Patients with primary biliary cirrhosis (PBC) who are symptomatic have a worse prognosis than those who are asymptomatic. The following prognostic score was developed at the Yale University School of Medicine.
Findings associated with poor prognosis:
(1) jaundice
(2) weight loss
(3) hepatomegaly
(4) splenomegaly
(5) ascites
(6) bridging fibrosis or cirrhosis on liver biopsy
(7) cholestasis on liver biopsy
(8) periportal hepatocellular necrosis on liver biopsy
Parameters identified in multivariate analysis:
(1) age: older age unfavorable
(2) hepatomegaly at onset: unfavorable
(3) portal fibrosis without bridging fibrosis or cirrhosis: favorable
(4) serum bilirubin: favorable if low, unfavorable if high
Parameter |
Finding |
Value |
hepatomegaly |
present at onset |
1 |
|
absent at onset |
0 |
liver biopsy |
portal fibrosis without bridging fibrosis or cirrhosis |
1 |
|
bridging fibrosis or cirrhosis present |
0 |
serum bilirubin |
> 5.0 mg/dL |
0.82 |
|
1.5 - 5.0 mg/dL |
0 |
|
< 1.5 mg/dL |
-0.73 |
prognostic score =
= (0.037 * (age in years)) + (0.74 * (hepatomegaly value)) - (1.34 * (portal fibrosis value) + (bilirubin value)
Interpretation:
• The lower the score the better.
Purpose: To predict the prognosis in a patient with primary biliary cirrhosis (PBC) based on clinical findings.
Specialty: Gastroenterology, Immunology/Rheumatology
Objective: clinical diagnosis, including family history for genetics, other testing, severity, prognosis, stage
ICD-10: K74.3,