Morbidity and mortality in the antiphospholipid syndrome

PURPOSE OF REVIEW: Thrombotic complications are the most common cause of death and serious morbidity in antiphospholipid syndrome (APS). Functional prognosis is poor in a significant number of patients with APS. Moreover, catastrophic APS is an unusual form of presentation that represents less than 1% of APS cases reported but with a high mortality rate (around 50%). There is a growing awareness to determine optimal prognostic markers and therapeutic measures to prevent these important complications in the APS. RECENT FINDINGS: Up to 20% of patients from a European cohort of APS cases developed APS-related manifestations during a 5-year study period. Specifically, recurrent thrombotic events appeared in 16% of patients, stroke being the most common manifestation. The mortality rate during this period was 5%. In addition to severe thrombotic events (myocardial infarction, stroke), infections and hemorrhages accounted for one third of deaths. Regarding catastrophic APS, the mortality rate was 50% in the earliest published series. However, a recent report described a drop in the mortality rate by some 20% because of the use of full anticoagulation, corticosteroids, plasma exchanges, and intravenous immunoglobulins. Cerebral involvement is the main cause of death, being present in one third of patients. SUMMARY: Patients with APS develop significant morbidity and mortality despite current therapy.