ステロイド中断後回腸壊死と腎症急性増悪をきたした全身性エリテマトーデスの1例

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A patient with systemic lupus erythematosus developed intestinal infarction and exacerbation of lupus nephritis after abrupt discontinuance of corticosteroid therapy. A 31-year-old woman was diagnosed as systemic lupus erythematosus two years ago when she had facial erythema, polyarthralgia, thrombocytopenia, proteinuria, casturia and positive antinuclear antibody. Prednisolone 45mg daily was begun with clinical and laboratory improvement. Prednisolone was gradually tapered to 17.5mg, when she stopped to take medication by herself. One month later abdominal pain and distention developed, and she was admitted to this hospital. On examination she had dehydration, fever, abdominal distention and renal failure. Prednisolone was administered with little clinical improvement. On the tenth hospital day because of massive melena, she underwent emergency laparotomy, which revealed necrosis of the terminal ileum. After the operation, disseminated intravascular coagulation and progressive renal failure developed and the patientdied on the 9th day after laparotomy.Histopathologic examination of the resected terminal ileum demonstrated fibrinoid necrosis of small arteries and arterioles with thrombi in the necrotic intestinal walls.In many cases of systemic lupus erythematosus associated with intestinal necrosis, necrotizing vasculitis was found. However, there was one case report of systemic lupus eryt-hematosus associated with hemorrhagic necrosis of the intestines without evidence of vasculitis, in which intimal hyperplasia, medial elastosis and reduplication of the laminaelastica in the small mesenteric arteries were found. In our case the necrosis of the terminal ileum might have developed due to ischemia from necrotizing vasculitis and thrombi.Postmortem examination demonstrated marked fibrinoid necrosis in glomerular tufts and intimal thickening of the afferent arterioles, which might explain rapid deterioration of renal function. Fibrinoid necrosis was also found in the spleen and the pancreas.It is suggested that the abrupt discontinuance of corticosteroid therapy may have aggravated the systemic vasculitis causing necrosis of the terminal ileum and rapidly progressive renal failure.
日本臨床免疫学会の論文

ステロイド中断後回腸壊死と腎症急性増悪をきたした全身性エリテマトーデスの1例

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