Background/Purpose:

Juvenile Localised Scleroderma (jlSc) is believed a rare autoimmune disease, which occurs 10 times more often then systemic sclerosis in childhood and is believed to have a prevalence of one per 100 000 children(1). There is no prevalence data published.

Methods:

We used Truven MarketScan® commercial insurance claims data from the United States for the years 2010 through 2014, inclusive. MarketScan contains billing records associated with physician office visits, outpatient infusions, and pharmacy claims, among other data, and is intended to be representative of all persons covered by employer-sponsored health insurance in the United States. In each individual calendar year, we identified all persons in the claims data who were less than 16 years old.

Results:

The results for each calendar year are shown in the Table. There were approximately 1600-2200 children per year with diagnoses of localized scleroderma and <3% of them had concurrent diagnoses of systemic sclerosis or mixed connective tissue disease. The prevalence estimates in each year ranged from 3.2 to 3.6 per 10,000 children. The proportion of children with localized scleroderma who received methotrexate in each year ranged from 3.2% to 4.4%.

Conclusion:

This prevalence data showes a higher than expected prevalence compared to the published incidence data with with 2.7 per 100 000 to 2.5 children per million per year. We need more prevalence data from other resources to reassure our findings.

Table

The estimated prevalence of juvenile localised Scleroderma in the United States

References

1. Laxer RM, Zulian F. Localized scleroderma. Curr Opin Rheumatol. 2006;18(6):606-13.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/assessing-the-prevalence-of-localized-scleroderma-in-childhood-using-dministrative-claims-data-from-the-united-states/