Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is an autoinflammatory disease accompanied by systemic symptoms including lymphadenopathy, serositis, and hepatosplenomegaly. Recently, we encountered a patient previously diagnosed with sJIA three years ago who developed hematochezia and weight loss with endocscopy findings consistent with indeterminate colitis, a form of inflammatory bowel disease (IBD). This association is scantly known with only one case report detected in the literature search.

Methods: We distributed a survey to all members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) and the Pediatric Rheumatology Bulletin Board asking physicians to report their cases of patients with both sJIA and IBD. Demographic, clinical, laboratory, and therapeutic information were collected on identified patients to gain further insight into this possible association. The data was analyzed, using descriptive statistics, specifically, means, median, and ranges for continuous variables and frequencies and proportions for categorical variables.

Results: Our survey identified 14 patients with diagnoses of sJIA and IBD. 13 of the patients were first diagnosed with sJIA. Five patients were from the United States, 7 from Europe, and 2 from South America. The average age at sJIA diagnosis was 9.9 years (range 6-14.5 years). Nine patients had a persistent sJIA course; 4 patients had a monocyclic course and 1 patient had a polycyclic course. Prior to their IBD diagnosis, the majority of patients were treated with corticosteroids (79%), NSAIDs (79%), and methotrexate (71%) for sJIA. Seven patients received TNF-α inhibitor (etanercept in 5/7). 7 patients received anti-IL-1 therapy, and 4 patients received an IL-6 inhibitor.  The average age of the patients at IBD diagnosis was 12.3 years (range 9-17 years).  During the 3 months prior to the IBD diagnosis, 6 patients were in remission, 6 were in an active flare, one had not yet received the sJIA diagnosis, and one answer was missing from the survey. Ten patients were diagnosed with Crohn’s disease and 4 with indeterminate colitis.  11/14 patients received a TNF-α inhibitor for IBD, with 10/11 demonstrating a full response to this class of medications. Other medications used in treatment include corticosteroids (57%), methotrexate (36%), azathioprine (43%), oral antibiotics (29%), and enteral formula therapy (36%). After both diagnoses were made in this cohort, 4 patients had quiescence of both sJIA and IBD diseases, 6 experienced an IBD flare with quiescent sJIA disease, 3 patients had a sJIA flare with quiet IBD, and one patient had a flare of both diseases. 

Conclusion: IBD morbidity is rare in patients with sJIA. It is most commonly manifest as Crohn’s disease with persistent sJIA.  Its appearance and flares do not typically correlate with sJIA activity, and the majority of these patients respond to monotherapy with a monoclonal TNF-α inhibitor. The therapeutic implications, however, are notable, given that many children with sJIA with systemic features do not respond to TNF inhibition. The biology underlying the association of these two diseases and their response to therapy is not clear and warrants further study.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/inflammatory-bowel-disease-in-children-with-systemic-juvenile-idiopathic-arthritis/