PT  - JOURNAL ARTICLE
AU  - Mara Taraborelli
AU  - Rossella Reggia
AU  - Francesca Dall’Ara
AU  - Micaela Fredi
AU  - Laura Andreoli
AU  - Maria Gerosa
AU  - Ariela Hoxha
AU  - Laura Massaro
AU  - Marta Tonello
AU  - Nathalie Costedoat-Chalumeau
AU  - Patrice Cacoub
AU  - Franco Franceschini
AU  - Pier Luigi Meroni
AU  - Jean Charles Piette
AU  - Amelia Ruffatti
AU  - Guido Valesini
AU  - Eon Nigel Harris
AU  - Angela Tincani
TI  - Longterm Outcome of Patients with Primary Antiphospholipid Syndrome: A Retrospective Multicenter Study
AID  - 10.3899/jrheum.161364
DP  - 2017 Aug 01
TA  - The Journal of Rheumatology
PG  - 1165--1172
VI  - 44
IP  - 8
4099  - http://www.jrheum.org/content/44/8/1165.short
4100  - http://www.jrheum.org/content/44/8/1165.full
SO  - J Rheumatol2017 Aug 01; 44
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.