Morbidity and mortality in the antiphospholipid syndrome

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.

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.

Patients with APS develop significant morbidity and mortality despite current therapy.