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Background Antiphospholipid syndrome (APS) is an autoimmune disease characterized mainly by thrombosis and/or pregnancy morbidity in the presence of antiphospholipid (aPL) antibodies. Significant mortality and morbidity has been reported in patients with APS. Objectives To characterize a single center cohort of patients with APS (including non-criteria manifestations) and to assess their survival rates, causes of mortality and associations with disease characteristics. Methods Records of 240 patients with APS were studied. Demographic characteristics, cumulative clinical and laboratory features, autoantibody profiles were retrieved from the existing database. Unattending patients were contacted by telephone and were searched on the national death information system (NDIS). Patients who were non-responsive to telephone calls, not seen in the outpatient clinic within the last 6 months and could not be traced on the NDIS were considered lost-to follow-up. Results There is a total of 240 patients in this cohort 118 of whom had primary APS and 122 had APS associated with SLE. 83% of the patients were female. The mean age at diagnosis was 41±12, duration of disease and follow-up were 110±85 and 92±82 months respectively. Of 240 patients, 65% had vascular thrombosis (VT), 24% had pregnancy morbidity (PM), 10% had both VT and PM and 3 had catastrophic antiphospholipid syndrome. Overall 29% patients had thrombotic risk factors among which smoking followed by homozygous factor V Leiden mutation were the most prevalent. Of patients with VT 49% had arterial (a), 38% had venous (v) and 13% had both a and v thrombosis. 95 patients experienced a single thrombotic episode whilst 62 had more than once, most commonly v followed by a thrombosis. In the PM group, there were 31 patients with fetal deaths beyond 10 weeks, 39 with 3 or more abortions before 10 weeks, 18 with premature labour and 14 with pre-eclampsia. Overall, 46% of patients had aCL IgG, 36% had IgM and LA was present in 54%. Anti-β2GPI was tested in 117 patients and 38% were positive for IgG and 25% for IgM. Of the non-criteria manifestations of APS, the most common presentation was heart valve lesions followed by thrombocytopenia. In total there were 25 deaths and 19 patients were lost to follow-up. Survival rates were 94, 86, 78 and 71% at 5, 10, 15 and 20 years respectively. Comparison of survival between patients with VT and PM displayed a marked reduction in the VT group (p=0.04) whilst primary and lupus associated APS patients had similar rates. Causes of death could be identified in 19 patients. Myocardial infarction, alveolar hemorrhage and pulmonary hypertension were the leading causes of death. Mortality was significantly associated with LA positivity (p=0.02). Conclusions In this cohort, the most prevalent manifestation of APS was arterial thrombosis. Long-term follow up revealed that patients with APS had significant mortality and APS-related manifestations dominated the causes. Thrombotic patients had a reduced survival relative to PM patients. No single aPL test was sufficiently sensitive in the evaluation of APS. LA positivity was a risk factor for reduced survival. Disclosure of Interest None declared

Background Takayasu’s arteritis (TA) is a large vessel vasculitis of the young women in their reproductive age. It’s been previously reported as having conflicting results on the pregnancy outcome in TA. Objectives Herein, we reported the pregnancy outcome and the effect of the pregnancy on the disease course in a well established TA cohort. Methods TA patients diagnosed by ACR criteria and had the history of pregnancy were included into the study. Clinical and laboratory findings including serum ESR and CRP levels were evaluated both retrospectively and prospectively. Pregnancy and newborn outcome consists of the history of abortus, delivery mode, prematurity, intrauterin growth retardation, preeclampsia and eclampsia that were obtained from medical records. Data were noted into a predefined protocol. Disease activity and damage evaluated by Kerr criteria and VDI and concomittant immunosuppressives were recorded within the 3rd trimester and 6 months of pre and postpartum period in a subgroup prospectively followed-up. Pre-diagnosis (pre-d) and post-diagnosis (post-d) pregnancies were compared according to the maternal and fetal outcome. Results Eighty-two pregnancies in 34 patients were assessed in TA cohort of 103 patients. Only 10% of all pregnancies were planned. Mean (median) age of 34 patients at pregnancy period were 24,5±6,6 (22). Subclavian (85%), carotis (44%) and pulmonary arteries (26%) were the most frequently involved vessels. Hypertension (47%), pulselessness (%34), mild pulmonary hypertension (18%) related to valvular disease (26%) were the prominent clinical features. Comparison of fetomaternal prognosis between the pre-d (63 in 24 TA) and post-d (19 in 15 TA) pregnancies demonstrated that hypertension (3% vs 16%), preeclampsia (0% vs 10%), prematurity (2% vs 10%) and Cesarean section (CS) (9% vs 37%) were significantly high in post-d pregnancies. Eight patients (of 4 newly diagnosed) prospectively followed-up during their pregnancies. Glucocorticoid (GC) (4 pts) and azathioprin (2 pts) were maintained after taking the patients’ consents. Four patients had flare during the 3rd trimester. Two of 4 patients had taken 10 mg/d GC and other 2 patients denied to be treated. The patient had renal artery involvement complicated with preeclampsia and the other experienced accelerated hypertension. Five TA patients underwent Ceaserean section mainly because of the probable risk of cerebral hypoperfusion during the delivery. One TA patient who was exposed to infliximab during the conception, has since been followed-up succesfully at the 3rd trimester under the low dose of GC. There were no obvious fetomaternal abnormality in TA cohort. No flare was observed during the 6th month of postpartum period. VDI scores did not increase in all patients. Conclusions Majority of the pregnancies were found as successfull in a well established TA cohort. Higher rates of preeclampsia, prematurity and CS in post-d pregnancies were attributed to the exposure of GC treatment and physicians’ bias for delivery route. CS and prematurity were found as similar with our healthy population rates. Flares did not cause damage accrual in a short postpartum follow-up period. Flare has to be differentiated from the aberrant physiological changes and acute phase increase during the pregnancy. Close monitorization of arterial tension could be helpful for maintaining succesful pregnancy Disclosure of Interest None Declared

Objectives To determine the association of nailfold videocapillaroscopy(NVC)findings and telangiectasia score with DU history and severity of peripheral vascular involvement(PVI) in Systemic Sclerosis(SSc). Methods Fifty-nine SSc patients fulfilling Leroy and Medsger criteria were evaluated including Telangiectasia Score(TS)(Shah AA et al. J Rheumatol 2010), Modifiye Rodnan Skin Score(MRSS), Valentine Activity Scale(VAS) and Medsger Severity Scale(SS). Qualitative(early, active and late patterns) and semiquantitative assessments [capillary number(CN), irregularly enlarged capillaries(IEC), giant capillaries(GC), capillary ramifications(CR), microhaemorrhages(H), capillary array disorganisation(CAD) and microangiopathy evolution score(MES)]. was performed by NVC(Sulli et al. Ann Rheum Dis 2008) Results The mean age of patients was 45.6 and 91,5% were females. The mean duration of Raynaud’s(RS), non-Raynaud symptoms(NRS), skin involvement(SI)(year) were 6.1±6.5, 3.1±2.0, 3.0±2.0 respectively. Of the patients 20(34%) were diffuse cutaneous,35(59%) were limited and 4(7%) were sine-scleroderma;13(22%) were anti-centromere(+) and 29(49%) were anti-Scl70(+). DU history (DU+) was present in 27(46%) and telangiectases were present in 34(58%). When we compare DU- and DU + groups, the mean score of CN was 1.4±0.7 vs 2.0±0.5*(p Conclusions DU history and severe PVI in SSc was associated with capillary loss and microangiopathy. ‘Early’ NVC pattern was very rare in patients with DU history and was not found in severe PVI. Severe PVI in males was more frequent than females. Telangiectasia scores were not found to be related to PVI. NVC may be a helpful method in the assessment of SSc patients for PVI prognosis, warranting prospective studies. Disclosure of Interest None Declared