Objectives:To report the clinical presentation and outcomes of the APS in Hong Kong Chinese patients.Methods:The HKAPS registry was established in early 2020 by the Hong Kong Society of Rheumatology to study the outcomes of Chinese patients with APS treated in public hospitals in Hong Kong. Patients aged ≥18 years were identified by the Hospital Authority Clinical Data retrieval system using the ICD-10 diagnostic code of APS. The medical history and APS diagnosis was verified by sub-investigators in different hospitals using the 2006 modified consensus criteria for the APS. Eligible patients were classified into definite APS or probable APS, which was further categorized into primary (without underlying rheumatic diseases) and secondary types. The presentation, treatment and outcomes of these patients were summarized.Results:232 APS patients (76.3% women) were identified. All were ethnic Chinese. A total of 160 patients fulfilled the 2006 criteria for APS (definite APS) while 72 patients had probable APS (anti-phospholipid [aPL] antibodies positive once or with non-criteria manifestations). In those with definite APS, the mean age at diagnosis was 44.9±15.8 years and the female to male ratio was 3.1:1. APS was primary in 82 patients while 78 patients had concomitant rheumatic diseases (SLE in 95% of patients). 130(81%) patients had thromboembolic manifestations, 20(13%) had obstetric morbidities and 10(6%) had both. In patients with secondary APS, 23% thrombotic or obstetric manifestations occurred before diagnosis of the rheumatic diseases (ie. evolved from primary APS). Lupus anticoagulant was present in 81(51%) patients, moderate/high titers of anti-cardiolipin were present in 90(56%) patients and anti-β2glycoprotein-1 was present in 6(4%) patients. Sixteen (10%) patients were double positive while 1 patient was triple positive for these aPL antibodies. Among the thromboembolic manifestations, arterial thrombosis (n=84) (ischemic stroke 77%, myocardial infarction 4.8%, peripheral vascular disease with limb/digital gangrene 2.4%, retinal artery 2.4%, splenic artery 1.2%, bowel infarct 1.2%) was more common than venous thrombosis (n=73) (calf vein thrombosis ± pulmonary embolism 86%, cerebral veins 2.7%, inferior vena cava/iliac veins 6.8%, retinal vein 2.7%, splenic vein 1.4%). The following treatment regimens were used in our APS patients: warfarin (71%), aspirin plus subcutaneous heparin (4%), aspirin + warfarin (3.1%), aspirin alone (17%) and the direct oral anticoagulant (DOAC) (5%). Bleeding complications developed in 35(22%) patients. Over a mean follow-up of 9.9±7.0years, recurrence of thromboembolic or obstetric manifestations recurred in 39(24%) and 6(4%) patients, respectively. A total of 29(18%) patients succumbed (median time to death: 6.4 years) and the causes of death were: pneumonia (24%), septicemia (17%), intracranial hemorrhage (14%), myocardial infarction (10%), ischemic stroke (3%), bowel infarct (3%), pulmonary hypertension (6.9%) and sudden death with unknown causes (14%).Conclusion:APS in southern Chinese is relatively uncommon and most cases were associated with SLE. In contrast with the Caucasians, venous thrombosis related to APS is less frequent than arterial thrombosis in Chinese patients. With long-term anticoagulation treatment, the outcome is satisfactory with relatively low rates of recurrence and mortality. Expansion of the sample size to study factors associated with recurrence and mortality by involving more hospitals is in progress.Disclosure of Interests:None declared
Impact of Adopting Population Pharmacokinetics for Tailoring Prophylaxis in Haemophilia A Patients: A Historically Controlled Observational Study
