Abstract
We report a 29-year-old Japanese woman who had a 5-year history of adult onset Still’s disease (AOSD) and showed exacerbation in the second remission with hemophagocytic syndrome (HPS) and disseminated intravascular coagulopathy (DIC) after she developed acute deterioration of liver functions from the carrier state of hepatitis B virus (HBV). On admission, she showed a progressive rise in transaminase with an increase of HBV-DNA polymerase (DNAp). Although liver functions were immediately improved by the administration of Stronger Neo-Minophagen C (SNMC), AOSD was exacerbated with a fever, skin eruptions, elevation of LDH, pancytopenia and abnormal findings in coagulation studies with highly elevated ferritin and sIL-2R. Bone marrow aspiration demonstrated the increase of myeloid cells and histiocytes which showed hemophagocytosis. The administration of high dose methyl-prednisolone combined with the therapy for DIC ameliorated her general condition. There have been several reports that glycyrrhizin, a major component of SNMC, enhances interleukin (IL)-12 production, which is known to have functions such as the induction of Th1 subset from naive Th cells and the activation of macrophages together with IL-18. Moreover, HBV has been reported to induce IL-18 production. Taken together, the activation of macrophages and Th1 cells might be induced by glycyrrhizin and reactivated HBV through the overproduction of IL-12 and/or IL-18, resulting in the exacerbation of AOSD in our case.
