Thrombotic recurrence, organ damage likely in patients with primary antiphospholipid syndrome
A large number of patients face new thrombotic events and organ damage in spite of therapy, while evolution toward connective tissue disease (CTD) is infrequent, according to a recent study.
Researchers retrospectively reviewed medical records of patients with primary antiphospholipid syndrome (PAPS) followed in six centres for ≥15 years to assess the long-term frequency of thrombotic recurrences, obstetrical complications, organ damage, severe comorbidities and evolution toward CTD in PAPS.
A total of 115 patients were analysed (mean age at diagnosis 33 years; 88 percent women) and followed between 1983 and 2014.
There were 50 patients (44 percent) who had at least a thrombotic event for a total of 75 events and an annual incidence of 3.5 percent during a median follow-up of 18 years (range, 15 to 30 years). Those with previous thrombotic history experienced more frequent thromboses (p=0.002).
Six patients (5 percent) had a catastrophic antiphospholipid syndrome. Oral anticoagulation did not appear to protect patients with thrombotic onset against recurrences (p=0.26).
Of the 87 pregnancies in 52 women, 78 percent were successful. Functional damage occurred in 29 percent of patients. Patients with a thrombotic history (p=0.004) were likely to accrue damage, which was also significantly associated with arterial events (p<0.001), especially stroke, but not with demographics, serology or treatment.
A total of 14 major bleeding episodes were recorded in 18 patients who received anticoagulants. Severe infections affected six patients (5 percent), with one fatality. Eight patients (7 percent) had a diagnosis of a solid cancer.
In total, 16 patients (14 percent) developed an autoimmune disease and 13 (11 percent) a full-blown picture of CTD, according to researchers.