Retinal manifestations in catastrophic antiphospholipid syndrome

Abstract

AbstractPurpose We present a case report with an ocular vascular event and multiple organ thrombosis and dysfunction in the presence of antiphospholipid antibodies.Methods We report a case of a 79 years old female related visual loss in left eye. She had a venous occlusion and vitreous haemorrhage. In two weeks presented a maculopapular, bullous skin lesions on the legs, rapidly followed by widespread cutaneous necrosis and skin ulceration on all the limbs showing the tendons tissues of the heels. Later, she had an extension of thrombotic events to other vessels in the body. With medical treatment, there was initially a small functional improvement and then a general degradation in 10 weeks, followed by the death of the patient secondary to sever pulmonary emboli in the intensive care unit.Results It was found histopathologic evidence of multiple small‐vessel occlusions, and laboratory confirmation of the presence of antiphospholipid and anticardiolipin antibodies, in high title. It was refractory to anticoagulation, steroids and immunomodulation treatmentConclusion Catastrophic antiphospholipid syndrome is an autoimmune disorder characterized by a rapidly progressive life‐threating disease. Catastrophic occurs when three or more organ systems are affected by thromboses in a very short time less than a week. Roughly 50% of these patients have systemic lupus erythematosus. The overall mortality of this desvasting syndrome is about one‐half.