Background/Purpose:

Juvenile systemic Sclerosis (jSSc) is an orphan disease. There are some data regarding the incidence, but nearly no data exist regarding the prevalence of jSSc. To plan prospective trials, it is very important to gain data regarding the prevalence of jSSc.

Methods:

We used Truven MarketScan® commercial insurance claims data from the United States for the years 2010 through 2014, inclusive. In each individual calendar year, we identified all persons in the claims data who were less than 16 years old. Among these persons, we identified all persons with at least 1 physician diagnosis code for systemic sclerosis (ICD-9 710.1) and then removed any person with a diagnosis code for localized scleroderma (ICD-9 701.0). From this remaining group of patients, we identified patients with at least one filled prescription or infusion claim for immunosuppressant medications often used to treat systemic sclerosis, namely methotrexate, mycophenolate mofetil, or cyclophosphamide. We assumed these children had jSSc and calculated the estimated prevalence and 95% confidence interval using a Poisson distribution.

Results:

The results for each calendar year are shown in the Table. Very few children received diagnosis codes for systemic sclerosis. Approximately 70% of these patients did not have concurrent diagnoses of localized scleroderma, but only approximately 14% of those had treatment with immunosuppressant medications often used to treat systemic sclerosis. The prevalence estimates were approximately 3 per 1 million children and were relatively stable from year to year.

Conclusion:

Using diagnosis codes and medication usage from administrative claims, we estimated the prevalence of jSSc to be approximately 3 per 1 million children.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/assessing-the-prevalence-of-juvenile-systemic-sclerosis-in-childhood-using-administrative-claims-data-from-the-united-states/