Immunoglobulin G4-associated sclerosing cholangitis in a patient with Crohn’s disease

Summary

The authors present the case of a 56-year-old patient treated for Crohn’s disease and primary sclerosing cholangitis (PSC). Histologic examination of a liver biopsy, which was performed to evaluate the advancement of liver disease, alerted us to the fact that the patient might have immunoglobulin G4-associated sclerosing cholangitis (IASC). We did not initially consider this disease because the patient lacked some of its characteristic features. He had not suffered from autoimmune pancreatitis and the development of liver disease was slow, with only a slow increase in serum concentrations of cholestatic enzymes. The onset of IASC is usually sudden with obstructive icterus. IASC usually presents in older patients, and its isolated occurrence without autoimmune pancreatitis is described in only 2% of patients. The coincidence of inflammatory bowel disease is also unusual for IASC. A high serum concentration of IgG4 is not sufficient to establish a diagnosis because it can be observed in patients with cholangiocellular cancer or PSC. IASC diagnosis is based on a combination of histological, imaging, biochemical, and clinical findings. However, there are no universally accepted diagnostic criteria. Crucial in our case was good clinical and laboratory response to the administration of corticosteroids, which is one of the essential features of imunoglobulin G4 associated diseases.

Key words:
immunoglobulin G – sclerosing cholangitis – Crohn’s disease

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE „uniform requirements“ for biomedical papers.

Submitted:
22. 6. 2016

 Accepted:
9. 8. 2016