Clinical Features Of Primary Sclerosing Cholangitis: An Analysis Of 12 Cases

Objective: To study the clinical features, diagnosis and therapies of primary sclerosing cholangitis (PSC). Methods: 12 cases of primary sclerosing cholangitis were reviewed retrospectively. Clinical manifestations, diagnostic studies, treatments as well as patients' response to therapy were examined and analyzed. Results: The 12 patients, 8 male and 4 female, had an average age of 47.67 years (range 17-71 years) at diagnosis. Among them, 1 case of ulcerative colitis and cholangiocarcinoma each was found concurrently. Jaundice and fatigue were two symptoms most frequently seen, followed by weight loss, pruritus, fever and abdominal uncomfortableness. Hepatosplenomegaly, ascites and edema at lower limbs were present in some cases during physical examination. Liver function tests showed abnormal results characterized by an elevated serum alkaline phosphatase level (306.25±231.35U/L) which was seen in nearly all cases. Serum bilirubin levels rose significantly with cholestatic characteristics. Some cases had altered blood concentration of immunoglobulins and complements. Perinuclear antineutrophilcytoplasmic antibody (p-ANCA) was positive in 7cases (58.33%), antinuclear antibody (ANA) was positive in 2 cases, and antimitochondrial antibody (AMA) was positive in 1 case. Diagnosis of PSC in all cases was confirmed by endoscopic retrograde cholangiopancreatography, magnetic resonance cholangiopancreatography or percutaneous transhepatic cholangiography. Main findings in cholangiography consisted of multifocal structuring, irregularity and dilation involving both intra- and extrahepatic bile ducts. Liver biopsy showed different degrees of portal inflammation and fibrosis with bile duct proliferation or obliteration that were compatible with histological changes of PSC. 8 cases received medical therapy using ursodeoxycholic acid (UDCA), 1 case received liver transplantation, and 1 case had cholangiocarcinoma resection with choledochojejunostomy. 6 of the 8 cases drove benefits from medical therapy with symptomatic and biochemical alleviation. The liver transplantation receiver had symptoms relieved and liver function return to almost normal after operation. Conclusion: Primary sclerosing cholangitis often occurs in mid-aged population with a male predominance. The clinical manifestations of PSC are variable and nonspecific. The presence of cholestatic liver test abnormalities in patients particularly with inflammatory bowel disease should alert clinicians to the possibility of PSC and be further examined by ERCP or MRCP for diagnosis. Follow-up study and medical therapy are required for PSC patients in order to monitor the development of colorectal and biliary malignancies as well as advancement of liver disease. Liver transplantation remains an effective treatment for eligible patients with suitable timing.