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Abstract Number: 1282

Assessing the Prevalence of Juvenile Systemic Sclerosis in Childhood Using Administrative Claims Data from the United States

Ivan Foeldvari1, Timothy Beukelman2 and Fenglong Xie3, 1Hamburg Center for Pediatric and Adolescent Rheumatology, Hamburg, Germany, 2Pediatric Rheumatology, University of Alabama at Birmingham, Birmingham, AL, 3Division of Clinical Immunology & Rheumatology, University of Alabama at Birmingham, Birmingham, AL

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: Juvenile scleroderma and pediatric rheumatology

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Session Information

Date: Monday, November 6, 2017

Title: Pediatric Rheumatology – Clinical and Therapeutic Aspects Poster II: Lupus and Related Disorders, Myositis, Scleroderma and Vasculitis

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

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.

Year

N of Total Children

Diagnosis Code for Systemic Sclerosis

No Diagnosis Code for Localized Scleroderma

Use of Methotrexate, Mycophenolate Mofetil, or Cyclophosphamide

Estimated Prevalence per 1,000,000 Children [95% CI]

2010

5,888,868

254

186

23

3.9 [2.5-5.9]

2011

6,231,475

249

185

22

3.5 [2.2-5.3]

2012

6,278,116

217

170

26

4.1 [2.7-6.1]

2013

4,950,018

175

120

17

3.4 [2.0-5.5]

2014

4,933,522

138

91

14

2.8 [1.6-4.8]

Conclusion:

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


Disclosure: I. Foeldvari, None; T. Beukelman, None; F. Xie, None.

To cite this abstract in AMA style:

Foeldvari I, Beukelman T, Xie F. Assessing the Prevalence of Juvenile Systemic Sclerosis in Childhood Using Administrative Claims Data from the United States [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/assessing-the-prevalence-of-juvenile-systemic-sclerosis-in-childhood-using-administrative-claims-data-from-the-united-states/. Accessed .
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