OC 06.5 - Prevalence and Severity of Bleeding Events in Thrombotic Antiphospholipid Syndrome - Should They be Included in the Damage Index for Antiphospholipid Syndrome?

Saturday, July 9, 2022

2:00 PM – 2:15 PM

Location: ICC Capital Suite Room 10&11

Presentation Format: In-Person

Presenting Author(s)

Pedro Gaspar, MD

Physician
North Lisbon University Hospital Centre, Lisbon, Portugal; Instituto de Medicina Molecular (IMM), Cellular Immunology Lab, Faculty of Medicine, University of Lisbon, Lisbon, Portugal
Póvoa De Santa Iria, Lisboa, Portugal

Co-Author(s)

FF

Filipa Farinha

Researcher
Centre for Rheumatology, Division of Medicine, University College London, London, UK
London, England, United Kingdom
Zara Sayar, MBChB MD MRCP FRCPath

Consultant Haematologist
University College London Hospitals NHS Foundation Trust, London, UK; Whittington Hospital, London, UK
London, England, United Kingdom
ME

Maria Efthymiou

Senior Research Fellow
Haemostasis Research Unit, Department of Haematology, University College London, London, UK
London, England, United Kingdom
Hannah Cohen, MD

Professor of Haematology and Consultant Haematologist
Department of Haematology, University College London Hospitals NHS Foundation Trust, London, UK
London, England, United Kingdom
DI

David Isenberg

Professor
Department of Rheumatology, University College London Hospitals NHS Foundation Trust, London, UK
London, England, United Kingdom

Background: Thrombotic antiphospholipid syndrome (APS) patients are treated with life-long anticoagulation, associated with increased bleeding risk. In lupus, treatment-related events (e.g.: diabetes, cataracts) are considered to be damage. In contrast, the Damage Index for Antiphospholipid Syndrome (DIAPS) does not include bleeding events (BE) in damage accrual.

Aims: To characterize the cumulative prevalence and severity of BE and its related damage in thrombotic APS patients.

Methods: Single-centre retrospective analysis of thrombotic APS patients (2006 Sydney criteria). BE were classified according to ISTH definitions for minor-BE (mi-BE), clinically relevant non-major-BE (CRNM-BE), and major-BE in non-surgical (Mns-BE) and surgical (Ms-BE) patients. Patients were then classified in two groups: 1) major-BE: Mns-BE and Ms-BE; and 2) non-major-BE: mi-BE and CRNM-BE. Damage events were recorded as: a) BE with persistent neurologic deficits; b) BE complicated by total/partial organ resection; c) BE resulting in decreased organ function.

Results: We identified 197 APS patients (female, 71.1%; primary APS, 65.9%; Caucasian, 72.4%; median age at APS onset 40 years; median follow-up time, 10 years), all on anticoagulant and/or antiplatelet agents. 169 BE occurred in 80 patients (40.6%), of whom 50 (62.5%) had non-major BE (126/169, 74.6% of events), and 30 (37.5%) had major-BE (43/169, 25.4% of events) (Table 1). Mucocutaneous BE were the most frequent (32.5%) and none led to damage. Central nervous system (CNS) was the organ/system most affected by major-BE (55.8%), followed by gastrointestinal (n=7, 16.3%) and genitourinary (GU) (n=4, 9.3%). Damage occurred in 11.8% (n=20) of BE and affected 7.6% (n=15) of patients (50.0% with major BE). Most of the damage was associated with CNS (8.3% of all BE) and GU events (2.4% of all BE).

Conclusion(s): Approximately 40% of patients experienced at least one BE and almost 20% of those were left with permanent disability. We suggest that BE should be incorporated into DIAPS.