Pediatric Raynaud Phenomenon Workup

Updated: Jun 23, 2022
  • Author: Suzanne C Li, MD, PhD; Chief Editor: Lawrence K Jung, MD  more...
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Workup

Laboratory Studies

Laboratory evaluation should be performed to better evaluate the patient for secondary Raynaud phenomenon (RP). Routine screening to look for inflammation and general organ function should include erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), CBC count with differential, comprehensive metabolic panel with plasma creatinine, and urinalysis.

Autoantibody screening can include antinuclear antibody (ANA), anticentromere antibody (ACA), and anti-scl 70 antibody; these autoantibodies have the highest sensitivity for predicting evolution from primary Raynaud phenomenon to secondary Raynaud phenomenon. [71, 9] Most pediatric patients with secondary Raynaud phenomenon have a +ANA (85-100%). [15, 28, 72] However, a significant number of pediatric patients with primary Raynaud phenomenon also have a +ANA. [73, 6] The presence of ACA is associated with the development of CREST syndrome and limited systemic sclerosis in patients with Raynaud phenomenon, whereas anti-scl 70 antibody is associated with the development of diffuse systemic sclerosis. [2, 1]

In adult patients with scleroderma, the presence of ACA is associated with an increased risk for digital ischemia, including ulcers and digit loss. [2]

ACA is rarely found in pediatric systemic sclerosis patients. In adults, presence of systemic sclerosis–specific antibodies with scleroderma pattern of nailfold capillaries is highly associated with progression to systemic sclerosis, with 47% evolving by 5 years, 69% evolving by 10 years, and 79% evolving by 15 years.

Additional autoantibody and complement screening can be performed in patients suspected of having conditions that could evolve to other connective tissue diseases such as systemic lupus erythematosus, mixed connective tissue disease, or Sjögren syndrome. These include anti-Sm, anti-double stranded DNA ab, rheumatoid factor, antiribonucleoprotein, anti-Ro, anti-La, and antiphospholipid antibodies. [28] About 30% of pediatric patients with primary Raynaud phenomenon or secondary Raynaud phenomenon were found to have antiphospholipid antibodies. [28] A study of adult systemic lupus erythematosus patients did not find any association between antiphospholipid antibodies and Raynaud phenomenon occurrence. [74]

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Imaging Studies

Nailfold capillaries can be visualized with digital video capillaroscopy, dermoscope, widefield microscope, or handheld ophthalmoscope. An abnormal pattern is more often present in patients with secondary Raynaud phenomenon than in patients with primary Raynaud phenomenon. [15] Nigrovic et al reported that 13% of pediatric patients with primary Raynaud phenomenon had an abnormal pattern compared with 54% of patients with secondary Raynaud phenomenon. [28]

If digital video capillaroscopy is available, then changes in the pattern can be followed because progressive worsening is associated with development of connective tissue disease. [49, 69]

Other imaging techniques include laser Doppler fluxometry (LDF), laser Doppler imaging (LDI), infrared thermography (IT), and Doppler ultrasound. LDF and LDI monitor superficial blood flow; scanning LDI has the advantage over LDF of allowing a large area to be evaluated without requiring surface contact. [75] IT measures surface temperature, which can serve as an indirect assessment of changes in blood flow in response to treatment or cold challenge. [75] A study comparing nailfold capillaroscopy (NFC), LDI, and IT evaluation of primary Raynaud phenomenon, secondary Raynaud phenomenon, and control subjects found all these techniques could differentiate patients with systemic sclerosis secondary Raynaud phenomenon from primary Raynaud phenomenon from control subjects after a cold challenge test. [76] NFC showed the highest level of discrimination, but each test provided additional information. [76]

Doppler ultrasound may be useful for identifying Raynaud phenomenon patients and differentiating primary from secondary Raynaud phenomenon. In one color Doppler study, the authors could distinguish acute vascular occlusion (eg, from antiphospholipid antibodies) from chronic occlusion as would be found in Raynaud phenomenon. Six percent of primary Raynaud phenomenon patients were found to have narrowing or occlusion of some proper digital arteries, compared with 63% of secondary Raynaud phenomenon patients; secondary Raynaud phenomenon patients also had more global involvement than primary Raynaud phenomenon patients. [77]

Another study reported different power Doppler patterns for primary Raynaud phenomenon and secondary Raynaud phenomenon, and that the power Doppler score was better than NFC at identifying secondary Raynaud phenomenon patients (86% vs 50%). [78]

In another study, secondary Raynaud phenomenon patients were found to have narrower radial and digital artery diameters, lower flow volumes at baseline and after cold provocation, and longer times for normalizing flow volumes in digital, ulnar, and radial arteries; these differences had moderate to high sensitivity and high specificity for differentiating primary Raynaud phenomenon from secondary Raynaud phenomenon. [79]

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Other Tests

Other testing methods for Raynaud phenomenon include finger systolic blood pressure measurements, Doppler ultrasonography, plethysmography, and cold challenge testing. [15, 75, 32, 9] None of these tests is needed for standard office assessment or monitoring of patients with Raynaud phenomenon.

Other tests, such as chest radiography, pulmonary function tests, ECG, echocardiography, high-resolution lung CT scanning, or GI evaluation, may be needed to evaluate a patient with potentially evolving secondary Raynaud phenomenon.

Procedures such as cold challenge testing are not needed in evaluating or monitoring most patients with Raynaud phenomenon.

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Histologic Findings

No permanent pathology is generally associated with primary Raynaud phenomenon except in rare severe causes. [75, 18]

Among patients with secondary Raynaud phenomenon, those with scleroderma have the most severe and most studied pathology. Arterioles and capillaries of 50-500 μm in diameter are affected; the number of capillaries decrease. [80] Vasculature shows endothelial cell swelling, fibrous intimal lesion formation with narrowing of the lumen, inflammatory cell infiltrates, and thickening of the basement membrane; intravascular thrombi may also be present, causing further vessel occlusion. [80, 20] Other changes seen in the skin include cytoskeletal alterations, junctional separation between cells and between cell and matrix, perivascular edema, platelet aggregation, and infiltration of T lymphocytes, mast cells, and dermal dendrocytes. [80]

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