Scleroderma renal crisis in progressive systemic sclerosis: a case report

Abstract

発症より10年後腎クリーゼにて急性腎不全を起こした進行性全身性硬化症の1例を経験した.急激な腎機能低下, コントロール不良な高血圧, 大量の消化管出血を認め, 治療開始約1カ月後死亡した.本症例では入院時すでにDICと腎不全が高度であったこと, 消化管出血によりアンギオテンシン変換酵素阻害剤であるenalaprilが経口投与できなかったことが予後不良であった原因と考えられた.剖検時の腎の光顕所見で動脈内膜の肥厚と軽度の腎硬化像が認められた.

We report a case of progressive systemic sclerosis with scleroderma renal crisis 10 years after onset. The patient (female) had progressive renal dysfunction, hypertension which was difficult to control, and massive gastrointestinal bleeding. An angiotensin converting enzyme inhibitor (enalapril) could not control her hypertension. Only intravenous nicardipine had a slight effect on her hypertension. Hemodialysis and plasma exchange, did not reverse the renal crisis, and the patient died. Microscopic examination of her kidney showed thickening of the capillary walls and mild nephrosclerosis.