Nebiyou Wondimagegnehu, MD1, Agazi Gebreselassie, MD, MSc1, Jacqulani Laurin, MD2; 1Howard University Hospital, Washington, DC; 2Johns Hopkins Sibley Memorial Hospital, Washington, DC
Introduction: IgG4-related sclerosing cholangitis (IgG4-SC) is commonly seen in patient with prior history of autoimmune pancreatitis in 80% of cases. Presence of clinical, lab and imaging findings consistent with sclerosing cholangitis in patients with IBD(inflammatory bowel disease) and no prior history of autoimmune pancreatitis (AIP) could be misleading to a diagnosis of primary sclerosing cholangitis (PSC). This has a significant bearing in terms of treatment options and prognosis.
Methods: A 53-year-old Caucasian male with a medical history of Crohn’s disease who presented to hepatology clinic due to elevated liver associated enzymes (LAES). He was diagnosed with Crohn's Disease approximately 2 years ago, after being symptomatic for several years with diarrhea and was on mesalamine. He was noted to have elevated LAES. He had normal LAES few months prior to presentation. Laboratory findings - Alkaline phosphatase 549 U/L, AST 79 U/L, ALT 54 U/L, total bilirubin 1.1 mg/dL, and elevated liver alkaline phosphatase isoenzyme 84. Liver associated work up includes, negative viral hepatitis markers, negative ANA, negative AMA, negative Antismooth muscle Ab, normal alpha-1-antitrypsin level, normal ferritin, and normal ceruloplasmin.
Imaging with MRI of the abdomen and MRCP with and without contrast showed intrahepatic biliary duct have an irregular beaded appearance with alternating areas of short segment strictures and dilatation which is consistent with sclerosing cholangitis. IgG4 level was significantly elevated- 250mg/dl. Patient was treated with prednisone at a starting dose of 40 mg daily and LAES normalized. Tapering steroid caused flare of LAES. Subsequently, he was able to maintain normal LAES with initiation of immunosuppressive medication and dose of steroid tapered to lowest dose. Discussion: IgG4-SC and PSC show similar imaging of the bile ducts, although their disease concept, pathophysiology, therapy, and prognosis are different; therefore, it is important to distinguish these two diseases. Steroid trials should be carried out only by experts familiar with AIP and IgG4-SC or in patients with diagnostically challenging cases of IgG4-SC without AIP or other organ involvement only after exclusion of cholangiocarcinoma.
Disclosures: Nebiyou Wondimagegnehu indicated no relevant financial relationships. Agazi Gebreselassie indicated no relevant financial relationships. Jacqulani Laurin indicated no relevant financial relationships.