Simultaneous macroamylasemia and macrolipasemia in a patient with systemiclupus erythematosus in remission

We report the fourth case of simultaneous macroamylasemia and macrolipasemi a. A 39-year-old woman had been treated for systemic lupus erythematosus fr om 1982 to 1993, She was found to have an unexplained increase in serum amy lase and lipase activities since 1996. Immunoprecipitation assay showed tha t amylase was bound to IgA2-kappa and IgA1-kappa (IgA2>IgA1), whereas lipas e was bound to IgA1-kappa, During a follow-up period up to December 1999, t he patient did not develop any additional autoimmune or lymphoproliferative disorders. In the situation of atypical clinical features associated with hyperamylasemia and hyperlipasemia, the possibility of macroenzymes should be considered.