Bleeding events in thrombotic antiphospholipid syndrome: prevalence, severity, and associated damage accrual.

Background: Life-long anticoagulation increases bleeding risk in patients with antiphospholipid syndrome (APS). The Damage Index for Antiphospholipid Syndrome does not include bleeding events in damage accrual. Objectives: We aimed to characterize the prevalence, severity, and damage associated with bleeding events in patients with APS. Methods: This was a single-center retrospective analysis of patients with thrombotic APS (2006 Sydney criteria). Bleeding events were reviewed up to 43 years and classified according to the ISTH definitions into 2 groups: 1) major bleedings and 2) nonmajor bleedings (minor bleedings and clinically relevant nonmajor bleedings). Damage events were recorded as bleeding events a) resulting in permanent (>6 months) decrease in organ function and b) complicated by total/partial organ resection. Results: Among 197 patients (2412 patient-years [PYs] of follow-up), all of whom had been exposed to antithrombotic therapy, 40.6% experienced 167 bleedings (6.9 events per 100 PYs), of whom 61.3% had nonmajor bleedings (77.2% of bleedings: 42.6% minor, 57.4% clinically relevant nonmajor) and 38.8% had major bleedings (22.8% of bleedings; 1.6 events per 100 PYs). Soft/connective tissue was affected in 44.3% of bleedings, and 94.6% were nonmajor bleedings. Central nervous system was affected in 20.9% of bleedings, and 62.9% were major bleedings. Bleeding events were spontaneous in 90.4% of cases, and thrombocytopenia was likely involved in 62.2% of bleedings. Damage occurred in 11.4% of bleedings and affected 7.6% of patients. Most of the damage was associated with central nervous system events (8.4% of all bleedings). Conclusion: Approximately 40% of patients experienced at least 1 bleeding, and almost 8% of patients were left with organ damage not recognized by the current version of the Damage Index for Antiphospholipid Syndrome.