Extraintestinal Manifestations of Inflammatory Bowel Disease

Abstract

Extraintestinal manifestations (EIMs) of inflammatory bowel disease (IBD) have been reported to involve almost every organ system. This article focuses on the prevalence (where known) and clinical aspects of the more commonly described EIMs of Crohn disease (CD) and ulcerative colitis (UC). Because of limited data from pediatric populations, much of the information in this review is from adult reports. An important consideration is whether EIMs represent manifestations of the same pathogenetic mechanisms or distinctly different disease processes from the underlying IBD, possibly coincidental to the presence of IBD or as a consequence of the IBD or its treatment. EIMs may present before the onset of gastrointestinal symptoms, during the natural course of disease, and rarely may even follow colectomy in patients with UC. The prevalence of at least 1 EIM in recent population-based studies of patients with IBD ranges from 6% to 47% (1–4). Several reports from pediatric centers suggest a greater prevalence in younger patients. Grossman and DeBenedetti (5) reported that 68% of 41 children with IBD had EIMs, and a recent review of pediatric patients by Stawarski et al (6) reported that 50% with UC and 80% with CD had at least 1 EIM. In this review, we provide an overview of EIMs that have been reported to be associated with UC and CD, emphasizing the pediatric experience when such information is available. EIMs are subdivided into several categories: colitis related, specifically skin, eye, joint, and mouth, where the activity of the EIM parallels the activity of the underlying intestinal disease; hepatobiliary; impaired growth; EIMs secondary to complications of or as direct extensions of bowel disease, more frequently noted in patients with CD than with UC and including nephrolithiasis, obstructive uropathy, cholelithiasis, and pancreatitis; and EIMs that cannot be categorized clearly in one of the other groups, such as amyloidosis and cancer (7). EIMs involving vascular, hematological, pulmonary, cardiac, and neurological systems probably belong to this last group. Impaired growth, unique to the pediatric age group, may be an EIM potentially related to all of these categories. Iatrogenic EIMs, such as drug-induced bone marrow suppression and corticosteroid-associated myopathy, are not discussed (Table 1). TABLE 1 Extraintestinal manifestation of inflammatory bowel disease