Wallach's Interpretation of Diagnostic Tests: Pathways to Arriving at a Clinical Diagnosis (200 page)

   Serum IgM is increased in approximately 75% of patients; levels may be very high (four to five times normal). Other serum immunoglobulins are also increased.

   Hypocomplementemia.

   Polyclonal hypergammaglobulinemia. Serum IgM is increased in approximately 75% of patients with failure to convert to IgG antibodies; levels may be very high (four to five times normal). Other serum immunoglobulins are also increased.

   Biopsy of the liver categorizes the four stages and helps assess prognosis, but needle biopsy is subject to sampling error because the lesions may be spotty; findings consistent with all four stages may be found in one specimen.

   Serum ceruloplasmin is characteristically elevated (in contrast to Wilson disease).

   Liver copper may be increased 10–100 times normal; correlates with serum bilirubin and advancing stages of disease.

   ESR is increased one to five times normal in 80% of patients.

   Urine contains urobilinogen and bilirubin.

   Laboratory findings of steatorrhea:

   Serum 25-hydroxyvitamin D and vitamin A are usually low.

   PT is normal or restored to normal by parenteral vitamin K.

   Laboratory findings due to associated diseases:

   More than 80% have one, and >40% have at least two, other circulating antibodies to autoimmune disease (e.g., RA, autoimmune thyroiditis [hypothyroidism in 20% of patients], Sjögren syndrome, scleroderma) although not useful diagnostically.