PRIMARY SCLEROSING CHOLANGITIS - AN EXPERIENCE FROM INDIA

Primary sclerosing cholangitis (PSC) is considered to be rare in India . The aim of the present study was to investigate the incidence, clini cal profile and outcome of PSC seen in a tertiary care centre. Over a period of 10 years (July, 1984-June, 1994) 18 patients of PSC were dia gnosed at cholangiography (14 patients by endoscopic retrograde cholan giopancreatography, two patients by percutaneous transhepatic cholangi ography and two patients by both methods). The presence of secondary c auses, such as choledocholithiasis, biliary tract surgery, congenital biliary tract anomalies, cholangiocarcinoma and pancreatic diseases,we re excluded. These patients were evaluated retrospectively with respec t to their clinical presentation, radiological findings, presence of a ssociated idiopathic ulcerative colitis (IUC), treatment instituted an d outcome. The mean (+/- s.d.) age at diagnosis of PSC was 39.0 (+/- 1 6.1) years with a male: female ratio of 1.57:1. Nine (50%) patients ha d associated IUC. The diagnosis of IUC preceded that of PSC in all but one case. Fifteen (83.3%) patients had cholestatic jaundice at presen tation, while three (16.7%) patients had asymptomatic rise of alkaline phosphatase. Three (16.7%) patients had recurrent cholangitis and fiv e (27.8%) patients developed portal hypertension during the course of the disease. At cholangiography, intrahepatic radicles were involved i n all and extrahepatic radicles in 12 (66.6%) cases. Patients were man aged with steroids (n = 7), colchicine (n = 3), ursodeoxycholic acid ( UDCA; n = 2) and methotrexate (n = 1), along with symptomatic measures . Mean duration of follow up available in 11 (61%) patients was 20.1 m onths (range: 1 month-8 years). Four (36.4%) patients died. Steroids a nd colchicine did not have any effect while the one patient on UDCA an d one on methotrexate showed improvement. In conclusion, in India PSC does not seem to be a rare entity. Its clinical profile and outcome ar e somewhat similar to those seen in Western countries.