Longterm Outcome of Patients with Primary Antiphospholipid Syndrome: A Retrospective Multicenter Study

Mara Taraborelli, Rossella Reggia, Francesca Dall'Ara, Micaela Fredi, Laura Andreoli, Maria Gerosa, Ariela Hoxha, Laura Massaro, Marta Tonello, Nathalie Costedoat-Chalumeau, Patrice Cacoub, Franco Franceschini, Pier Luigi Meroni, Jean Charles Piette, Amelia Ruffatti, Guido Valesini, Eon Nigel Harris, Angela Tincani

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)1165-1172
Number of pages8
JournalJournal of Rheumatology
Issue number8
Publication statusPublished - Aug 2017


  • Journal Article


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