SAT0408 Long Term Outcome of Primary Antiphospholipid Syndrome Patients: A Multicenter Study
Authors: M. Taraborelli, F. Dall'Ara, R. Reggia, M. Fredi, M. Gerosa, L. Massaro, M. Tonello, P. Cacoub, N. Costedoat-Chalumeau, F. Franceschini, P.L. Meroni, J.C. Piette, A. Ruffatti, G. Valesini, A. Tincani
Journal: Annals of the Rheumatic Diseases (2015)

<h3>Background</h3> Data on the long-term outcome in primary antiphospholipid syndrome (PAPS) patients (pt) are still very limited. <h3>Objectives</h3> To assess the prevalence of recurrences,organ damage,severe comorbidities (infections, hemorrhages,cancers),mortality,evolution in connective tissue disease (CTD), in long-standing PAPS.To compare non criteria manifestations,serological alterations,cardiovascular risk factors and therapy between onset and follow-up. <h3>Methods</h3> Medical records of PAPS pt followed in 6 centers for ≥15 years were retrospectively reviewed.Chi square for categorical and Student t test for continuous variables were used.P&lt;0.05 was considered significant. <h3>Results</h3> One hundred and sixteen pt (88% females) with PAPS followed between 1983 and 2014 with mean age at diagnosis of 33 (±10) and mean follow-up of 19 years (±5) were studied.Fifty-one pt (44%) had at least a thrombotic event during follow-up. Thromboses were more frequent in patients with previous thrombotic history (p:0.003,OR:4.68, 95%CI:1.6-14.3) and anticoagulant treatment was not protective against recurrences (p:not significant).Six pt (5%) had a catastrophic event. Fifty-two women had 87 pregnancies, that were successful in 76% of cases. Thirty-one (27%) pt had organ damage (permanent loss of function) in at least one system (15% neurological,3% renal,5% cardiac,4% ocular, 1% peripheral vascular,1% osteoarticular,1% gastrointestinal).Damage was significantly associated to a thrombotic history (p:0.008,OR:11.8,95%CI:1.6-245.3) and to arterial events (p&lt;0.001,OR:17.1,95%CI:4.4-77.4),but not to any demographical, serological or therapeutical variable. An anatomical damage (documented ischemic lesion) was present in 54% of pt (n=63).Twenty-four major bleeding episodes were recorded in 18 pt all on anticoagulants.Severe infections (4 bacterial,2 viral) affected 6 pt (5%).A cancer (solid in 100%) was diagnosed in 8 pt (7%) at a mean age of 51 years (±6).One patient (1%) with a chronic bowel ischemia died for sepsis.Fourteen pt (12%) developed a CTD (7 Systemic Lupus Erythematosus,2 Sjogren, 5 Undifferentiated CTD).Compared to diagnosis at the end of the follow up we observed: less patients with anti-cardiolipin IgG (p:0.014) but more with antinuclear antibodies (p:0.01) and C4 reduction (p:0.025);less using estroprogestinics (p&lt;0.001),more with hypercholesterolemia (p 0.043),hypertension (p:0.004),cancer (p:0.02);more using steroids (p:0.04),hydroxychloroquine (p&lt;0.001),immunosuppressants (p&lt;0.01),anticoagulants (p:0.003),anti-hypertensive drugs (p&lt;0.001). <h3>Conclusions</h3> Despite therapy, a high proportion of pt experienced new thrombotic events,while pregnancy outcome was significantly improved.Organ damage developed in a significant proportion of pt and was associated with arterial events. Evolution in CTD was not common but occurred during follow-up.PAPS is a disease of young people characterized by relevant morbidity and premature mortality. <h3>Disclosure of Interest</h3> None declared