Primary anti-phospholipid antibody syndrome (APS): Current concepts

Primary anti-phospholipid syndrome (APS) is a thrombophilic state characterized by recurrent arterial and Venous thrombosis, recurrent pregnancy loss, and the presence of circulating anti-phospholipid antibodies that may be responsible for thrombophilia and pregnancy morbidity. Ophthalmologic features are present in 15-88% of the patients with primary APS, thus ophthalmologists are one of the first physicians to whom the patient will present, An accurate diagnosis may save the patient from recurrent, potentially , life-threatening thrombosis. In the U.S.A., an estimated 35,000 new cases of APS-related venous thrombosis Occur each bear in a population that is several decades younger than the patient population typically affected by thrombosis. Clinical features, such as chorea, transverse myelitis, cardiac valvular lesions. and accelerated atherosclerosis, are hypothesized to be due to a direct tissue-antibody interaction and cannot be explained purely by thrombosis. The use of recently proposed well-defined diagnostic criteria, and better standardization of laboratory, assays for the anti-phospholipid antibodies should help enable epidemliological surveys to establish the prevalence of these antibodies in patients with thrombosis and in the general population. Diagnosis of APS should be considered in all patients with recurrent systemic or ocular thrombosis in the absence of known risk factors. Several well-deigned prospective studies show all increased risk,, of thrombosis in the presence of medium to high antibody, level. With ocular involvement in as mail 88% V IN L IS of APS patients, an ophthalmic assessment should be an integral part of the clinical work-up of any patient With suspected or Confirmed APS. The presence of isolated ocular thromophilia with persistently, elevated anti-phospholipid antibodies or lupus coagulant should confirm die diagnosis of APS. Management of these patients must be a multi-disciplinary effort with either a rheumatologist or a hematologist having the overall responsibility for coordinating treatment and monitoring the patient's immune status and anticoagulation. Treatment of isolated ocular thrombophilia in the presence of moderate to high titers of anti phospholipid antibodies Should be on the same principles as patients with APS to prevent recurrent ocular or cerebral thrombosis. (C) 2002 bv Elsevier Science Inc. Adl rights reserved.