RESUMEN
BACKGROUND/AIMS: Estimation of prognosis becomes increasingly important in primary biliary cirrhosis (PBC) with advancing disease and also with regard to patient management. The ubiquitous used Pugh scoring for severity of disease is simple while the Mayo model which has been validated for survival estimates is more sophisticated. We wanted to investigate whether Pugh and Mayo scores correlate (they have 3 of 5 variables in common) and if so whether a survival probability based on Mayo data could be affixed on Pugh classes and scores obtained in the same patients. METHODOLOGY: All variables used for Mayo Clinic Prognostic Model (Mayo) scoring and Pugh-Child-PBC (Pugh) scoring were available in 143 PBC patients of the Pan European database Euricterus. Pugh scores P5-P15 and has classes A (P5-6), B (P7-9) and C (P10-15). We subdivided P5 in P5A (patients with albumin > 40 g/l plus prothrombin time < or = 12 secs) and P5B (the other patients in P5). We designed a category Pugh Early (PE) for patients with P5A characteristics and bilirubin < 17 mmol/l. Mayo scores R0-R15-with 1-7 years survival probabilities S-and has risk classes Low (L), Intermediate (Int), High (H) and Very High (VH). RESULTS: The estimated survival probabilities of the 143 patients ranged from 88% at 7 years to 0% at 1 year, median 14% at 5 years. The Pugh and Mayo scores correlated r = 0.87 (p < 0.0001) and except age with P, all Mayo and Pugh variables correlated with both R and P at p < 0.0001. Survival in Pugh class A was median 43% at 7 years and was not different from survival in Mayo L+Int (p 0.58). In Pugh class B 7 years survival was 2%, not different from Mayo H (p 0.25). Survival in Pugh C was median 24% at 1 years and better than Mayo VH (p 0.02). Between P5A (survival 78% at 7 yr) and R 3-4; P5B-6 (40% at 7 yr) and R5; P7 (22% at 7 yr) and R6; P8-11 (12% at 5 yr) and R7-8; and P12-14 (5% at 1 yr) and R9-10 no significant differences were found. From P8 upward there was a steep increase in death rate. PE has a 7 year survival of at least 89%. Charts of projected survival estimates for Pugh scores and classes are presented. CONCLUSION: It was possible (affixing Mayo to Pugh) to define 1-7 years survival probabilities to Pugh classes and scores for the last 7 years of the disease, i.e. the most important period for therapeutic decisions. These results need to be validated in other PBC populations.