The association of primary sclerosing cholangitis and celiac disease is unc
ommon. Herein, we report on 2 different cases which developed this associat
ion. Case 1 was a 59 year-old female who firstly complained of symptoms of
cholestasis. The diagnosis of primary cholangitis was made on liver biopsy,
and the endoscopic retrograde cholangiopancreatography (ERCP) showed narro
wing and irregularity of the extra- and intrahepatic bile ducts. The result
s were positive for antiendomysial antibodies and the jejunal biopsy confir
med the coexistence of celiac disease, which was asymptomatic until that mo
ment. The gluten-free diet ameliorated the index of cholestasis. Case 2 was
an old man suffering from undiagnosed celiac disease for at least 5 years
prior to admission at our Department. The diagnosis was based on the histol
ogical examination of a jejunal biopsy. The patient did not follow the glut
en-free diet and was again admitted to our Department 6 years later with sy
mptoms of cholestasis. The liver biopsy and ERCP confirmed the diagnosis of
primary sclerosing cholangitis.