A 60-Year-Old Woman with Primary Biliary Cholangitis and Crohn's Ileitis Following the Suspension of Ursodeoxycholic Acid

Abstract

BACKGROUND There is a recognized association between inflammatory bowel disease (IBD) and hepatobiliary autoimmune disease, particularly primary sclerosing cholangitis (PSC). There have been fewer reported cases of IBD and primary biliary cholangitis (PBC), which is treated with ursodeoxycholic acid (UDCA). This report presents the case of a 60-year-old woman with PBC who was diagnosed with Crohn's ileitis after suspension of UDCA treatment. CASE REPORT A 66-year-old female patient with PBC was admitted to our department for irrepressible chronic diarrhea and recurrent abdominal pain. PBC was diagnosed on the basis of serological data: chronic (>6 months) increase in alkaline phosphatase (ALP) associated with positivity for specific anti-nuclear antibodies (sp100 and gp210), without requiring a liver biopsy and a magnetic resonance cholangiopancreatography to rule out PSC. Given the intolerance and non-responsiveness according to the Toronto criteria (ALP <1.67 times the normal limit after 2 years) to UDCA at 15 mg/kg/day, an oral monotherapy treatment using obeticholic acid at 5 mg/day was prescribed. The patient complained of abdominal pain and upper gastrointestinal symptoms. The endoscopic/histologic and radiologic examinations supported the diagnosis of Crohn's ileitis. Given the potential benefits to PBC patients of what is described as off-label therapy, budesonide at a dosage of 9 mg/day p.o. was also administered. One month after discharge, an improvement was observed both in the cholestasis indices and in gastrointestinal symptoms. CONCLUSIONS This report presents a case of PBC in which the patient was diagnosed with Crohn's ileitis after cessation of treatment with UDCA, and highlights the importance of recognizing the association between autoimmune hepatobiliary disease and IBD.

Figure 1.

Timeline of clinical events. CD – Crohn’s disease; AMA – antimitochondrial autoantibodies; ANA – antinuclear autoantibodies; cACLD – compensated advanced chronic liver disease; GI – gastrointestinal; PBC – primary biliary cholangitis; GGT – gamma-glutamyl transferase; ALP – alkaline phosphatase; UDCA – ursodeoxycholic acid.

Figure 2.

Endoscopic view of the cecum and ileocecal valve (black arrows).

Figure 3.

Photomicrograph of biopsy showing the pathological features of Crohn’s disease. (A) Biopsy of the rectal mucosa site, showing inflammatory infiltrate rich in plasma cells and eosinophils, with the presence of non-necrotizing epithelioid granuloma (black arrow) (20× magnification). (B) Biopsy of the mucosa of the left colon characterized by distorted and mucin-depleted glands, with chronic inflammatory infiltrate, rich in plasma cells and eosinophils, with activity (20× magnification). (C) Biopsy of the mucosa of the right colon with evident glandular distortion and presence of cryptic abscesses (20× magnification). (D) Biopsy of the mucosa of the transverse colon with inflammatory sparing of the glandular elements (skip lesion) (20× magnification). (E) Ileal mucosa, with rare residual villi (black arrow), with intense chronic active inflammatory infiltrate and mucosal damage (10× magnification).