Raynaud's Phenomenon
Raynaud's Phenomenon: Excerpt from The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter
Brian V. Reamy and Douglas C. Warren
Raynaud’s phenomenon (RP) is an episodic vascular disorder of varying severity that results in digital artery ischemia and manifests clinically as a sequence of color changes in the digits. It affects between 3% to 20% of the general population (1).
Approach
The important steps in the evaluation of suspected RP are to establish its presence, then to distinguish primary RP (idiopathic RP or Raynaud’s disease) from secondary RP.
A. Classification. Primary RP is distinguished by the lack of an associated illness. Secondary RP indicates that an underlying disease is responsible. The differentiation between primary and secondary RP is readily made on the basis of the history and physical examination.
B. Epidemiology. RP occurs four times more commonly in women than men. Primary RP usually begins before the age of 30 and a familial history is sometimes present. The prevalence is influenced by geographic location, with more cases reported in colder climates.
C. Special concerns. Although primary RP is a relatively benign disorder, secondary RP is associated with significant morbidity and mortality because of the underlying illness, as well as tissue necrosis from prolonged vasospasm.
History
A. Description of attack. What is the sequence of color changes, including their location, frequency, and duration? Are there associated symptoms?
1. The classic sequence of events is a symmetric, white, blanching, sharply demarcated discoloration, followed by cyanosis, and then redness with resolution of the attack after approximately 15 minutes. Two colors, most commonly white and blue, are sufficient for a firm diagnosis. The presence of only one color makes the diagnosis tenuous.
2. Fingers are usually involved, whereas the thumb and toes are less commonly affected. Rarely, the earlobes, nose, and lips are involved.
3. Associated symptoms can include clumsiness, paresthesias, or pain.
4. Symmetric, mild attacks without tissue loss are typical of primary RP.
5. Asymmetric, prolonged, or intensely painful attacks associated with tissue injury are characteristic of secondary RP.
B. Triggers
1. Cold exposure is by far the most common trigger.
2. Other triggers include emotional stress and nicotine.
C. Underlying illnesses or causes. Are there symptoms or a known underlying disease that suggest secondary RP is present? The most common disorders are connective tissue diseases, especially systemic sclerosis or CREST syndrome; atherosclerotic disease; hyperviscosity syndromes; occupational exposure to intense vibration or vinyl choloride; and medications such as beta- blockers, clonidine, ergot drugs, and some chemotherapeutic agents (vinblastine, bleomycin, and cisplatin).
D. Other information. Does the patient take unopposed estrogen or have a history or migraine headaches, chest pain, or possible Helicobacter pylori infection?
1. Unopposed estrogen therapy or the presence of H. pylori infection can be associated with an increased risk of RP (2,3).
2. Patients with RP report a higher prevalence of migraine headache, and both variant angina and musculoskeletal chest pain (4) (Chapters 2.7 and 7.1).
Physical examination
A. Focused physical examination (PE). The goal of the PE is to uncover sentinel markers for illnesses responsible for secondary RP. In primary RP, the physical examination should be normal. Evaluate for thoracic outlet syndrome, examine distal extremity pulses, and perform an Allen test. Signs of scleroderma, such as sclerodactyly or telangiectasia should be sought, and digital necrosis or ulceration should be identified.
B. Additional PE. If the history suggests an underlying connective tissue disorder, examine the heart, lungs, joints, skin, and nervous system.
Testing
A. Clinical laboratory tests. No “gold standard” test for RP exists. Evaluation is aimed at uncovering secondary causes and should include a complete blood count, erythrocyte sedimentation rate, and antinuclear antibodies (1).
B. Other tests. Angiography can be considered with evidence of vascular occlusive disease. Abnormal nailfold capillary microscopy is a reliable indicator of the presence of underlying connective tissue disorders or of the risk of developing such a disorder. It is a useful adjunctive test in cases where the distinction between primary and secondary RP is unclear. It is performed by placing a drop of immersion oil on the nailfold and observing the capillaries with an ophthalmoscope set at diopter 40. The absence of fine capillaries and the presence of markedly dilated or tortuous vessels are characteristic abnormal findings.
Diagnostic assessment
A history of typical color changes of the digits is essential in the diagnosis of RP. Characterization of the attack as primary or secondary RP is accomplished through history, PE, and basic screening laboratory tests. More than 85% of patients with RP have the primary form, but 10% of patients with apparent primary RP will manifest an underlying disorder an average of 10 years from the onset of their RP (5). Nailfold capillary microscopy and laboratory tests to confirm specific connective tissue diseases should be performed in those cases where a suspicion for secondary RP exists, such as RP onset after age 30, asymmetric digital involvement, prolonged attacks resulting in tissue injury, or abnormal screening laboratory test results. Periodic monitoring for transition to secondary RP in these patients is prudent.
References
1. Wigley FM, Flavahan NA. Raynaud’s phenomenon. Rheum Dis Clin North Am 1996;22:765–781.
2. Fraenkel L, Zhang Y, Chaisson CE, Evans SR, Wilson PWF, Felson DT. The association of estrogen replacement therapy and the Raynaud phenomenon in postmenopausal women. Ann Intern Med 1998:129:208–211.
3. Gasbarrini A, Massari I, Serricchio M, et al. Helicobacter pylori eradication ameliorates primary Raynaud’s phenomenon. Dig Dis Sci 1998;43:1641–1645.
4. O’Keeffe ST, Tsapatsaris NP, Beetham WP. Increased prevalence of migraine and chest pain in patients with primary Raynaud disease. Ann Intern Med 1992;116: 985–989.
5. Spencer-Green G. Outcomes in primary Raynaud phenomenon: a meta-analysis of the frequency, rates, and predictors of transition to secondary diseases. Arch Intern Med 1998:158:595–600.
Book Source Details
- Book Title: The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter
- Author(s): Robert B. Taylor (editor)
- Year of Publication: 2000
- Copyright Details: The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter, Copyright © 2000 Lippincott Williams & Wilkins.
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