Can the histologic changes of cystic fibrosis-associated hepatobiliary disease be predicted by clinical criteria?

Background: Correlation between clinical parameters and histology changes in cystic fibrosis liver disease has not been documented. The purpose of this study was to determine the histologic spectrum of cystic fibrosis liver disease and the degree to which a clinical scoring system can identify subjects with significant histologic abnormalities. Methods: We reviewed the predictive value of physical examination, biochemical parameters, and a clinical liver score, incorporating physical examination and biochemical parameters, in predicting significant abnormalities of liver histology in 43 cystic fibrosis patients who underwent hepatic biopsy. Biopsies were scored by two masked pathologists for fibrosis, inflammation, inspissation, fatty infiltration, and congestion. Results: Significant histologic disease was present in 56% of patients despite little biochemical or physical examination evidence of disease. No single parameter used in the scoring system predicted the type or degree of the liver disease. The clinical liver score had a sensitivity of 85% and a specificity of 82% in predicting significant histologic changes, yet it was unable to predict the specific lesion. Conclusions: Significant histologic liver disease is common in cystic fibrosis, although the exact nature of the lesion cannot be predicted without liver biopsy. A clinical liver score that was developed for this may be useful in determining which patients require more definitive evaluation.