1. Longterm Outcome of Patients with Primary Antiphospholipid Syndrome: A Retrospective Multicenter Study.
- Author
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Taraborelli M, Reggia R, Dall'Ara F, Fredi M, Andreoli L, Gerosa M, Hoxha A, Massaro L, Tonello M, Costedoat-Chalumeau N, Cacoub P, Franceschini F, Meroni PL, Piette JC, Ruffatti A, Valesini G, Harris EN, and Tincani A
- Subjects
- Adult, Antiphospholipid Syndrome complications, Disease Progression, Female, Follow-Up Studies, Humans, Male, Middle Aged, Retrospective Studies, Treatment Outcome, Young Adult, Anticoagulants therapeutic use, Antiphospholipid Syndrome drug therapy, Thrombosis etiology
- Abstract
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.
- Published
- 2017
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