Survival and prognostic factors of death risk in antiphospholipid syndrome: results of 8-year follow-up

Abstract

Aim. To evaluate survival and mortality in antiphospholipid syndrome (APS) as well as prognostic factors of APS deterioration.
Material and methods. We retrospectively studied 248 case histories of patients admitted to the Institute of Rheumatology for 8 years. Primary APS was diagnosed in 35 patients, SLE+APS (according to criteria of ACR, 1982) - in 122 patients and SLE without APS - in 91 patients. Mean age was 31.2 ± 15.0 years (range from 14 to 63), median length of follow-up from the time of diagnosis was 11.9 ± 5.4 years. During 8 year period all the patients annually and the latest 5 years at least twice a year were examined for the presence oflgG and IgM-anticardiolipin antibodies (aCL) and lupus anticoagulant (LA). Thrombotic events were verified with special techniques.
Results. Thirty-eight patients (15%) died during the follow-up period. Mean age of the deceased was 35.4 ± 12.2 years (range 21-52 years) and the disease duration 8.6 ± 8.2 years (range 0.6-20), the median length of the survival from the time of the diagnosis was 6.2 ± 4.3 years. The 8-year survival for SLE patients without APS was 98%, for those with SLE+APS - 75% and for patients with primary APS - 83%. The presence of APS in SLE patients was significantly associated with high mortality (X2 - 12.3, freedom = 4, p = 0.006). Cox regression analysis revealed that the activity of the disease at onset, arterial thrombosis, especially recurrent, thrombocytopenia, valvular disease of the heart, capillaritis, digital necrosis and nephritis were independent risk factors for mortality (p < 0.05). Conclusion. Thus, long-term follow-up is necessary for patients with antiphospholipid antibodies especially with APS which lowers survival of SLE patients. Such patients need early corrective therapy to prevent thrombotic events.

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