Acute phase reactants in patients with Wilson's disease

Background: Wilson's disease (WD) is a rare disorder of copper metabolism frequently associated with hepatic involvement leading to liver cirrhosis. Serum concentration of the negative acute phase reactant human fetuin A/α2HS-glycoprotein (AHSG) is a good predictor of mortality due to liver failure in patients with alcoholic cirrhosis. Our aim was to study the AHSG and another acute phase reactant in WD with special respect to liver cirrhosis.

Patients and methods: Serum concentrations of positive (α1-acid glycoprotein/orosomucoid, OROSO) and negative (AHSG) acute phase reactants were studied in 50 patients with Wilson's disease (29 men, 21 women, age:33.9±12.4 years, mean±SD, each treated with d-penicillamine and vitamin B6). The diagnosis of Wilson disease was based on the international scoring system. AHSG and OROSO levels were determined by radial immunodiffusion. Results were compared to age matched healthy controls (n=51).

Results: The OROSO levels were lower in WD patients compared to healthy controls (53±19 versus 87±15; p<0.001). There was no difference in serum AHSG levels between Wilson disease patients and healthy controls (598±148 vs. 605±88;NS). The AHSG levels were slightly elevated in subgroup of WD patients with non cirrhotic liver involvement (676±149, n=24) and lower in patients with liver cirrhosis (490±58;n=10; p<0.001 compared to each other and also to healthy controls). In patients with liver involvement the AHSG level of 510µg/ml was associated with cirrhosis with 70.0% sensitivity and 83.3% specificity as determined by the receiver operator curve analysis (p=0.001). Serum AHSG levels ≤510µg/ml were associated with a significantly increased risk of liver cirrhosis (Relative risk: 3.807, 95% CI.:1.117–12.973, p=0.030).

No correlation was found between the levels of OROSO or AHSG and any investigated chemical laboratory tests and the type of Wilson disease gene mutation.

Conclusion: Our results confirm that low serum AHSG concentration (below 510µg/ml) could be considered as an indicator for liver cirrhosis in Wilson disease similarly to that of other aetiology.