TY - JOUR
T1 - Inflammatory bowel disease in children with systemic juvenile idiopathic arthritis
AU - CARRA Legacy Registry Investigators
AU - Maller, Justine
AU - Fox, Emily
AU - Park, K. T.
AU - Paul, Sarah Sertial
AU - Baszis, Kevin
AU - Borocco, Charlotte
AU - Prahalad, Sampath
AU - Quartier, Pierre
AU - Reinhardt, Adam
AU - Schonenberg-Meinema, Dieneke
AU - Shipman-Duensing, Lauren
AU - Terreri, Maria Teresa
AU - Simard, Julia
AU - Lavi, Idit
AU - Chalom, Elizabeth
AU - Hsu, Joyce
AU - Zisman, Devy
AU - Mellins, Elizabeth D.
AU - Abramson, L.
AU - Anderson, E.
AU - Andrew, M.
AU - Battle, N.
AU - Becker, M.
AU - Benham, H.
AU - Beukelman, T.
AU - Birmingham, J.
AU - Blier, P.
AU - Brown, A.
AU - Brunner, H.
AU - Cabrera, A.
AU - Canter, D.
AU - Carlton, D.
AU - Caruso, B.
AU - Ceracchio, L.
AU - Chang, J.
AU - Charpentier, P.
AU - Clark, K.
AU - Dean, J.
AU - Dedeoglu, F.
AU - Feldman, B.
AU - Ferguson, P.
AU - Fox, M.
AU - Francis, K.
AU - Gervasini, M.
AU - Goldsmith, D.
AU - Gorton, G.
AU - Gottlieb, B.
AU - Graham, T.
AU - Syed, R.
AU - White, A.
N1 - Publisher Copyright:
© 2021 Journal of Rheumatology. All rights reserved.
PY - 2021/4/1
Y1 - 2021/4/1
N2 - Objective. The incidence of inflammatory bowel disease (IBD) in juvenile idiopathic arthritis (JIA) is higher than in the general pediatric population. However, reports of IBD in the systemic JIA (sJIA) subtype are limited. We sought to characterize sJIA patients diagnosed with IBD and to identify potential contributing risk factors. Methods. Using an internationally distributed survey, we identified 16 patients with sJIA who were subsequently diagnosed with IBD (sJIA-IBD cohort). Five hundred twenty-two sJIA patients without IBD were identified from the CARRA Legacy Registry and served as the sJIA-only cohort for comparison. Differences in demographic, clinical characteristics, and therapy were assessed using chi-square test, Fisher exact test, t-test, and univariate and multivariate logistic regression, as appropriate. Results. Of the patients with sJIA-IBD, 75% had a persistent sJIA course and 25% had a history of macrophage activation syndrome. sJIA-IBD subjects were older at sJIA diagnosis, more often non-White, had a higher rate of IBD family history, and were more frequently treated with etanercept or canakinumab compared to sJIA-only subjects. Sixty-nine percent of sJIA-IBD patients successfully discontinued sJIA medications following IBD diagnosis, and sJIA symptoms resolved in 9 of 12 patients treated with tumor necrosis factor-α (TNF-α) inhibitors. Conclusion. IBD in the setting of sJIA is a rare occurrence. The favorable response of sJIA symptoms to therapeutic TNF-α inhibition suggests that the sJIA-IBD cohort may represent a mechanistically distinct sJIA subgroup. Our study highlights the importance of maintaining a high level of suspicion for IBD when gastrointestinal involvement occurs in patients with sJIA and the likely broad benefit of TNF-α inhibition in those cases.
AB - Objective. The incidence of inflammatory bowel disease (IBD) in juvenile idiopathic arthritis (JIA) is higher than in the general pediatric population. However, reports of IBD in the systemic JIA (sJIA) subtype are limited. We sought to characterize sJIA patients diagnosed with IBD and to identify potential contributing risk factors. Methods. Using an internationally distributed survey, we identified 16 patients with sJIA who were subsequently diagnosed with IBD (sJIA-IBD cohort). Five hundred twenty-two sJIA patients without IBD were identified from the CARRA Legacy Registry and served as the sJIA-only cohort for comparison. Differences in demographic, clinical characteristics, and therapy were assessed using chi-square test, Fisher exact test, t-test, and univariate and multivariate logistic regression, as appropriate. Results. Of the patients with sJIA-IBD, 75% had a persistent sJIA course and 25% had a history of macrophage activation syndrome. sJIA-IBD subjects were older at sJIA diagnosis, more often non-White, had a higher rate of IBD family history, and were more frequently treated with etanercept or canakinumab compared to sJIA-only subjects. Sixty-nine percent of sJIA-IBD patients successfully discontinued sJIA medications following IBD diagnosis, and sJIA symptoms resolved in 9 of 12 patients treated with tumor necrosis factor-α (TNF-α) inhibitors. Conclusion. IBD in the setting of sJIA is a rare occurrence. The favorable response of sJIA symptoms to therapeutic TNF-α inhibition suggests that the sJIA-IBD cohort may represent a mechanistically distinct sJIA subgroup. Our study highlights the importance of maintaining a high level of suspicion for IBD when gastrointestinal involvement occurs in patients with sJIA and the likely broad benefit of TNF-α inhibition in those cases.
KW - Autoinflammation
KW - Cytokine inhibitors
KW - Inflammatory bowel disease
KW - Pediatric rheumatology
KW - Systemic juvenile idiopathic arthritis
UR - http://www.scopus.com/inward/record.url?scp=85104818803&partnerID=8YFLogxK
U2 - 10.3899/JRHEUM.200230
DO - 10.3899/JRHEUM.200230
M3 - Article
C2 - 32541073
AN - SCOPUS:85104818803
SN - 0315-162X
VL - 48
SP - 567
EP - 574
JO - Journal of Rheumatology
JF - Journal of Rheumatology
IS - 4
ER -