AB0595 Antiphospholipid syndrome (HUGHES SYNDROME) is a disease with protean faces: multidisciplinary approaches on serbian cohort of aps patients
Authors: L. Stojanovich, A. Djokovic, N. Stanisavljevic, G. Bogdanovic, M. Zdravkovic
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterised by thrombophilic state and circulating antiphospholipid antibodies (aPL) including anti beta2-GPI. <h3>Objectives</h3> Since than it became one of the most systemic conditions. In the last three and half decades, a variety of clinical manifestations involving almost all organs and tissues (cardiac, pulmonary, neurological, renal, cutaneous, hematologic, gastrointestinal, ocular, skeletal and endocrinology), have been described associated with antiphospholipid antibodies (aPL). <h3>Methods</h3> Our study comprises a total of 608 patients: 420 primary APS (PAPS) patients and 188 SLE patients with secondary APS (SAPS). aPL analysis included detection of aCL, aß2GPI, and LA. <h3>Results</h3> Thrombosis was diagnosed in 46.5% patients, with higher prevalence in PAPS compared to SAPS patients: 51.2% and 38.3%, respectively, p=0.045. Pseudoinfective endocarditis was observed in 12.8% secondary APS patients and 3.1% in primary APS patients (p=0.004). 30% of the patients with high levels of aCL IgG antibodies had valve thickening and dysfunction, as compared to 4.1% without valve abnormalities (p=0.002). Presence of ß2GPI IgG was significantly related to stroke, and overall ß2GPI (IgG and IgM) positivity was significantly related to TIA in SAPS patients. Valvular manifestations were significantly related to TIA in both groups of patients and were independent risk factors for TIA in PAPS (OR 3.790 CI 1.597–8.998 p=0.003: table 2). In PAPS, epilepsy correlated with ß<sub>2</sub>GPI-IgM, migraine with aCL-IgM, thrombocytopenia with aCL-IgM, aCL-IgG, anti ß<sub>2</sub>GPI-IgG and LA. Livedo reticularis was more prominent in PAPS with high levels of aCL-IgG. Skin ulcerations were more prevalent in aCL-IgM positive SAPS patients and epilepsy more frequently had high levels of anti ß<sub>2</sub>GPI-IgG in SAPS. <h3>Conclusions</h3> In this cross-section analysis of a large cohort of APS patients we analysed that APS patients can be presented with a wide variety of thrombotic and nonthrombotic manifestations. The key the success is multidisciplinary approach in all time of patient's life. Antiphospholipid syndrome is really a disease with protean faces. <h3>References</h3> [1] Hughes GR. The antiphospholipid syndrome: ten years on. Lancet1993; 342:341–4. [2] Ardalan MR, Vahedi A. Antiphospholipid syndrome: A disease of protean face. J Nephropathology 2013; 2(1):81–4. [3] . Stojanovich L, Markovic O, Marisavljevic D, at all. Influence of antiphospholipid antibody levels and type on thrombotic manifestations: results from the Serbian National cohort study. Lupus 2011; 0: 1–8. [4] Stojanovich L, Kontic M, Djokovic A, et al. Association between systemic non-criteria APS manifestations and antibody type and level: results from the Serbian national cohort study. Clin Exp Rheumatol. 2013; 31(2):234–42. <h3>Acknowledgements</h3> Acknowledgement: Funding: This work was supported by research grant number 175041, and TR 32040 for 2011–2018, issued by the Ministry of Science of the Republic of Serbia. <h3>Disclosure of Interest</h3> None declared