Reversible blindness with macrophage activation syndrome: an unusual presentation of secondary antiphospholipid syndrome

Abstract

Blindness due to retinal vascular thrombosis is a dreadful complication of antiphospholipid syndrome (APS). The latter may be present in isolation (ie, primary) or may occur secondary to other connective tissue diseases like lupus. Here we present an adolescent girl with bilateral painless loss of vision as a result of central retinal vessel thrombosis due to secondary APS. Her condition was further complicated by the presence of autoimmune haemolytic anaemia and the development of macrophage activation syndrome while being evaluated for her blindness. Prompt treatment with glucocorticoids and anticoagulants could halt the devastating disease process.

Keywords: Haematology (incl blood transfusion); Immunology; Retina; Systemic lupus erythematosus.