A definition of Juvenile Scleroderma in simple, easy to understand language

Why Would A Child Have Raynaud's?
Written by Thomas J.A. Lehman, M.D.
Chief, Pediatric Rheumatologist
New York, NY

Everyone reading this news letter already knows that adults may get Raynaud’s, but how many of you realize that children may get it too? Fortunately Raynaud’s in childhood seems to be rare. (a complete review of the literature failed to produce a single estimate of its prevalence). In my large pediatric rheumatology practice I see fifteen to twenty new cases every year. Pediatric rheumatologists see many children with Raynaud’s, but there are probably many more who never see a doctor because their hands get cold or are dismissed by their doctors without further investigation because, “it happens sometimes.”

Many people think Raynaud’s only happens in the cold, but I’ve treated children who had Raynaud’s in 80 degree weather. It can happen with embarrassment, air conditioning, or any other event that alters blood flow. It’s not just fingers. Some children have toes that blanch. Some children will see the tip of their nose turn white, or even their ear lobes.

Raynaud’s in children occurs because the blood vessels in the fingers (or elsewhere) are clamping down in spasm. It’s the same thing that happens in adults. But children are supposed to have young and healthy blood vessels. They aren’t using jack hammers or working with chemicals that may cause Raynaud’s. Does that mean the children all have diseases like lupus or scleroderma? No fortunately not!! Many thin teenage girls have Raynaud’s, just like their mothers. How can you be sure?

There are a number of important steps to take if you have a child with Raynaud’s. First make sure that’s what is happening. Remember Raynaud’s is not simply cold hands. True Raynaud’s requires a three phase color change. The fingers have to blanch white, then turn red as they warm up, then get a bluish/purplish dusky color as the poor circulation returns. If the fingers don’t blanch white, it’s not Raynaud’s.

If a child definitely has Raynaud’s they should be evaluated by a rheumatologist, preferably a pediatric rheumatologist with experience in the area. Studies have been done on a number of these children and it appears to be possible to separate those we should from those we shouldn’t worry about. All children with Raynaud’s should be tested for antinuclear antibodies (ANA). Many with nothing wrong will test positive, but if there are no findings on exam it is unlikely that a child who tests negative for ANA has any underlying condition. However, a negative test once, is not a lifetime guarantee. If things change, get tested again. In addition, primary Raynaud’s is infrequent in boys. A boy with Raynaud’s always deserves extra attention.

If a child is ANA positive further evaluation should be done to make sure they have normal blood work including a normal blood count, a normal ‘chemistry panel,’ a normal sedimentation rate, and a normal urine. Many doctors will do all these tests when they draw the initial ANA. Once the physician knows the ANA is positive they should test for anti-centromere antibodies, anti-scl 70, and the subtypes of ANA. I also test for antiphospholipid antibodies, RF, and anti-thyroid antibodies. Any further testing will depend on the clinical picture. Some children with Raynaud’s will have a significant underlying connective tissue disease (secondary Raynaud’s). All of these children should be under the care of an experienced pediatric rheumatologist. So should all children with significant abnormal blood tests. Most parents’ children will have normal tests. Positive tests for ANA in low titer are very common. If the ANA is positive, but the physical exam is normal and there are no other laboratory findings, it’s still primary Raynaud’s.

So what should you do now? The most important thing for parents of children with primary Raynaud’s is common sense. Keep their hands warm. Make them wear gloves. You want to make sure they pay attention to their fingers, but you don’t want to make them, “different from everyone else.” Let them go out and play in the snow with the other kids. Just make them dress more warmly and come in to be checked on periodically. Don’t let them take a job at the supermarket where they have to work in the cold storage room or freezer cabinet. If a child with Raynaud’s has a finger that turns white and numb that doesn’t get better after an hour head for the doctor’s office or the local emergency room.

Even children with primary Raynaud’s can have real problems with cold finger tips. Children with significant problems can be treated with calcium channel blockers or other agents to relieve their symptoms. There aren’t established pediatric doses for these medications, so you need to find a physician with the experience to use them in children. However, most children with primary Raynaud’s don’t require medication. If a child has severe primary Raynaud’s or secondary Raynaud’s make sure they are under the care of an experienced rheumatologist.

For more information on Raynaud's please contact:
The Raynaud's Association, Inc.

94 Mercer Ave.
Hartsdale, NY 10530
800-280-8055 (914-946-5808 if calling within the NY area)
Email: info@raynauds.org

Please keep in mind, this webpage is for your information only.
Please check with your child's physician for any treatments.

For more information on Juvenile Scleroderma, contact:

Juvenile Scleroderma Network, Inc.
1204 W. 13th Street, San Pedro, CA 90731

Tel: (310)519-9511 (Pacific Time)
24 Hour Support Line: 1-866-338-5892 (toll-free)

Speak to another JSD parent for emotional and logistical support provided by home-based JSD volunteers. For medical advice, please contact your child's physician.

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