The antiphospholipid syndrome - often overlooked cause of vascular occlusions?

The antiphospholipid syndrome (APS) was fully recognized as a clinical entity in the early 1980s. Still, more than 30 years later, the epidemiology of APS is not well described, and furthermore, APS remains a challenge in terms of both diagnostic issues and clinical praxis involving a wide range of specialties. To date, there are no diagnostic criteria for APS. The present classification criteria rely on a combination of clinical manifestations and persistently positive tests for antiphospholipid antibodies (aPL). Clinical symptoms comprise vascular thrombosis, which can affect any vascular bed, including venous, microvascular and arterial vessels, and a set of pregnancy morbidities including early and late miscarriages, foetal death and preeclampsia. APS is more frequent among patients with other autoimmune diseases, and it is especially common in systemic lupus erythematosus (SLE). Importantly, APS symptoms can present in almost any medical specialty, but general knowledge and most previous clinical studies have essentially been confined to haematology, rheumatology and obstetrics/gynaecology. However, recent data demonstrate a relatively high prevalence of aPL also in patients from the general population who suffer from vascular occlusions or pregnancy complications. It is important that these patients are recognized by the general health care since APS is a treatable condition. This review aims to summarize the present knowledge on the history, pathogenesis, clinical manifestations and treatment of APS in order to urge a wide range of clinicians to consider comprehensive assessment of all patients where the diagnosis APS may be conceivable.