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Abstract Number: 2294

The Presentation and Initial Treatment of Systemic Juvenile Idiopathic Arthritis According to Observational Data from the United States and the United Kingdom

Timothy Beukelman1, Roberto Carrasco2, Yukiko Kimura3, Laura Schanberg4, Wendy Thomson5, Kimme L. Hyrich6, For the CARRA Registry Investigators7 and For the CAPS Investigators Group8, 1Pediatric Rheumatology, University of Alabama at Birmingham, Birmingham, AL, 2Centre for Musculoskeletal Research, Institute of Inflammation and Repair, The University of Manchester, Manchester, United Kingdom, 3Pediatric Rheumatology, Hackensack Univ Medical Ctr, Hackensack, NJ, 4Duke University, Durham, NC, 5Institute of Inflammation and Repair, The University of Manchester, Manchester, United Kingdom, 6Centre for Musculoskeletal Research, University of Manchester, Manchester, United Kingdom, 7CARRA, Durham, NC, 8CAPS, Manchester, United Kingdom

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: juvenile idiopathic arthritis (JIA)

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Session Information

Session Title: Pediatric Rheumatology - Clinical and Therapeutic Aspects: Systemic Juvenile Idiopathic Arthritis, Spondyloarthropathy and Miscellaneous Pediatric Rheumatic Diseases

Session Type: Abstract Submissions (ACR)

Background/Purpose: Systemic JIA (sJIA) treatment has changed dramatically with the introduction of biologic agents, although treatment approaches may differ between countries. We characterized and compared presenting features and initial treatment of sJIA at pediatric rheumatology (PR) centers in the United States (US) and United Kingdom (UK).

Methods: The US data source was the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry, an observational registry established in 2010 that collects clinical data on children with incident or prevalent rheumatic disease from >55 clinical sites throughout the US. Children with sJIA who were enrolled in the Registry within 9 weeks of their first encounter with PR were included in the analysis of presenting features and children with clinical data available from 9 to 15 months following their first encounter with PR were included in the analysis of initial treatment. The UK data source was the Childhood Arthritis Prospective Study (CAPS), an observational study established in 2001 that collects clinical data at regular intervals from a cohort of children with newly diagnosed JIA from 7 UK PR centers. All children with sJIA were included in the analysis of presenting features and children with clinical data available from the 12 month visit were included in the analysis of initial treatment. We also evaluated a subset of CAPS children enrolled since 2009 to match the CARRA enrollment period. Presenting features and initial treatments were compared using chi-square, Fisher’s exact, and Wilcoxon ranksum tests.

Results: Presenting features are shown in Table 1 and medication use in the first 12 months is shown in Table 2. Disease manifestations were similar between the US and UK except hepatosplenomegaly was more frequent in the US (p=0.01). Elapsed time from first symptom to evaluation by PR was similar but slightly shorter in the US (p=0.01), and overall most children were evaluated within 2 months. Differences in enrollment procedures precluded valid comparisons of disease activity measures. US patients were more likely to receive biologic agents (specifically anakinra) and systemic glucocorticoids in the first 12 months of disease compared to UK patients.

Conclusion: Presenting features of children with sJIA were generally similar in the two countries. Compared to the UK, initial treatment of sJIA in the US more frequently included anakinra and, to a lesser extent, systemic glucocorticoids, which may represent differences in medication coverage.

Table 1. Presenting Features.

 

Characteristic

CARRA Registry (US)

(N = 64)

CAPS (UK)

(N = 90)

CAPS (UK) enrolled since 2009

(N = 21)

Age at first encounter with pediatric rheumatologist (years) (median, IQR)

5.8 (3.2-10.7)

6.7 (3.5-10.6)

7.8 (3.6-10.6)

Female (n, %)

43 (67%)

58 (64%)

12 (57%)

Caucasian race (n, %)

53 (83%)

76 (88%)

17 (90%)

Elapsed time from symptom onset to first encounter with pediatric rheumatologist (months) (median, IQR)

1.0 (0.5-2.4)

1.5 (0.75-3.5)

1.8 (1.2-3.5)

Active joint count (median, IQR)

3 (1-7)

3 (1-6)

5 (2-8)

Physician global assessment (median, IQR)

4 (2-6)

5.6 (3.6-7.2)

6.1 (5.2-6.9)

Parent global assessment (median, IQR)

5 (2-7)

3.4 (0.7-6.2)

5.5 (3.4-8.5)

Pain score

5 (2-7)

4.8 (1.1-7.1)

7.2 (3.8-8.0)

CHAQ score

0.6875

(0.25-1.75)

1.25

(0.375- 2.06)

1.43

(0.625-2.125)

Evanescent rash (n, %)

52 (81%)

75 (83%)

16 (76%)

Lymphadenopathy (n, %)

20 (31%)

26 (29%)

7 (33%)

Hepatosplenomegaly (n, %)

15 (23%)

8 (9%)

3 (14%)

Serositis (n, %)

6 (9%)

3 (3%)

0 (0%)

Table 2. Initial Medication Use in the First 12 Months of Disease

 

Medication

 

CARRA Registry (US) (N=75)

CAPS (UK)

(N=74)

CAPS (UK) enrolled since 2009

(N=21)

P value for comparison between CAPS enrolled since 2009 and CARRA

Systemic Glucocorticoid

60 (80%)

62 (84%)

12 (57%)

0.03

Methotrexate

46 (61%)

65 (88%)

16 (76%)

ns

Cyclosporine

6 (8%)

7 (10%)

1 (5%)

ns

Any Biologic

46 (61%)

16 (22%)

6 (29%)

0.008

Any IL-1 Inhibitor

33 (44%)

3 (4%)

1 (5%)

0.0006

     Anakinra

28 (37%)

3 (4%)

1 (5%)

0.003

     Canakinumab

1 (1%)

0 (0%)

0 (0%)

ns

     Rilonacept

4 (5%)

0 (0%)

0 (0%)

ns

Tocilizumab

9 (12%)

6 (8%)

5 (24%)

ns

Any TNF Inhibitor

12 (16%)

11 (15%)

1 (5%)

ns

 


Disclosure:

T. Beukelman,

Novartis Pharmaceutical Corporation,

5,

Genentech and Biogen IDEC Inc.,

5,

UCB,

5,

Pfizer Inc,

2;

R. Carrasco,
None;

Y. Kimura,

Novartis Pharmaceutical Corporation,

2,

Novartis Pharmaceutical Corporation,

5;

L. Schanberg,

Novartis Pharmaceutical Corporation,

2,

UCB Pharma,

5,

Eli Lilly and Company,

5;

W. Thomson,
None;

K. L. Hyrich,

Pfizer Inc,

9,

Abbott Immunology Pharmaceuticals,

9;

F. the CARRA Registry Investigators,
None;

F. the CAPS Investigators Group,
None.

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