Correspondence to Author: Avani Sharma, Vani Malhotra, Parveen Malhotra, Ankit Goel, Harman Singh, Rahul Siwach, Pranav Malhotra, Anuj Sharma. 

Department of Medical Gastroenterology and Obstetrics & Gynecology, Pt. B.DS PGIMS, Rohtak, Haryana, & VMMC & Safdarjung Hospital, New Delhi, India

DOI: 10.52338/jjogastro.2026.5316

Abstract:

Introduction:Primary sclerosing cholangitis (PSC) is a chronic, progressive liver disease where bile ducts become inflamed, scarred, and narrowed, leading to bile buildup and liver damage. The exact cause is unknown, but it is strongly linked to inflammatory bowel disease (IBD) and is likely an autoimmune condition. Symptoms can include fatigue, itchy skin, and abdominal pain, and a liver transplant is often the only cure.
Case Report: We report a fourty year-old female, not a known case of any chronic illness and presented for first time with jaundice during second trimester of pregnancy and at that point of time was diagnosed to be having intrahepatic cholestasis of pregnancy. All her viral markers were negative. After delivery, jaundice did not completely subside and itching, though became less but persisted. She used to have intermittent exacerbation of jaundice which was associated with fever and chills. It used to subside after symptomatic treatment. These episodes remained for next few years but repeated ultrasonogram were reported to be normal. Later on, she developed pedal edema and at this point of time was diagnosed to be having changes of chronic liver disease and splenomegaly. She was put on standard treatment for chronic decompensated liver disease but despite that she progressed and developed persistent pancytopenia due to hypersplenism. She needed repeated blood transfusions for severe anemia and repeated injectable antibiotics for recurrent infection. Thus, on consultation with surgical team, she underwent splenectomy for hypersplenism. Her pancytopenia improved substantially and for the first time, ultrasonogram abdomen revealed dilated and irregular common bile duct. The MRCP done revealed multiple strictures and dilatation at level of intrahepatic ducts and dilated CBD with stricture at lower end, suggestive of primary sclerosing cholangitis. The liver biopsy also showed features of PSC. Her ursodeoxycholic acid has been increased to 25 mg/kg body weight, in addition to other symptomatic therapy including diuretics, multivitamin, calcium etc
Conclusion: In pregnancy, as a body undergoes various physiological and hormonal changes, pregnant patients may present with symptoms of liver diseases, which normally subside after delivery. However, diagnosis and occurrence of PSC during pregnancy is quite rare but cannot be ignored. Therefore, critical attention shall be paid to pregnant women who report any fluctuations in liver enzymatic levels or complain of pruritis, as late diagnosis can lead to severe complications and maternal and fetal mortality.

Keywords: Primary Sclerosing Cholangitis, Cholangitis, Jaundice, Itching, Fatigue, MRCP .

Citation:

Dr.Parveen Malhotra, Primary Sclerosing Cholangitis - An Unusual Presentation. Japanese Journal of Gastroenterology 2026.