%0 Journal Article %A Mara Taraborelli %A Rossella Reggia %A Francesca Dall’Ara %A Micaela Fredi %A Laura Andreoli %A Maria Gerosa %A Ariela Hoxha %A Laura Massaro %A Marta Tonello %A Nathalie Costedoat-Chalumeau %A Patrice Cacoub %A Franco Franceschini %A Pier Luigi Meroni %A Jean Charles Piette %A Amelia Ruffatti %A Guido Valesini %A Eon Nigel Harris %A Angela Tincani %T Longterm Outcome of Patients with Primary Antiphospholipid Syndrome: A Retrospective Multicenter Study %D 2017 %R 10.3899/jrheum.161364 %J The Journal of Rheumatology %P jrheum.161364 %X Objective To assess the longterm frequency of thrombotic recurrences, obstetrical complications, organ damage, severe comorbidities, and evolution toward connective tissue disease (CTD) in primary antiphospholipid syndrome (PAPS). Methods Medical records of patients with PAPS followed in 6 centers for ≥ 15 years were retrospectively reviewed. Results One hundred fifteen patients were studied: 88% women, followed between 1983 and 2014 with a mean (± SD) age at diagnosis of 33 (± 10) years. During a median followup of 18 years (range 15–30), 50 patients (44%) had at least a thrombotic event for a total of 75 events and an annual incidence of 3.5%. Thromboses were more frequent in patients with previous thrombotic history (p = 0.002). A catastrophic antiphospholipid syndrome occurred in 6 patients (5%). The use of oral anticoagulants in patients with thrombotic onset did not appear to be protective against recurrences (p = 0.26). Fifty-two women had 87 pregnancies, successful in 78%. Twenty-nine percent of patients accrued functional damage. Damage was significantly associated with a thrombotic history (p = 0.004) and with arterial events (p < 0.001), especially stroke, but not with demographics, serology, or treatment. Twenty-four major bleeding episodes were recorded in 18 patients, all receiving anticoagulants. Severe infections affected 6 patients (5%), with 1 fatality. A solid cancer was diagnosed in 8 patients (7%). Altogether, 16 patients (14%) developed an autoimmune disease and 13 (11%) a full-blown picture of CTD. Conclusion Despite therapy, a high proportion of patients experienced new thrombotic events and organ damage, while evolution toward CTD was infrequent. %U https://www.jrheum.org/content/jrheum/early/2017/05/25/jrheum.161364.full.pdf