Background While acute manifestations related to APS are well known and their treatment is quite standardized, informations on the long-term outcome in affected patients are still very limited 1–3, even though the disease has been known for at least 30 years.
Objectives Primary objective was to assess the prevalence of thrombotic and obstetrical recurrences, severe complications, organ damage (and related risk factors) and mortality in PAPS with long-standing disease. We also compared systemic involvement, serological alterations, cardiovascular risk factors and therapy between the onset and the follow-up.
Methods Medical records of patients with PAPS regularly and prospectively followed in our center for at least 15 years were retrospectively reviewed. Fisher's exact test for categorical variables was used. P values <0.05 were considered significant.
Results Thirty-five Caucasian patients (33 female and 2 male) with diagnosis of PAPS (Miyakis et al 2006) followed from 1984 to 2013 with a mean age at onset of 32 years (SD 8.17) and a median follow-up of 20.5 years (range 15-30) were included in this analysis. A thrombotic event appeared in 28% of patients during follow up (venous in 50%, arterial in 40% and both in 10%). Nineteen patients (57%) had a total of 28 pregnancies, that was successful in 75% of cases. No CAPS were observed during follow-up. Hemorrhagic, infective, neoplastic events were recorded in 34%, 6% and 9% of patients respectively. Organ damage was present in 20% of patients at the end of follow-up (17% neurological deficit and 3% chronic kidney failure)and was statistically significantly associated to a previous thrombotic history (p:0.027) and in particular to a previous arterial thrombosis (p<0.001). One patient (3%) died for sepsis due to chronic bowel ischemia. No statistically significant variations were observed between the onset and follow-up for systemic involvement, serological alterations but for cardiovascular risk factors (less estroprogestinic use p: 0.002) and therapy (more Hydroxicloroquine p<0.001; more Immunosuppressant p: 0.055; more anti-hypertensive p:0.006).
Conclusions Despite therapy, a high proportion of patients experiences new thrombotic events and severe complications, while pregnancy outcome seems to be quite good. Organ damage progresses in a significant proportion of patients and it is associated with previous arterial events. Unluckily PAPS can be mortal. A strict control of cardiovascular risk factors and effective treatment are needed to improve the long term outcome.
Erkan D, Yazici Y, Sobel R, Lockshin MD. Primary antiphospholipid syndrome: functional outcome after 10 years. J Rheumatol. 2000;27:2817-21
Cervera R, Khamashta MA, Shoenfeld Y, Camps MT, Jacobsen S, Kiss E, et al. Morbidity and mortality in the Antiphospholipid syndrome during a 5-year period: A multicenter prospective study of 1000 patients. Ann Rheum Dis 2009;68:1428-32
Grika EP, Ziakas PD, Zintzaras E, Moutsopoulos HM, Vlachoyiannopoulos PG. Morbidity, mortality, and organ damage in patients with Antiphospholipid syndrome. J Rheumatol. 2012; 39:516-23.
Disclosure of Interest None declared