Background Data on the long-term outcome in primary antiphospholipid syndrome (PAPS) patients (pt) are still very limited.
Objectives 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.
Methods 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<0.05 was considered significant.
Results 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<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<0.001),more with hypercholesterolemia (p 0.043),hypertension (p:0.004),cancer (p:0.02);more using steroids (p:0.04),hydroxychloroquine (p<0.001),immunosuppressants (p<0.01),anticoagulants (p:0.003),anti-hypertensive drugs (p<0.001).
Conclusions 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.
Disclosure of Interest None declared