Prognostic factors and clustering of serious clinical outcomes in antiphospholipid syndrome

We assessed whether initial clinical presentations suggestive of antiphosph olipid syndrome (APS) predicted the subsequent rate and type of serious cli nical outcomes. Eighty-two consecutive patients with anticardiolipin antibo dies or lupus anticoagulant were followed for 814 person-years after a firs t event suggestive of APS (livedo reticularis, thrombocytopenia, autoimmune haemolysis, thrombosis, central nervous system manifestations, recurrent a bortions). The hazard of developing a second event was largest in patients with antibodies recognizing beta 2 glycoprotein I who had autoimmune haemol ysis as the first event (hazard ratio HR 2.70, p = 0.018) and smallest in p atients without such antibodies who had recurrent abortions as their first event (HR 0.37, p = 0.028). Subsequent serious events in patients with veno us and arterial thromboses, recurrent abortions, central nervous system man ifestations and autoimmune haemolytic anaemia were likely to be of the same type as the presenting event (odds ratio (OR) 3.76, 5.90, 77.7, 6.92, and 7.13, respectively). Adjusting for therapy, the rate of subsequent serious events was 6.86-fold higher (p = 0.0001) in patients presenting with two ev ents, 1.56-fold higher (p = 0.038) in autoimmune haemolysis presentations, 1.69-fold higher (p = 0.004) in patients with anti-beta 2-glycoprotein-I an tibodies, and 46% (p = 0.063) lower in thrombocytopenia presentations. Init ial clinical features determine the long-term evolution of APS, and specifi c types of clinical manifestations cluster during the course of the disease .