ＳＬＥにｓｙｓｔｅｍｉｃ Ｗｅｂｅｒ Ｃｈｒｉｓｔｉａｎ病を伴いタクロリムスが著効を示した１例

Abstract

We report a 30 years old systemic lupus erythematosus (SLE) female patient, who had been diagnosed with systemic Weber Christian disease, presented with acute onset of high fever, hepatomegaly, lower leg edema with multiple subcutaneous nodules. Laboratory tests showed elevated CRP, fibrinogen and liver enzymes, especially LDH and LAP, with coagulation abnormalities. There is a marked enlarged fatty liver in both abdomen echo and CT and chest X-ray films showed pleural effusion in both sides, without pulmonary lesions. However, complements, ANA and anti-DNA antibody etc concerning activity of SLE didn't moved. A diagnosis of systemic Weber Christian disease was made based on clinical and laboratory examinations and histopathological findings. The therapy of standard treatment, such as steroid, plus pulse steroid therapy was performed. The response, however, is unpredictable. After this, tacrolimus hydrate was administed intravenously in an initial dose of 1.2 mg/body and in oral maintenance of 1.0 mg/ body with a drastic response and patient completely recovered within 1 month. To our knowledge, this is the first report of SLE complicated systemic Weber Christian disease and is the first report of successful treatment of systemic Weber Christian disease with tacrolimus hydrate.