RT Journal Article SR Electronic T1 Longterm Outcome of Patients with Primary Antiphospholipid Syndrome: A Retrospective Multicenter Study JF The Journal of Rheumatology JO J Rheumatol FD The Journal of Rheumatology SP 1165 OP 1172 DO 10.3899/jrheum.161364 VO 44 IS 8 A1 Mara Taraborelli A1 Rossella Reggia A1 Francesca Dall’Ara A1 Micaela Fredi A1 Laura Andreoli A1 Maria Gerosa A1 Ariela Hoxha A1 Laura Massaro A1 Marta Tonello A1 Nathalie Costedoat-Chalumeau A1 Patrice Cacoub A1 Franco Franceschini A1 Pier Luigi Meroni A1 Jean Charles Piette A1 Amelia Ruffatti A1 Guido Valesini A1 Eon Nigel Harris A1 Angela Tincani YR 2017 UL http://www.jrheum.org/content/44/8/1165.abstract AB 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.