Complex Antiphospholipid Syndrome Successfully Controlled for 17 Years with Personalized Enoxaparin Therapy.

Abstract

Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by the presence of antiphospholipid antibodies and venous and arterial thrombotic events, including obstetric complications. We describe the case of a 56-year-old female diagnosed with APS with triple antibody positivity and multiple disease-associated manifestations, namely recurrent purpuric lesions, adrenal insufficiency due to infarction, acalculous cholecystitis, and three spontaneous abortions. Her follow-up was marked by severe thrombotic and haemorrhagic events, notably splanchnic vein thrombosis and haemorrhagic shock after a renal biopsy, as well as the diagnosis of systemic lupus erythematosus 8 years after the APS diagnosis. Chronic anticoagulation with enoxaparin, with dosage guided by anti-factor Xa activity, resulted in stability without complications over 17 years. This case emphasizes the importance of personalized therapeutic strategies and close monitoring in patients with APS.

Learning points: This patient presented with rare manifestations of antiphospholipid syndrome (APS), such as acalculous cholecystitis, Budd-Chiari syndrome, and Addison's disease, all linked to ischemic events caused by vascular thrombosis. These atypical presentations highlight the varied and severe clinical spectrum of APS, requiring physicians to be vigilant about unusual complications.After multiple severe thrombotic and haemorrhagic events, the patient was successfully managed with long-term enoxaparin, with dose adjusted according to anti-factor Xa activity (which allowed for a significant dose reduction in comparison with the predicted empiric dose).The patient remained event-free for 17 years, reflecting the importance of personalizing anticoagulation therapy.