C0073 - A Unique Case of Small Duct Primary Sclerosing Cholangitis Leading to Recurrent Cholestatic Hepatitis

Yoshio Wagner, BA¹, Ahmed Elbanna, DO², Brian Ginnebaugh, MD³, Syed-Mohammed Jafri, MD^2
1Wayne State University, Detroit, MI; ²Henry Ford Health System, Detroit, MI; ³Wayne State University, Detroit, MN

Introduction: Small Duct Primary Sclerosing Cholangitis (sdPSC) is the narrowing of intrahepatic bile ducts which often leads to cholestasis and cirrhosis. We present a patient diagnosed with sdPSC which is an uncommon form of PSC.

Case Description/Methods: A 42-year-old male patient, with a history of hypertension and gunshot wound with bowel resection presented for abdominal pain associated with recurrent cholangitis. Previous history also included bouts of abdominal pain, jaundice and hyperbilirubinemia that occurred roughly three times a year. A liver biopsy was taken during admission and revealed mild portal inflammation and cholestasis, bridging fibrosis, and intracytoplasmic iron granules. One month later a colonoscopy showed patchy erythema with biopsies demonstrating mild acute proctitis. Given cholestatic pattern, + ANA 1:320, recurrent admissions with sepsis, and recent colonoscopy showing evidence of proctitis (biopsy proven), the patient was diagnosed with sdPSC with overlap of autoimmune cholangiopathy. The sdPSC was treated with a cadaveric liver transplant, and upon explant, the patient’s liver showed chronic obstructive cholangiopathy. His postoperative course was notable for persistently elevated bilirubin of which half was indirect. Upon ERCP, the liver showed a mild anastomotic stricture and tortuous duct which was treated with a stent. However, the stent was removed because of lack of frank stricture. The patient was started on a prednisone taper with tacrolimus. The patient is currently doing well post-transplant on low dose tacrolimus and mycophenolate.

Discussion: Clinical presentation of sdPSC often consists of pruritis, jaundice, and acute cholangitis from cholestasis, and fatigue, liver failure and hepatosplenomegaly from liver cirrhosis. Notably, the patient presented with hepatomegaly, and jaundice. With this presentation, PSC, sdPSC, and benign recurrent intrahepatic cholestasis (BRIC) were all possible diagnoses. Unremarkable MRCP imaging highlighted the unlikeliness of PSC, and liver biopsy showed bridging fibrosis which excluded BRIC. Clinical diagnosis of sdPSC is difficult and a high degree of clinical suspicion is needed. The cause for Small Duct Primary Sclerosing Cholangitis is unknown, but there is an association with irritable bowel disease, presence of HLA-B8 and HLA-DR3 and autoimmune disease like hyper IgM.


Disclosures:


Yoshio Wagner, BA¹, Ahmed Elbanna, DO², Brian Ginnebaugh, MD³, Syed-Mohammed Jafri, MD². C0073 - A Unique Case of Small Duct Primary Sclerosing Cholangitis Leading to Recurrent Cholestatic Hepatitis, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.