Carla Gobbi

First Latin American clinical practice guidelines for the treatment of systemic lupus erythematosus: Latin American Group for the Study of Lupus (GLADEL, Grupo Latino Americano de Estudio del Lupus)–Pan-American League of Associations of Rheumatology (PANLAR)

Systemic lupus erythematosus (SLE), a complex and heterogeneous autoimmune disease, represents a significant challenge for both diagnosis and treatment. Patients with SLE in Latin America face special problems that should be considered when therapeutic guidelines are developed. The objective of the study is to develop clinical practice guidelines for Latin American patients with lupus. Two independent teams (rheumatologists with experience in lupus management and methodologists) had an initial meeting in Panama City, Panama, in April 2016. They selected a list of questions for the clinical problems most commonly seen in Latin American patients with SLE. These were addressed with the best available evidence and summarised in a standardised format following the Grading of Recommendations Assessment, Development and Evaluation approach. All preliminary findings were discussed in a second face-to-face meeting in Washington, DC, in November 2016. As a result, nine organ/system sections are presented with the main findings; an ‘overarching’ treatment approach was added. Special emphasis was made on regional implementation issues. Best pharmacologic options were examined for musculoskeletal, mucocutaneous, kidney, cardiac, pulmonary, neuropsychiatric, haematological manifestations and the antiphospholipid syndrome. The roles of main therapeutic options (ie, glucocorticoids, antimalarials, immunosuppressant agents, therapeutic plasma exchange, belimumab, rituximab, abatacept, low-dose aspirin and anticoagulants) were summarised in each section. In all cases, benefits and harms, certainty of the evidence, values and preferences, feasibility, acceptability and equity issues were considered to produce a recommendation with special focus on ethnic and socioeconomic aspects. Guidelines for Latin American patients with lupus have been developed and could be used in similar settings.

Síndrome de Sjögren primario y embarazo: reporte de 18 casos

Primary Sjögrens syndrome (pSS) is a condition that predominantly affects women. Reports of pregnancy outcome in these patients are limited and contradictory. OBJECTIVE To describe pregnancy characteristics and outcomes and newborn morbidity in women with pSS. MATERIAL AND METHODS We included women with pSS who became pregnant after the onset of the symptoms of the disease. Clinical and serological characteristics, risk factors and previous maternal comorbidities are described. For each pregnancy in a woman with pSS, we recorded pregnancy course and outcome and newborn condition. RESULTS We assessed 11 patients with 18 pregnancies after the onset of pSS symptoms. All of them presented FAN +; 10 anti-Ro / SSA + and 7 anti-La / SSB +. The mean age in years at the onset of symptoms was 24.9 (SD 6.9) and at the time of pregnancy was 30.3 (SD 5.4). Thirteen pregnancies happened before the diagnosis, reporting only one miscarriage. Two preterm births, 1 case of oligohydramnios, 2 of premature membrane rupture and 2 low birthweight babies were reported after the onset of pSS symptoms. There was 1 newborn with congenital atrioventricular block and another with neonatal cutaneous lupus. All the women with pregnancy complications (n=6) had anti-Ro/SSA antibodies. CONCLUSIONS Almost half of the pregnancies assessed in women with pSS were associated with complications not attributable to factors other than the disease.

AB0485 Primary antiphospholipid syndrome: maternal and fetal outcome

Background Antiphospholipid antibodies (APLAs) have been associated with pregnancy loss and other obstetric complications, such as pre-eclampsia, fetal growth restriction and preterm delivery. Objectives The purpose of this work is to evaluate maternal and fetal pregnancy outcome in Primary Antiphospholipid Syndrome (PAPS). Methods We retrospectively studied PAPS patients according to Sydney Criteria who are attended to Materno Neonatal Hospital during the last 8 years. We evaluated demographic, obstetric and thrombotic clinical data. Maternal complications were evaluated: Preeclampsia, HELLP, Gestational Diabetes, Premature rupture of fetal membranes, arterial and venous thrombosis, mortality, the way of end of pregnancy, and others. Fetal outcome was evaluated as live birth, gestational age and weight at birth. Results 96 pregnancies in 68 patients were included. Maternal mean age was 30,75 years old, 84% were from Cόrdoba city, 70.5% did not have health insurance, and they have mean previous pregnancies of 4 with 1 live birth. Maternal complications were: Pre eclampsia in 12 patients (12.5%), Preterm delivery in 6 patients (6.25%), Premature rupture of fetal membranes in 8 (8.33%), Gestational Diabetes in 7 (7.29%), Arterial Thrombosis in 2 (2.08%), Venous thrombosis in 3 (3.12%). 33,69% have normal labour and 66,33% cesarean section. 86% of patients have live birth with mean gestational age of 36 weeks with mean weight at birth of 2.558 g and 73% of patients according to gestational age.Table 1. Maternal Complications Pre Eclampsia 12 (12,5%) Pre term delivery 6 (6,2%) Premature rupture of fetal membranes 8 (8,2%) Gestacional Diabetes 7 (7,3%) Arterial Thrombosis 2 (2%) Venous Thrombosis 3 (3,1%) Notch 10 (10%) Mortality 0 Conclusions PAPS pregnancies patients had a good maternal and fetal outcome in this study. References Bertolaccini ML, Sanna G2 Recent advances in understanding antiphospholipid syndrome. F1000Res. 2016 Dec 22;5:2908. doi: 10.12688/f1000research.9717.1. eCollection 2016. Pelusa HF, Pezzarini E, Basiglio CL, Musuruana J, Bearzotti M, Svetaz MJ, Daniele SM, Bottai H, Arriaga SM. Antiphospholipid and antioangiogenic activity in females with recurrent miscarriage and antiphospholipid syndrome.Ann Clin Biochem. 2016 Sep 16. pii: 0004563216672248. [Epub ahead of print. Moroni G, Doria A, Giglio E, Tani C, Zen M, Strigini F, Zaina B, Tincani A, de Liso F, Matinato C, Grossi C, Gatto M, Castellana P, Limardo M, Meroni PL, Messa P, Ravani P, Mosca M. Fetal outcome and recommendations of pregnancies in lupus nephritis in the 21st century. A prospective multicenter study.J Autoimmun. 2016 Nov;74:6–12. doi: 10.1016/j.jaut.2016.07.010. Epub 2016 Aug 2. Acknowledgements We are grateful whit Secyt subsidy UNC. Disclosure of Interest None declared

AB0524 Immunosuppressive Treatment in Patients with Primary SjÖgren Syndrome (PSS)

Background There is not enough evidence either to support the use of immunosuppressive drugs in pSS or for which specific clinical features they would be indicated. Objectives To evaluate the most frequently immunosuppressive drugs prescribed in pSS and to describe the clinical features that determined the indication,in a multicentric cohort of Argentinean patients with pSS. To analyze the clinical characteristics of patients with immunosuppressive therapy in comparison to patients that had not received it. Methods We analyzed subjects with pSS included in the data-base of the Argentine study group for SS (GESSAR). We considered the use of methotrexate, azathioprine, cyclophosphamide, leflunomide, mycophenolate, rituximab, belimumab and/or prednisone (>20mg/day). Results From 431 patients, 72 (16.7%) had received immunosuppressive therapy. Female: 92%; mean age: 59 years; mean time of the disease: 5 years. Immunomodulatory treatment with hydroxychloroquine in 60%. The drugs most frequently used were:prednisone (40.3%), methotrexate (34.7%), azathioprine (23.6%), cyclophosphamide (9.7%), rituximab (5.6%), mycophenolate (4,2%), leflunomide (2.8%), more than one drug (6.9%). The clinical features that determined the indication of therapy were arthritis (50.8%), parotid swelling (28%), purpura (19.7%), peripheral neuropathy (18.3%), pulmonary involvement (13.9%), autoimmune hepatitis (8.33%) and glomerulonephritis (5.1%). When we compared the clinical characteristics of patients with immunosuppressive treatment against patients without treatment, we found differences in age [59.4 vs 53.8; (p<0.01)], disease duration [5 vs 3; (p<0.01)]; low C3 level [27 vs 12,3;(p<0.01)]; arthritis [50,8 vs 29,4;(p<0.01)]; purpura [19.7 vs 5.5;(p<0.01)], pulmonary disease [13.9 vs 6.3;(p:0.02)] and autoimmune hepatitis [62.5 vs 37.5;(p<0.01)]. Multivariate analysis (adjusted for age and years of disease), with immunosuppressive treatment (dependent variable), showed that arthritis (OR:2.8;95%CI:1.5-5; p<0.01), purpura (OR: 4.4; 95%CI: 1.9-10.3; p<0.01) and autoimmune hepatitis (OR: 8.7; 95%CI: 1.9-40.4; p<0.01)] were independently associated with this indication. Conclusions A low number of our patients received treatment with immunosuppressive drugs. The most frequently prescribed drugs were methotrexate and azathioprine. The main clinical features in the immunosuppressant treated group were arthritis, purpura and autoimmune hepatitis. Disclosure of Interest None declared

AB1097 The Impact of Systemic Lupus Erythematosus on Work Productivity: Data from Patients from the Province of Cordoba, Argentina

Background Since SLE tends to occur during the productive years of life, the cost of the disease derives not only from direct health expenditures but also from the impact the disease has on work productivity. Objectives To describe the impact of SLE on work productivity and to assess the factors influencing this outcome measure. Methods We studied 225 patients (1987 ACR criteria), age ≥16 years. Work productivity was assessed with the WPAI:Specific Health Problem for SLE. The WPAI yeilds four scores: % work time missed, % of impairment at work, % of overall work impairment and % of non-work related activity impairment. The relationship between socioeconomic-demographic, self-reported quality of life (as per the Lupus PRO), clinical data [clinical manifestations, diagnostic criteria (1987 ACR criteria), disease activity (SELENA-SLEDAI), damage (SLICC Damage Index), co-morbidities (Charlson Index)] and the four scores of the WPAI was examined with the Man-Whitney U test and Spearmans Rho test. Variables with p≤0.10 in these analyses were then entered in a multivariable linear regression with each score of the WPAI as the dependent variable. Results Patients were predominantly females (89%) and they had a median (IQR) age at diagnosis of 26.0 (16.0) years. Median (IQR) disease duration was 96.0 (144.0) months. Median (IQR) percentage of work time missed was 0.0 (25.0)%, of impairment at work was 10.0 (56.0)%, of overall work impairment 0.0 (56.0)% and of activity impairment 40.0 (60.0)%. Variables significant in both, the univariable and multivariable analyses, are shown in Table below Conclusions While work productivity is not largely affected in this sample of patients, non-work related activities seems to have a much greater impact in SLE. Patients with a higher disease activity and number of co-morbidities and a lower self-perceived quality of life are at higher risk for work productivity impairment Disclosure of Interest None declared