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Pediatric Raynaud Phenomenon Follow-up

  • Author: Suzanne C Li, MD, PhD; Chief Editor: Lawrence K Jung, MD  more...
 
Updated: Apr 22, 2014
 

Further Outpatient Care

See the list below:

  • 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.[129, 130] 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, 30]
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Further Inpatient Care

See the list below:

  • 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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Inpatient & Outpatient Medications

See the list below:

  • See Medication.
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Transfer

See the list below:

  • 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.

  • 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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Complications

See the list below:

  • Digital pits, digital ulcers, autoamputation or gangrene, infection, and poor wound healing are potential complications.
  • If the patient develops digital ischemia, they are at risk for further ischemia due to damage to the local circulation.
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Prognosis

See the list below:

  • The prognosis is good for patients with primary Raynaud phenomenon. An adult community study found 36% of primary Raynaud phenomenon patients had persistent disease, while 64% of primary Raynaud phenomenon patients had resolution of this problem within 7 years.[29]
  • The prognosis can be poor for patients with secondary Raynaud phenomenon whose underlying disease is a scleroderma-related condition or systemic lupus erythematosus.[9] Other connective tissue diseases may also be associated with a poor outcome.
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Patient Education

See the list below:

  • Patients should be taught to recognize potential triggers and to avoid them when possible.
  • Patients should be taught to warm-up whenever they feel altered sensation or notice prolonged pallor or cyanosis.
  • Patients should be taught good digital hygiene and the importance of thoroughly treating peripheral injuries and wounds.
  • Patients should be instructed about their medications and potential side effects.
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Contributor Information and Disclosures
Author

Suzanne C Li, MD, PhD Associate Professor, Department of Pediatrics, Rutgers New Jersey Medical School; Senior Attending, Department of Pediatrics, Joseph M Sanzari Children’s Hospital, Hackensack University Medical Center

Suzanne C Li, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American College of Rheumatology, Childhood Arthritis and Rheumatology Research Alliance

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David D Sherry, MD Chief, Rheumatology Section, Director, Amplified Musculoskeletal Pain Program, The Children's Hospital of Philadelphia; Professor of Pediatrics, University of Pennsylvania School of Medicine

David D Sherry, MD is a member of the following medical societies: American College of Rheumatology, American Pain Society

Disclosure: Nothing to disclose.

Chief Editor

Lawrence K Jung, MD Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

James M Oleske, MD, MPH François-Xavier Bagnoud Professor of Pediatrics, Director, Division of Pulmonary, Allergy, Immunology and Infectious Diseases, Department of Pediatrics, Rutgers New Jersey Medical School; Professor, Department of Quantitative Methods, Rutgers New Jersey Medical School

James M Oleske, MD, MPH is a member of the following medical societies: Academy of Medicine of New Jersey, American Academy of Allergy Asthma and Immunology, American Academy of Hospice and Palliative Medicine, American Association of Public Health Physicians, American College of Preventive Medicine, American Pain Society, Infectious Diseases Society of America, Infectious Diseases Society of New Jersey, Medical Society of New Jersey, Pediatric Infectious Diseases Society, Arab Board of Family Medicine, American Academy of Pain Management, National Association of Pediatric Nurse Practitioners, Association of Clinical Researchers and Educators, American Academy of HIV Medicine, American Thoracic Society, American Academy of Pediatrics, American Public Health Association, American Society for Microbiology, Infectious Diseases Society of America, Pediatric Infectious Diseases Society

Disclosure: Nothing to disclose.

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Raynaud phenomenon showing demarcation of color difference.
Nine-year-old with Raynaud phenomenon. Notice discoloration of fingers.
 
 
 
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