Case Report

Ocular disorders as the prevailing manifestations of antiphospholipid syndrome: a case series

Evangelia Tsironi¹ , Nikolaos Gatselis² , Maria G Kotoula¹ , Kalliopi Zachou^2,3 , Maria Pefkianaki¹ , Fani Zacharaki¹ , Dimitrios Z Chatzoulis¹ and George N Dalekos^2,3

¹  Department of Ophthalmology, Medical School, University of Thessaly, Larissa, 41110, Greece

²  Department of Medicine and Research Lab of Internal Medicine, Medical School, University of Thessaly, Room No 23, 41110 Larissa, Greece

³  Research Group of Investigational Medicine, Institute of Biomedical Research and Technology, Centre for Research and Technology-Thessaly (CE.RE.TE.TH), Larissa, 41110, Greece

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Cases Journal 2009, 2:159doi:10.1186/1757-1626-2-159

Published:	20 October 2009

Abstract

Introduction

Antiphospholipid syndrome is an autoimmune disorder characterized by either a history of vascular thrombosis (one or more clinical episodes of arterial, venous, or small vessel thrombosis in any tissue or organ) or pregnancy morbidity in association with the presence of antiphospholipid antibodies. The systemic features of the syndrome are characterized by large variability depending on the affected organ(s). Among them, neurological and behavioural disturbances, dermatological features as livedo reticularis and renal, ocular, liver or valvular heart manifestations have been reported in antiphospholipid syndrome patients. However, studies on the frequency and clinical presentation of the ocular manifestations as the prevailing (first) sign of antiphospholipid syndrome in patients suffering from "unexplained" ocular disease are missing. Herein, we present three cases suffering from unexplained ocular disease as first manifestation of antiphospholipid syndrome.

Case presentation

All the three patients were referred to our department because of unexplained ocular features from the anterior or posterior segment and unexplained neuro-ophthalmologic symptoms. The first patient had bilateral retinal occlusive disease, the second and the third patient had unilateral nonarteritic anterior ischemic optic neuropathy with macular oedema. Moderate to high levels of antiphospholipid antibodies were detected in all of them at baseline as well as 6 to 12 weeks after initial testing confirming the presence of antiphospholipid antibodies. Anticoagulant treatment with acenocoumarol was instituted resulting in stabilization and/or improvement of ocular signs in all of them.

Conclusion

Due to the important diagnostic and therapeutic implications of antiphospholipid syndrome, the possibility of ocular features as the first clinical manifestation of antiphospholipid syndrome should be kept in mind of the physicians particularly in patients with no evident risk factors for ocular disease. In this case, prompt anticoagulant treatment and close follow-up seem to be essential for vision salvation and stabilization.