimulate an episode. Emotional distress, trauma, hormones, and chemicals found in smoke can also trigger RP.

Blanching fingers of RP

Blanching fingers of Raynaud's phenomenon.
Image attributed to Profpedia at English Wikipedia. No alterations were made to the image by PCE.
Image license: creativecommons.org/licenses/by-sa/4.0/legalcode.

In the workup of a patient with Raynaud's who has clinical evidence of systemic autoimmune disease, laboratory studies should include antinuclear antibodies (ANA), looking for a nucleolar pattern, anti-centromere, and anti-topoisomerase (ScL-70, IgG, and IgA - rarely IgM) antibodies. Symptomatic patients with an appropriate history should be screened for hypercoagulable states, cryoglobulinemia, anti-phospholipid syndrome (APLS), and hypothyroidism. One third of patients will exhibit low titer ANA in their serum, and 25% of these patients will also have an autoimmune disorder. It is also useful to utilize nailfold capillary microscopy; this is performed with an ophthalmoscope using a diopter setting of 40 and an immersion oil droplet placed on the cuticle. Dilated and tortuous capillary loops and areas of avascularity are often found in those at risk of developing a rheumatic disease, or who have underlying rheumatic disorders, whereas those with a negative nailfold exam rarely develop rheumatic disease.

Treatments for mild to moderate RP are primarily aimed at common sense measures to mitigate cold temperatures in both the core and extremities. Some recommendations for prevention include planning one's day to minimize exposure; wearing loose fitting clothing in layers; and wearing warm socks, hats, and scarves, as well as mittens or lined gloves. It is also important for patients with RP to avoid tobacco; sympathomimetic drugs such as decongestants, ADD, and ADHD medications; beta blockers; and diet pills where possible. As an extra measure for warmth, I often recommend hand placement in armpits in a cross-armed position. Hand and foot warmers placed in gloves and in shoes are also of some benefit when prolonged exposure cannot be avoided.

Many patients with underlying rheumatic disease use calcium channel blockers (CCBs), which cause peripheral vasodilation, thereby preventing vasospasm. Slow-release preparations of nifedipine, amlodipine, and diltiazem are usually well tolerated and efficacious. Nifedipine has the added benefit of platelet inhibition, which may increase its effectiveness. Side effects can include hypotension and peripheral edema.

If patients fail to respond to CCBs, direct vasodilators may be helpful. Topical 2% nitroglycerin ointment applied at the wrist in the morning and six hours later can be helpful, using a ¼ to ½ inch strip. A 12-hour rest period is required to maintain efficacy over time. Other direct vasodilators that can be tried are hydralazine, minoxidil, and niacin.

Additional treatments include indirect vasodilators, such as fluoxetine, losartan, sildenafil, and bosentanall of which have been effective in some trials.

For patients unresponsive to the above measures and who are experiencing an acute ischemic crisis with threat of a digital infarct, several steps should be considered:

  • Admit to the hospital
  • Put the patient in a warm and quiet environment
  • Control pain with a chemical sympathectomy (eg, with prostaglandin 1 or prostaglandin E), which can allow needed medications to get to the affected digits
  • Try to avoid narcotics; they increase vasospasm but can be used if needed
  • Anti-platelet therapy with aspirin 81 mg
  • Heparin, especially if the patient has APLS

Surgical options are available for those who are refractory to chemical intervention.

In summary, Raynaud's is estimated to affect between 3% and 4% of the population, but in colder climates this may be as high as 30%. Women outnumber men by a ratio of 4:1 to 9:1. Most patients respond to common sense measures of avoiding exposure to cold and using warm layered clothing, avoiding tobacco products, and avoiding certain medications. For those not responsive to these lifestyle interventions, CCBs are often effective and safe.

References
  • Herrick AL. Pathogenesis of Raynaud's phenomenon. Rheumatology (Oxford). 2005;44:587-596.
  • Hirschl M, Hirschl K, Lenz M, et al. Transition from primary Raynaud's phenomenon to secondary Raynaud's phenomenon identified by diagnosis of an associated disease: Results of ten years of prospective surveillance. Am Coll Rheum. 2006;54:1974-1981.
  • Lambova SN, Muller-Ladner U. The role of capillaroscopy in differentiation of primary and secondary Raynaud's phenomenon in rheumatic diseases: a review of the literature and two case reports. Rheumatol Int. 2009;29:1263-1271.
  • Thompson AE, Pope JE. Calcium channel blockers for primary Raynaud's phenomenon: a meta-analysis. Rheumatology (Oxford). 2005;44:145-150.

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Filed under: Miscellaneous , Rheumatology

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