Isolated ileitis associated with primary sclerosing cholangitis in three patients with Crohn’s disease

ABSTRACT

Background Primary sclerosing cholangitis (PSC) is associated with ulcerative colitis and extensive colonic involvement or ileocolitis in Crohn’s disease (CD). To our knowledge, no specific report of isolated ileitis associated with PSC in CD patients has been published in CD patients. Aim and methods We report three cases of patients with isolated Crohn’s ileitis associated with PSC and in whom colonic inflammation was never documented. Results Patients were followed up 10–23 years and each patient underwent 6–7 ileocolonoscopies: inflammation was located only in the terminal ileum, which was confirmed on surgical specimens in two patients. Small-duct PSC led to diagnosis of CD ileitis in one patient, while small and large-ducts PSC were evidenced after CD diagnosis in the other 2. PSC were regularly followed for 9–10 years. Conclusions Our three cases of PSC with isolated CD ileitis and long-term follow-up without any sign of colonic involvement argue against the concept that colonic mucosal inflammation is critical for the pathogenesis of PSC in inflammatory bowel disease.

KEYWORDS:

• Crohn’s disease
• ileitis
• primary sclerosing cholangitis

Disclosure statement

The authors declared no financial conflict of interest. The authors alone are responsible for the content and writing of this article.