We herein report on a 64-year-old Japanese female patient who presented wit
h a splenic artery aneurysm (SAA) associated with systemic lupus erythemato
sus (SLE). The saccular aneurysm, which measured 3 cm in diameter, was loca
ted in the proximal third of the splenic artery from the pancreas with a po
rtosystemic shunt. A double ligation of the splenic artery (the distal and
proximal sides of the aneurysm) was performed without a splenectomy. The po
stoperative course showed acute pancreatitis without either splenic infarct
ion or portal thrombus. To our knowledge, the closed association of SLE wit
h an aneurysmal dilatation of the splenic artery has not been previously re
ported. Both the pathogenesis and the management of SAA associated with SLE
are discussed following the presentation of this case. This is the first r
eported case of SAA associated with SLE.