Pediatric Raynaud Phenomenon Follow-up

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

Further Outpatient Care

Patients with secondary Raynaud phenomenon (RP) should be monitored by a pediatric rheumatologist if their underlying disease is related to a connective tissue disease.

Patients suspected of having a condition that may potentially evolve to secondary Raynaud phenomenon require more careful monitoring than patients with primary Raynaud phenomenon.

In adults, 12-20% of patients whose condition was initially classified as primary Raynaud phenomenon evolved into secondary Raynaud phenomenon over 10 or more years. [126, 127] Nailfold capillary abnormalities, strongly positive ANA, elevated ESR, and additional autoantibodies such as ACA are associated with a 30% frequency of development of secondary Raynaud phenomenon in adult patients within 5 years. [9]

A 20-year prospective study of 586 consecutive patients with Raynaud phenomenon who had no definite rheumatic disease at presentation found 12.6% developed definite systemic sclerosis. A positive systemic sclerosis–specific autoantibody (anticentromere antibody, anti-topoisomerase I, anti-RNA polymerase III, or anti-TH/To) and nailfold changes of enlarged capillaries or capillary loss were independent predictors of progression to definite systemic sclerosis; 79.5% of patients with one of these autoantibodies and abnormal nailfold changes at baseline developed systemic sclerosis.

Another adult study of over 3000 primary Raynaud phenomenon patients found progression from primary to secondary Raynaud phenomenon was associated with older age of Raynaud phenomenon onset (>40 y) and worsening attacks (more frequent, last longer). [19] In children, a similar frequency of conversion has been reported (18-23.6%), but over a shorter period (mean, 1.3-2.4 y). [6, 49]

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Further Inpatient Care

Patients with Raynaud phenomenon (RP) who have severe digital ischemia, uncontrolled pain, or impending digital amputation should be hospitalized. [7, 5]

Patients who are hospitalized should receive aggressive vasodilator therapy; patients should have doses increased as tolerated while monitoring vital signs and symptoms.

Combination of different classes of vasodilators or other classes of medications may be needed to control symptoms (eg, calcium channel blocker and phosphodiesterase inhibitor).

Patients may need intravenous prostanoid treatment.

Sympathetic block or sympathectomy may be needed to improve blood flow. Sympathetic block may be repeated intermittently as needed.

Patients should be kept in a warm environment, with warm covering of affected area (eg, socks, warm blanket).

Infusion of heparin for 24-72 hours has been used for rapidly progressing ischemia, but no trials have evaluated this treatment.

Patients may need to be treated with antibiotics if infection of the ischemic area is suspected.

Surgical debridement of necrotic or infected tissue may be needed.

Patients should be evaluated for other potential problems, such as coagulopathy or worsening of underlying vasculitis.

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Transfer

Patients with secondary Raynaud phenomenon should be monitored by a pediatric rheumatologist if the Raynaud phenomenon is due to a connective tissue disease. Patients with secondary Raynaud phenomenon from other underlying problems should be referred to other specialists as appropriate.

Patients may need to be referred to a surgeon for debridement of necrotic or infected digits or for a sympathectomy if digital loss is a risk.

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Deterrence/Prevention

Patients should stay warm by avoiding cold situations or dressing adequately in cold situations. Appropriate garb for cold weather includes a hat, mittens, boots, and layered clothing on the trunk. Other recommendations include the following:

  • Advise patients to avoid or stop medications and drugs that can aggravate the Raynaud phenomenon.

  • Advise patients to avoid continued trauma to the affected region.

  • Patients who have very low body weight may have decreased symptoms with weight gain.

  • Patients should avoid prolonged ischemia by promptly warming up whenever they feel altered sensation in their digits.

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