P2936 - Small Duct Primary Sclerosing Cholangitis Confounded by Intrahepatic Cholestasis of Pregnancy

Dewan Giri, MBBS¹, Gres Karim, MD¹, Michelle Shah, DO¹, Neelesh Rastogi, MD², Tatyana Kushner, MD^3
1Mount Sinai Beth Israel Medical Center, New York, NY; ²Mount Sinai Morningside and Mount Sinai West/Mount Sinai Beth Israel Medical Center, New York, NY; ³Icahn School of Medicine at Mount Sinai, New York, NY

Introduction: Primary Sclerosing Cholangitis (PSC) is a cholestatic disorder of the liver characterised by inflammation and multiple strictures of the intra- and extrahepatic biliary tree. Wee and Ludwig in 1985 described patients with cholestatic biochemistry and histological features of PSC but with negative cholangiography and coined the term "small-duct PSC ''. We present a case of this entity, a rare variant of the PSC spectrum, confounded by coincidental intrahepatic cholestasis of pregnancy (ICP).

Case Description/Methods: A 30-year-old, G2P1, female at 23 weeks gestation was referred to Liver Clinic for the evaluation of abnormal LFTs. Her first pregnancy was complicated by ICP that required induction at 37 weeks gestation. She had a history of intermittently abnormal LFTs since childhood and had extensive work-up at outside facilities including an inconclusive liver biopsy. Physical examination showed a gravid uterus but otherwise was normal. Laboratory findings included an AST of 38 U/L, ALT of 68 U/L and Alkaline phosphatase of 234 U/L, Total Bile Acids 6.8 umol/L, T.Bili 0.2mg/dl. At 27 weeks of gestational age, she started developing pruritis with increasing alkaline phosphatase and bile acids and was diagnosed with ICP again and started on ursodeoxycholic acid. At 35 weeks, 6 days gestation, she visited the ED for decreased fetal movements and was noted to have total bile acids to 139 umol/L and worsening liver enzymes. She was induced and had a successful vaginal delivery. Post-partum, her symptoms had resolved, but LFTs continued to remain elevated. She underwent MRCP which showed mild prominence of intrahepatic biliary ducts only. A repeat liver biopsy was pursued, which revealed portal fibrosis with obliteration of portal veins, frequent bile duct loss without ductular reaction, confirming a diagnosis of small-duct PSC. She then underwent ileocolonoscopy which ruled out concurrent IBD.

Discussion: Most cases of PSC are diagnosed as part of an evaluation of elevated liver enzymes. Diagnosis is usually made in appropriate settings with radiographic findings on cholangiogram; however, a liver biopsy is required for patients suspected to have small duct PSC. A high degree of suspicion and careful history is sometimes required to make this diagnosis, as was the case for our patient who had elevated liver enzymes preceding her pregnancy and post-partum. There are currently no approved treatments for PSC.


Disclosures:


Dewan Giri, MBBS¹, Gres Karim, MD¹, Michelle Shah, DO¹, Neelesh Rastogi, MD², Tatyana Kushner, MD³. P2936 - Small Duct Primary Sclerosing Cholangitis Confounded by Intrahepatic Cholestasis of Pregnancy, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.