Choroidal involvement in patients with catastrophic anti-phospholipid syndrome: Two case reports

Purpose: To describe choroidal involvement in catastrophic antiphospholipid syndrome (CAPS).

Methods: We report here two cases of bilateral CAPS choroidopathy in two female patients.

Results: Case report 1: A thirty-five-year-old female patient, with history of primary anti-phospholipid syndrome (APS), treated with anticoagulants, presented an acute renal failure following a salpingectomy. She complained of bilateral acute blurred vision. Ophthalmologic evaluation revealed visual acuity (VA) of 5/10, extensive serous retinal (SRD) detachment, areas of hypofluorescence on fluorescein angiography (FA), and non-perfusion areas in the choriocapillaris, on optical coherence tomography angiography (OCT-A), in both eyes. Considering the diagnosis of probable CAPS, the patient received intravenous pulse steroids, plasmapheresis, intravenous anticoagulation and haemodialysis, with favourable evolution. Case report 2: A thirty-three-year-old female patient, with history of systemic lupus erythematosus (SLE) and secondary APS, treated with corticosteroids, immunosuppressive agents and anti-coagulation, presented a myocardiac infarction. She complained of bilateral acute blurred vision. Ophthalmologic evaluation revealed VA of 1/10 in the RE and 6/10 in LE, bilateral extensive SRD, leakage points on FA and non-perfusion areas in the choriocapillaris on OCT-A. Criteria of probable CAPS were fulfilled. Treatment with intravenous pulse steroids, anticoagulation and reanimation modalities allowed VA improvement. Alveolar haemorrhage and cardiogenic shock led to fatal evolution.

Conclusion: Our case reports highlight the importance of early diagnosis and ophthalmic evaluation in CAPS. Multidisciplinary approach and rapid initiation of effective treatment, based on corticosteroids, anticoagulation and plasmapheresis, allow better vital and visual prognosis.