Development of Crohn’s Disease during the Course of HLA B27+ Juvenile Idiopathic Arthritis

Authors: M. Huml ;  J. Sýkora ;  V. Lád ;  K. Siala ;  R. Pomahačová ;  R. Vondráková 1
Authors‘ workplace: Dětská klinika UK LF a FN, Plzeň přednosta doc. MUDr. J. Kobr, PhD. ;  Radiodiagnostická klinika FN, Plzeň přednosta doc. MUDr. B. Kreuzberg, CSc. 1
Published in: Čes-slov Pediat 2009; 64 (1): 22-27.
Category: Case Report


Authors regard an extraordinary case of a 15-year-old boy with longstanding juvenile idiopathic arthritis (JIA), who developed fistulizing Crohn’s disease (CD).

This patient had been treated for rheumatic factor negative JIA for 4 years. Despite intensive therapy (low-dose methotrexate, corticosteroids), with each successive joint involvement his diagnosis was reclassified to enthesitis-related arthritis (ERA). ERA is considered to be a subgroup of JIA and formerly known as juvenile ankylosing spondylitis (JAS). Our patient had been operated on a perianal abscess, at that standpoint joint manifestations had deteriorated. Five months later, he was referred to our unit for draining perianal fistula since surgery. This child was diagnosed for perianal CD according to standard clinico-pathophysiological criteria. Fecal calprotectin as a non-invasive marker of gastrointestinal inflammation was elevated regarding age (>150 µg/g). Pancreatic organ specific antibodies and anti-Saccharomyces cerevisiae antibodies (ASCA) were positive. Heterozygous 4G/5G genotype for the type 1 plasminogen activator inhibitor (PAI-1) was discovered. He was then given infliximab and immunosuppressive therapy with prompt reverse of fistula drainage and joint manifestation also rapidly resolved.

Authors discuss the published case and summarize the links between JIA a CD in the clinical and basic science literature. The pathophysiology of these two diseases is not fully understood and may relate to immune-mediated mechanisms. Out data may contribute to a better understanding of the pathophysiology underlying CD. Based on our findings, to our best knowledge, this is the first case demonstrating CD onset in JIA in childhood.

Key words:
Crohn’s disease, juvenile idiopathic arthritis, HLA B27


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Neonatology Paediatrics General practitioner for children and adolescents
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