Inflammatory Bowel Disease in Children With Systemic Juvenile Idiopathic Arthritis

Abstract

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 sJIA patients who were subsequently diagnosed with IBD (sJIA-IBD cohort). 522 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’s exact test, t-test, and univariate and multivariate logistic regression as appropriate. Results 75% of sJIA-IBD patients had a persistent sJIA course; 25% had a history of MAS. sJIAIBD 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. 69% of sJIA-IBD patients successfully discontinued sJIA medications following IBD diagnosis, and sJIA symptoms resolved in 9/12 patients treated with 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 sJIA patients and the likely broad benefit of TNF-α inhibition in those cases.

Increased Demand for Therapeutic Drugs in Pediatric Ulcerative Colitis Patients With Extraintestinal Manifestations

“…The patient who was treated with JRA developed intestinal symptoms after a year and was diagnosed with UC. Among the patients with intestinal manifestation in JRA, those with clearly IBD often have a family history of IBD and do not improve with typical disease-modifying antirheumatic drugs, but improve with biological agents used for IBD ( 23 ). The patient who had been treated for myositis developed intestinal symptoms 2 months later, and was diagnosed with UC.…”

Atypical Presentation of Systemic Arthritis in a Toddler with Down Syndrome

“…The initial concerns for this patient were infection, malignancy, and inflammatory bowel disease due to the presentation of anemia, arthritis, and rash, especially in the setting of DS, as they have increased risk of malignancy and infection; however, they do not have any increased risk for inflammatory bowel disease [ 3 ]. Additionally, inflammatory bowel disease, specifically Crohn's disease, has been shown to be associated with juvenile idiopathic arthritis [ 10 ]; however, inflammatory bowel disease is rare in those with sJIA, and when it does occur, it is more commonly seen in older children [ 11 ]. Children with Down syndrome are at increased risk to develop Down syndrome-associated arthritis (DA), but it usually presents with polyarticular (five or more joints with active arthritis), antinuclear antibody (ANA), and rheumatoid factor negative disease (RF), rather than systemic disease involving hepatosplenomegaly, serositis, and lymphadenopathy [ 4 ].…”

Gastrointestinal endoscopic findings of autoimmune and autoinflammatory diseases in pediatric rheumatology patients

“…However, studies on pediatric patients are limited. The relationship between the increase in inflammatory cytokines and inflammatory bowel disease in JIA has been investigated [ 18 , 19 ]. Maller et al [ 18 ] stated that inflammatory bowel disease is rarely seen at the beginning of JIA and that patients should be evaluated in terms of inflammatory bowel disease when gastrointestinal findings are observed.…”

“…The relationship between the increase in inflammatory cytokines and inflammatory bowel disease in JIA has been investigated [ 18 , 19 ]. Maller et al [ 18 ] stated that inflammatory bowel disease is rarely seen at the beginning of JIA and that patients should be evaluated in terms of inflammatory bowel disease when gastrointestinal findings are observed. Pichler et al [ 16 ] reported that abdominal pain and diarrhea were the most common endoscopy indications in children with JIA and mild non-specific inflammation in the colon was mostly seen histopathologically.…”