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

Juvenile Scleroderma Update
Written by Dr. Thomas J.A. Lehman
Pediatric Rheumatologist
Hospital for Special Surgery, New York, NY

For the first time the 2004 annual meeting of the American College of Rheumatology featured a full 90 minute clinical symposium on childhood scleroderma. Organized by the members of the Juvenile Scleroderma Working group this symposium features sessions on progressive systemic sclerosis, localized forms of scleroderma and new advances in describing the disease.

One of the key obstacles to improving the care of children with scleroderma has been a failure by doctors to clearly define which group of patients they are talking about. We all know every patient is different from other patients, but it’s also true that ‘not all scleroderma is the same.’ Children with localized scleroderma may have strikingly different degrees of involvement. Although it’s less obvious, children with PSS can have strikingly different involvement as well. When discussing problems or therapies doctors need to be sure they are talking about the same groups of children. (Imagine cooking pies with round green fruit with a firm white flesh, stems, and a seeded core. If we all had apples there would be no problem. But what is someone had pears? They fit the description, but the results don’t make sense. We need to make sure our descriptions are specific enough that we don’t make that mistake).

The 90 minute session at the ACR meeting concentrated on three areas. Thomas J. A. Lehman MD from New York gave a 30 minute presentation on the early recognition of children with PSS and the importance of promptly initiating therapy to prevent long term damage. There still isn’t complete agreement on the best therapy for these children. Methotrexate, cyclosporine, cyclophosphamide and newer agents which block endothelial cell signaling were all discussed.

Dr. Francesco Zulian from the University of Padua in Italy followed with an excellent presentation on the varied forms of localized scleroderma in childhood. This is a very confusing area. At the summer 2004 meeting in Padua 20 experts from around the world met to discuss proper characterization of these children so we could be sure we were all talking about the same thing. By the end of the first afternoon it was clear we were going to have a difficult time. Everyone recognized the typical forms of focal scleroderma (a tight band on the leg or arm, hard round spots on the body, or tight hard skin on the scalp), but everyone had examples of children who clearly had more than one type of involvement. Does that mean it’s all the same thing? We don’t know yet. Much more needs to be done.

The final half hour presentation was made by Dr. Nicolo Ruperto from the Gaslini hospital in Genoa Italy. Dr. Ruperto (in abstentia since his wife had just delivered a baby – he sent a dvd) reported on the discussions of criteria for the diagnosis of PSS which were held in Padua. Working with the group of 20 experts and 150 case histories of children with scleroderma and similar diseases Dr. Ruperto was able to define a set of criteria which could be used to define children with PSS with increased certainty. These criteria will be very important in allowing us to make sure everyone in a study has the same disease. However, they aren’t writing in stone. There were several children where even the 20 world experts couldn’t quite agree on the proper diagnosis.

What do these things mean to children with scleroderma and their families? They mean that a significant group of pediatric investigators has now been organized to bring rapid progress to the treatment and care of children with scleroderma. So far the Juvenile Scleroderma working group has only had two meetings, but we look forward to moving forward as quickly as possible to find better drugs and an improved outcome for children with scleroderma. Want to help? It all costs money. If you have ideas for helping to support the next big meeting let us know. So far the meetings have been in Italy because the Italian parents of children with scleroderma were willing to do the hard work. They’ve done two. Hopefully we can find the resources in the US to support the next one.

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)

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