Liver transplantation for primary sclerosing cholangitis (PSC) – case report
| Author |
|---|
| Date | Start Page | End Page |
|---|---|---|
2024-05-10 | 118 | 118 |
Prieskyros nenurodytos.
Tutor: Irena Valantienė
INTRODUCTION Primary sclerosing cholangitis (PSC) is a rare disorder with progressive destruction of the bile ducts due to recurring inflammation and fibrosis. The clinical course of the disease is variable but may lead to cirrhosis and chronic liver failure. Approximately 40% of PSC patients require a liver transplant over their lifetime. However, the disease can recur after transplantation.
AIM OF THE STUDY
MATERIALS AND METHODS A 25-year-old man is being monitored by gastroenterologists after a second liver transplantation for PSC. The patient also has IBD – ulcerative colitis (UC). According to medical history, the patient was diagnosed with PSC in childhood. The disease eventually progressed to cirrhosis, complicated by ascites, and esophageal varices. Child-Pugh score was 12. The patient‘s condition deteriorated to liver failure (MELD score 20), and he underwent a liver transplantation by the age of 19 years old. Unfortunately, two years later, the disease recurred. The patient had multiple episodes of ascending cholangitis. MRCP was performed which showed narrowing of the ductus hepaticus communis. To improve bile outflow, a cutting balloon treatment was used to dilate duct stricture, however minimal effect was obtained. Therefore it was decided to use an endoscopic biliary stenting technique. A stent with a valve was used to prevent ascending inflammations. Transjugular liver biopsy was performed to evaluate PSC recurrence in the transplanted liver. Histological analysis showed PSC signs – bile duct loss, and portal fibrosis with inflammatory infiltrate. With further recurrence of ascending cholangitis and continued need for hospitalization, the patient was placed on the liver transplant waiting list for a second time. The liver was retransplantated within 4 years from the first transplantation. The patient is currently stable on tacrolimus immunosuppressive monotherapy. Long-term treatment with ursodeoxycholic acid is also prescribed. The UC is in endoscopic and clinical remission, maintained with mesalazine.
CONCLUSIONS PSC recurs in 8,6% to 27% of patients after liver transplantation within an average of 4,7 years. The disease usually presents in a severe form and requires retransplant. Unfortunately, it is well known that survival decreases with each liver retransplant.