Background: Antiphospholipid syndrome (APLS) carries a significant morbidity and can be fatal due to the risk of thrombosis and pregnancy complications. Malaysia is a multi-ethnic country with predominant Malay ethnicity, followed by Chinese and Indian. Due to its rarity, the clinical manifestations of this disease and the long-term outcomes in this multi-ethnic cohort were not well-studied.

Objectives: The objective of this study was to determine the clinical manifestations and the factors associated with damage accrual among patients with antiphospholipid syndrome.

Methods: This was a cross-sectional study and patients who fulfilled the revised 2006 Sapporo classification criteria for APLS were recruited from Hospital Canselor Tuanku Muhriz, Universiti Kebangsaan Malaysia (UKM). This study was conducted from July 2021 until July 2023 and has obtained ethical approval from the UKM research ethics committee (FF-2021-309). Their medical records were reviewed to determine the baseline demographic characteristics, the antiphospholipid antibody positivity characteristics and the initial clinical presentation of APLS. Presence of organ or disease damage at the last clinic visit was determined using the antiphospholipid syndrome damage index (DIAPS) score. Univariate and multivariate analyses were performed to determine the factors associated with organ/system damage.

Results: A total of 81 patients were included in the study and 72.8% (n=59) had APLS with Systemic Lupus Erythematosus (SLE). Majority of the patients were females (n=69, 85.2%) with predominant Malay (n=54, 66.7%), followed by Indian (n=15, 18.5%) and Chinese (n=12, 14.8%). Their mean age of onset was 33 ± 11.9 years old and the disease duration was 13.39 ± 6.71 years. Stroke was the commonest initial presentation (n=25, 38.5), followed by DVT (n=23, 28.3%) and pulmonary embolism (n= 11, 13.6%). Almost half of them, 42.9% (n=30) had thrombocytopenia at the initial presentation. A total of 49 patients (60.5%) accrued organ/system damage and the median DIAPS score in this cohort was 1 (IQR0-4). The commonest system damage was central nervous system due to stroke (n=25, 38.5%) followed by digital gangrene (n=12, 15%), pulmonary hypertension (n=6, 7.5%) and avascular necrosis (n=4, 5%). After adjustment of the ethnic and disease duration, the only factor that was associated with organ/system damage in the multivariate analysis was lupus anticoagulant positivity, OR 3.32 (95% C.I 1.03-10.77), p=0.04.

Conclusion: Prevalence of disease damage was high and lupus anticoagulant was the single independent factor associated with damage. Stroke was the commonest initial manifestation in our multi-ethnic Malaysia APLS patients, hence it is very important to screen for APLS in young patients presented with stroke.

REFERENCES: [1] Cervera R, Serrano R, Pons-Estel GJ, Ceberio-Hualde L, Shoenfeld Y, de Ramón E, et al. Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients. Ann Rheum Dis. 2015;74(6):1011-8.

[2] Torricelli AK, Ugolini-Lopes MR, Bonfá E, Andrade D. Antiphospholipid syndrome damage index (DIAPS): distinct long-term kinetic in primary antiphospholipid syndrome and antiphospholipid syndrome related to systemic lupus erythematosus. Lupus. 2020;29(3):256-62.

Acknowledgements: This study obtained financial grant from the Faculty of Medicine Fundamental Grant (FF-2021-309).

Disclosure of Interests: Syahrul Sazliyana Shaharir Astra Zeneca, Novartis, Pfizer, GSK, Astra Zeneca (30,000USD), Nurasyikin Yusof: None declared, Nor Rafeah Tumian: None declared, Rabani Remli: None declared, Rahana Abdul Rahman: None declared.