Clinical features of IgG4-associated sclerosing cholangitis: An analysis of 12 cases

Objective To investigate the clinical features, steroid response, and recurrence of Ig G4-associated sclerosing cholangitis (Ig G4-SC) , and to shorten the time for clinical diagnosis and reduce recurrence rate. Methods A total of 12 patients with Ig G4-SC who were admitted to Peking University First Hospital from December 2011 to March 2017 were enrolled, and they all met the clinical diagnostic criteria for Ig G4-SC in 2012. A retrospective analysis was performed for their clinical data, clinical features, and follow-up data. Results Of all 12 patients, the male/female ratio was 11∶ 1. The mean age of onset was 61. 7 ± 8. 6 years, and the mean time to a confirmed diagnosis was 24. 2 months. Of all 12 patients, 11 (92%) had jaundice, with a mean serum total bilirubin level of 198. 7 ± 57. 7 μmol/L. There were varying degrees of increase in serum alanine aminotransferase level (range 55-453 U/L) . Of all patients, 5 (41. 7%) had acute abdominal pain, with a mean CA19-9 level of 60. 8 ± 12. 5 U/ml. Serum Ig G4 was increased by 1. 5-18. 9 times, with a mean level of 7. 7 ± 2. 0 g/L. Magnetic resonance cholangiopancreatography (MRCP) performed for all patients showed typical manifestations including bile duct wall thickening, lumen stenosis, and intrahepatic and extrahepatic bile duct dilatation; 11 patients (92%) were found to have autoimmune pancreatitis, and 2 (16. 7%) had involvement of more than 3 visceral organs. In two patients who underwent biliary stent implantation before treatment, the biliary stent was removed after 4-6 weeks of steroid treatment. The mean duration of steroid response was 5. 4 months. Among the 8 patients who underwent steroid treatment, 4 (50%, 4/8) experienced recurrence during the reduction of the dose of steroid, among whom 2 experienced recurrence within 8-12 weeks after drug withdrawal. Conclusion Ig G4-SC needs a long time for prognosis, with frequent misdiagnosis and missed diagnosis. Serum Ig G4 level should be measured for elderly male patients with an acute onset of jaundice and bile duct stenosis, bile duct wall thickening, and involvement of the pancreas on MRCP. Steroid treatment should be given as early as possible after diagnosis. Patients may need at least 5-6 months to achieve steroid response. The dose of steroid should be reduced slowly and recurrence and tumorigenesis should be closely monitored.