J Gastroenterol 1994; 29:362-368 Journal of Gastroenterology I 9 Springer-Verlag 1994 Post-infantile giant cell hepatitis in an elderly female patient with systemic lupus erythematosus KAZUFUMI DOHMEN, 1 SHOJI OHTSUKA, 1 HARUKI NAKAMURA, l KOICHI ARASE, 1 YASUSHI YOKOGAWA, 1 RYOUKICHI ASAYAMA,[ SHIGEKAZU KUROIWA, 2 and HIROMI ISHIBASHI 3 Departments of ~ Internal Medicine, and 2Pathology, Hamanomachi General Hospital, 3-5-27 Maizuru, Chuo-ku, Fukuoka, 810 Japan 3First Department of Internal Medicine, Faculty of Medicine, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812 Japan Abstract: A 69-year-old Japanese female was admitted because of general fatigue. Laboratory data showed elevation of serum total bilirubin, transaminase, gamma-glutamyl transpeptidase, and creatinine levels. An immunological study revealed hypergammaglo- bulinemia, low titer of complement, and high titers of antinuclear antibody, anti-DNA antibody, and circul- ating immune complexes. Antibodies to parainfluenza virus 3 were positive. Histology of the liver disclosed numerous giant cell hepatocyte transformations with the lobular architecture being slightly distorted by portal inflammation and fibrosis. These findings led us to make a diagnosis of giant cell hepatitis associated with systemic lupus erythematosus. Prednisolone was effective in improving the anemia and the serum immunoglobulin, immune complex, and antinuclear antibody levels. The addition of cyclosporine to the initial corticosteroid therapy was also beneficial in decreasing the transaminase level and in improving liver histology. The patient died of acute pneumonitis and renal failure on the 166th day after admission. Parainfluenza virus 3 and autoimmune mechanisms were thus considered to be the causes of the giant cell hepatitis. Key words: giant cell hepatitis, systemic lupus ery- thematosus, azathioprine, cyclosporine, parainfluenza virus Introduction Giant cell hepatitis is characterized by a unique histo- pathology, featuring giant cells. This unusual disease Offprint requests to: K. Dohmen (Received for publication on Aug. 23, 1993; accepted on Oct. 22, 1993) process predominantly affects children; however, it does, rarely, occur in adults, in immunoincompetent individuals 1'2 or in those receiving either cytotoxic or immunosuppressive drugs. 3'4 The disease has also been attributed to viruses such as the hepatitis B virus,5 non-A non-B hepatitis virus, 6-8 human immunodefi- ciency virus, 9 cytomegalovirus, 1~ and, most recently, to paramyxovirus infection. 11 The precise cause of this disease, however, remains unknown. Regarding the prognosis of giant cell hepatitis, Phillips et al. ~ reported that this disease has a poor prognosis, while Lau et al. a2 concluded that giant cell hepatitis does not always indicate an ominous prog- nosis. Devaney et al. ~ and Kobayashi et al. 13 reported that patients with post-infantile giant cell hepatitis responded effectively to prednisolone. In this report we describe a case of systemic lupus erythematosus associated with post-infantile giant cell hepatitis. To our knowledge, only one case featuring the coexistence of these two rare disorders has been reported previously. 1 Case report A 69-year-old Japanese female was admitted because of fatigue. One year previously she had been admitted to another hospital with a clinical diagnosis of auto- immune hepatitis. Prednisolone had been administered with an initial dosage of 40mg/day, later tapered to 25mg/day over a 10-month period. However, the patient stopped taking prednisolone on her own 2 months before admission to our hospital. She had no family members with liver diseases. She had never been to any developing countries and had never been exposed to potential hepatotoxic drugs before the onset of the disease. On admission her height was 147.7cm and weight 41.5kg. Her temperature was 36.6~ pulse rate 104