Pediatric Raynaud PhenomenonRaynaud Phenomenon (RP) refers to a transient vasospasm of peripheral arteries and arterioles that classically results in triphasic color changes in the affected region. The initial artery and arteriole vasospasm causes pallor (white), followed by cyanosis (blue) due to dilation of the capillaries and venous stasis (deoxygenated blood), with continued artery and arteriole vasospasm. The arteries and arterioles then dilate, causing rapid return of blood flow (red, reactive hyperemia). The fingers are the most commonly affected region, and Raynaud phenomenon is typically triggered by cold exposure or stress.

Raynaud phenomenon can be primary (idiopathic), meaning no associated diseases are present, or secondary, meaning that another condition is believed to be the cause of the Raynaud phenomenon. Connective tissue diseases are the most common cause of secondary Raynaud phenomenon, but several medications and many other conditions are also associated with Raynaud phenomenon.

Pathophysiology

The pathophysiology of Raynaud phenomenon is not completely understood; the vasospasm that occurs in the digital arteries, precapillary arterioles, and cutaneous arteriovenous shunts involves both central and peripheral mechanisms. The pathophysiology of secondary Raynaud phenomenon also involves structural changes to the vasculature that worsens the severity of the Raynaud phenomenon. Raynaud phenomenon can be thought of as an exaggerated normal response to cold and emotional stress.

Clinical Presentation

History form the patient

Patient history should include affected sites, frequency and severity of attacks, duration of attacks, color pattern, triggers, seasonality, and associated symptoms (ie, numbness, paresthesia, pain). The most commonly affected sites are the fingers, toes, ears, nose, and, rarely, nipples.The triggers of Raynaud phenomenon in children are similar to those described in adults. Cold and emotional stresses are the most common triggers; primary Raynaud phenomenon can also be triggered by exercise.Episodes are more common in the winter than in the summer, and serious ischemia is also more common in the winter.

Patients and their parents should be queried about changes in digits such as pits, ulcers, or poor healing, and about the presence of infection in affected digits. They should also be asked about possible associated or precipitating factors including frostbite, drug or toxin exposure, infection, vibration injury, family history of Raynaud phenomenon, family history of connective tissue diseases, history of migraine,weight loss or eating disorders, and cardiovascular diseases.

Patients should also be questioned for any history suggestive of connective tissue disease such as fever, fatigue, rash (malar, vasculitic, dermatomyositis), morning stiffness, arthralgia, myalgia, dysphagia, peripheral edema, lymphadenopathy, or oral sores.

Physical examination

A general physical examination should be performed to evaluate for signs suggestive of rheumatic disease or other conditions associated with secondary Raynaud phenomenon.

Pediatric patients with primary Raynaud phenomenon generally have normal nailfold capillary findings.

Digit tips should be examined for pits, ulcers, and healing problems. Other signs potentially associated with rheumatic diseases include livedo reticularis, rash (malar, vasculitic, dermatomyositis), arthritis, skin edema or tightening, abnormal peripheral pulses, weakness, and oral ulcers.

Diagnosis and Workup

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. Most pediatric patients with secondary Raynaud phenomenon have a +ANA (85-100%). However, a significant number of pediatric patients with primary Raynaud phenomenon also have a +ANA.

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

Management

  • Patients with Raynaud phenomenon (RP) should avoid situations and stressors that aggravate the disease (eg, vibration, cold exposure, digital trauma).
  • Patients should avoid medications and drugs that aggravate the disease .
    • A pediatric rheumatologist should be consulted to evaluate patients suspected of having or developing secondary Raynaud phenomenon.
    • Other subspecialists, such as a hematologist, oncologist, or endocrinologist, should be consulted if the underlying condition for Raynaud phenomenon falls in their domain.
    • Consultation with a surgeon may be needed for patients with threatened digit ischemia.

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