One of the main causes of death is hypeensive renal crisis, characterised by rapidly developing malignant hypeension and renal failure. Hypeensive renal crisis is much more likely to occur in DCSS than in LCSS, and in patients with topoisomerase 1 antibodies. Hypeension should be treated aggressively with ACE inhibitors, even if renal impairment is present. DAVIDSON&;S 22nd EDITION;PAGE NO 1113