Clinical characteristics of systemic lupus erythematosus associated thrombotic thrombocytopenic purpura / 中华全科医师杂志

ABSTRACT

We retrospectively reviewed the clinical records of 12 systemic lupus erythematosus (SLE) patients with thrombotic thrombocytopenic purpura (TTP) admitted into Peking Union Medical College Hospital (PUMCH) during October 2008 to September 2013.TTP occurred in 0.45％ (12/2 693) of patients with SLE.The median age (2 males,10 females) was 23 years.Their SLEDAI scores ranged from 12 to 30.All patients had significant thrombocytopenia and renal abnormalities.Ten of them bad acute renal failure.Cardiac involvement and neurological deficits were observed in 9 and 8 of them respectively.Eight of them had injuries of more than 4 organs.Renal biopsy of 3 patients revealed lupus nephritis (type Ⅳ,n =2 ; type Ⅴ,n =1) and thrombotic microangiopathy.Eleven TTP patients received steroid pulse therapy (1 g/d,3 days) and six of them survived.Six patients were rescued by therapeutic plasma exchange and 4 of them recovered.Among another 6 patients not on plasma exchange therapy,only 3 recovered.Finally 2 died,3 gave up with exacerbated symptoms after therapy and 7 survived.