PT  - JOURNAL ARTICLE
AU  - F P Vleggaar
AU  - H R van Buuren
AU  - P E Zondervan
AU  - F J W ten Kate
AU  - W C J Hop
AU  - the Dutch Multicentre PBC study group
TI  - Jaundice in non-cirrhotic primary biliary cirrhosis: the premature ductopenic variant
AID  - 10.1136/gut.49.2.276
DP  - 2001 Aug 01
TA  - Gut
PG  - 276--281
VI  - 49
IP  - 2
4099  - http://gut.bmj.com/content/49/2/276.short
4100  - http://gut.bmj.com/content/49/2/276.full
SO  - Gut2001 Aug 01; 49
AB  - The clinical and pathological findings of four females with primary biliary cirrhosis (PBC) with an unusual and hitherto not well recognised course are reported. Patients suffered severe pruritus and weight loss with progressive icteric cholestasis which did not respond to such treatments as ursodeoxycholic acid and immunosuppressives. In all cases liver histology revealed marked bile duct loss without however significant fibrosis or cirrhosis. Further diagnostic studies and repeat biopsies confirmed the absence of liver cirrhosis as well as other potential causes of hyperbilirubinaemia. Comparison of the fibrosis-ductopenia relationship for our cases with that for a group of 101 non-cirrhotic PBC patients indicated that in the former the severity of bile duct loss relative to the amount of fibrosis was significantly higher. The proportion of portal triads containing an interlobular bile duct was 3%, 4%, 6%, and 10% compared with 45% (median; range 8.3–100%) for controls (p<0.001). Three patients received a liver transplant 6–7 years after the first manifestation of PBC because of progressive cholestasis, refractory pruritus, and weight loss, while the fourth patient is considering this option. In one case cirrhosis had developed at the time of transplantation while the others still had non-cirrhotic disease. These cases suggest that cholestatic jaundice in non-cirrhotic PBC may be secondary to extensive “premature” or accelerated intrahepatic bile duct loss. Although the extent of fibrosis may be limited initially, progression to cirrhosis appears to be inevitable in the long run. Despite intact protein synthesis and absence of cirrhotic complications, liver transplantation in the pre-cirrhotic stage for preventing malnutrition and to improve quality of life should be considered for these patients.PBCprimary biliary cirrhosisAMAantimitochondrial antibodyUDCAursodeoxycholic acid