Background and aims The antiphospholipid syndrome (APS) has wide clinical and outcome spectrum overtime. The aim of this study was to assess the real life prevalence of morbi-mortality APS during 10 year-follow-up of a single referral centre.
Methods The cohort included 160 consecutive APS patients followed by the Autoimmune Diseases Unit of Hospital Clínic Barcelona from 2003 to 2013. Epidemiological, clinical, immunological and treatment features were analysed prospectively.
Results The cohort consisted of 126 (79%) women and 34 (21%) mens. Mean (SD) age at diagnosis was 39 (14) years. The diagnostic causes were thrombosis (56.3%), obstetric morbidity (26.9%) and both (16.9%). 65% were primary APS, 22.5% associated with systemic lupus erythematosus (SLE), 8.8% associated with lupus-like syndrome and 3.7% associated with other diseases. Fifty-five patients were lost to follow-up (3.4% every year). In evolution, 10 primary APS patients were reclassified as SLE-associated APS and 2 patients developed catastrophic APS. Table 1 shows the frequencies of the main APS clinical manifestations at baseline and during the 10-year-follow-up. At diagnosis, 95% received antithrombotic treatment: low dose antiaggregants (39.5%), oral anticoagulants (67.1%), heparin (2.6%). During the study, 72.7% of recurrences were without antithrombotic treatment and 27.3% were despite it. Eleven major bleeding episodes occurred and 2 were fatal. The global mortality rate was 6.9% and 50% in catastrophic APS. Table 2 shows the main causes of death and Figure 1 is a Kaplan–Meier survival curve.
Conclusions This study shows long-term morbidity and mortality of a large APS patient cohort and exposed the real-life experience of a referral unit.
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