Abstract A 26-year-old Japanese woman with systemic lupus erythematosus (SLE) developed severe hyperten-sion and an increased active renin concentration (ARC), ischemic colitis, and splenic infarction. She had antiphospholipid antibodies (APA), multiple intrarenal microaneurysms, and multiple stenoses of the mesenteric arteries. Combination therapy with antihypertensive agents, aspirin, warfarin, and corticosteroids (30 mg daily) controlled her abdominal symptoms and hypertension. Multiple intrarenal microaneurysms in SLE with APA may be the cause of severe hypertension and elevated serum ARC.