Abstract
Background Juvenile onset Systemic Lupus Erythematosus (jSLE) is a rare multisystem autoimmune disease with variable clinical features and severity. jSLE is a common cause of secondary antiphospholipid syndrome (APS). Our aim is to describe the clinical characteristics, treatment, and outcomes of jSLE with secondary APS in our cohort.
Methods A retrospective descriptive study of all jSLE patients with APS from January 2013- December 2022 (10 years) seen at the Paediatric Rheumatology Unit, Selayang Hospital, Malaysia. Clinical, laboratory, treatment and outcome data were collated from hospital electronic medical records.
Results We identified 11 patients with total of 14 thrombotic events. The mean age of SLE diagnosis: 11.0 years (8.9–14.9) and mean age of first APS diagnosis: 13.9 years (10.2–21.3). The majority were Malays (36%) and Indians (36%) and the female-to-male ratio was 10:1. Most were venous thrombosis (79%) predominantly lower limb deep vein thrombosis (73%). Arterial thrombosis occurred in 14%, affecting the internal carotid and central retinal artery; while one (7%) had a mixed arterial (splenic infarct) and venous thrombosis (bilateral pulmonary artery). Of those tested, positive antiphospholipid antibodies include lupus anticoagulant (80%), beta 2-glycoprotein (56%) and anticardiolipin antibody (22%). Risk factors included obesity (4/11), overweight (4/11), hypertension (3/11) and high cholesterol (3/11). The mean SLEDAI score was 7 at time of thrombosis (range 0–22). Eight patients had a prior diagnosis of jSLE and were receiving Prednisolone (100%), Hydroxychloroquine (75%), Mycophenolate mofetil (38%), Azathioprine (25%) or Cyclophosphamide (13%). All received heparin during the acute phase (10/11 sc Enoxaparin) followed by warfarin. One required surgical intervention (transcatheter embolectomy and inferior vena cava filter insertion). All patients survived with complete clinical resolution of thrombotic episodes.
Conclusions Antiphospholipid-associated thrombosis in juvenile SLE is predominantly venous, sometimes recurrent and occurs during active SLE disease. Unlike adults, traditional risk factors are uncommon in jSLE and the overall prognosis is good.
