Mechanic and colleagues' report on granulomatous disease in common variable immunodeficiency [1] described one patient with primary biliary cirrhosis. To our knowledge, this pairing has not been previously reported. We have been following a woman who was found to have both disorders at age 72 years. She had been experiencing repeated pulmonary infections for 7 years. By 1995, her IgG level was 40 mg/dL and her IgA level was 50 mg/dL. The IgM level was increased to 368 mg/dL with a small IgM monoclonal spike. The absolute lymphocyte count was only 672 cells/mm³ with 110 B cells, 370 helper T cells, and 100 suppressor T cells. Human gammaglobulin administered every 2 months has eliminated most infections.

Because of persistently elevated alkaline phosphatase levels (maximum, 250 U/L) and mild increases in alanine aminotransferase and aspartate aminotransferase levels, liver biopsy was performed. Examination of the specimen showed primary biliary cirrhosis. Antimitochondrial antibody of the IgM class was present at a titer of 1:2560. Ursodiol, 600 mg/d over the next 18 months, resulted in normalization of the alkaline phosphatase and liver enzyme levels. The antimitochondrial antibody titer decreased to 1:40.

Despite profound reductions in T and B cells, the patient was capable of mounting a specific antibody response to mitochondrial antigen. For most patients with only primary biliary cirrhosis, the titer remains elevated despite therapy. It is impossible to know whether gammaglobulin, ursodiol, or both therapies have contributed to the reduction of the antibody titer. It is also possible that the decrease in the titer reflected the instability of an immune response in a patient with common variable immunodeficiency. Normalization of the liver enzyme levels, however, indicates improvement in liver pathology. We concur that it is important to diagnose hypogammaglobulinemia because of the possibility that it contributes to the injurious granulomatous changes.