An autopsy case of systemic lupus erythematosus with progressive liver dysfunction

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An autopsy case of systemic lupus erythematosus (SLE) with progressive liver dysfunction is reported. A 12-year-old female was admitted in October 1982 because of eruption on cheeks, hemolytic anemia and proteinuria. On admission, her laboratory examination revealed an elevated anti-single-stranded (ss) DNA antibody, severe coagulopathy, hypoalbuminemia and hypocomplementemia. After the administration of prednisolone, 40 mg/day, she looked clinically well. However, coagulopathy, hypocomplementemia and disturbance of ICG excretion (31.6%) were not improved. Laparoscopy, that was carried out under the supplement of coagulant factors, indicated that her hepatic injury was due to chronic active hepatitis. Therefore, azathiopurine was administered in combination with prednisolone. The titers of anti-ss and double stranded (ds) DNA antibodies, however, were gradually elevated. After the pulse therapy with methylprednisolone, 1 g/day, anti-ss and dsDNA antibody tests became negative, but she finally died in June 1983 because of DIC, followed by hepatic failure. An autosy revealed that the liver was in an early stage of liver cirrhosis showing massive necrosis, fibrosis, marked regeneration of bile stasis.It has been assessed that the liver involvement in SLE is mild and unworthy of attention for the management of the disease. However, her hepatic injury was closely related to the disease activity and progressive, indicating the association of hepatic damage with SLE. The liver involvement may be important as one of the prognostic indicators in some SLE patients.
日本臨床免疫学会の論文

An autopsy case of systemic lupus erythematosus with progressive liver dysfunction

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