Background/Objective: Data on IgG4-related disease (IgG4-RD) come almost exclusively from cohorts from Asia, Europe, and North America. We conducted this study to describe the clinical presentation, phenotype distribution, and association with sex, ethnicity, and serological markers in a large cohort of Latin American patients with IgG4-RD. Methods:We performed a multicenter medical records review study including 184 Latin American IgG4-RD patients. We assigned patients to clinical phenotypes: group 1 (pancreato-hepato-biliary), group 2 (retroperitoneal/ aortic), group 3 (head and neck-limited), group 4 (Mikulicz/systemic), and group 5 (undefined). We focused the analysis on how sex, ethnicity, and clinical phenotype may influence the clinical and serological presentation. Results:The mean age was 50.8 ± 15 years. Men and women were equally affected (52.2% vs 48.8%). Fifty-four patients (29.3%) were assigned to group 1, 21 (11.4%) to group 2, 57 (30.9%) to group 3, 32 (17.4%) to group 4, and 20 (10.8%) to group 5. Male sex was associated with biliary tract (odds ratio [OR], 3.4; 95% confidence interval [CI], 1.36-8.26), kidney (OR, 3.4; 95% CI, 1.28-9.25), and retroperitoneal involvement (OR, 5.3; 95% CI,. Amerindian patients presented more frequently with atopy history and gallbladder involvement. Group 3 had a female predominance.Conclusions: Latin American patients with IgG4-RD were younger, and men and women were equally affected compared with White and Asian cohorts. They belonged more commonly to group 1 and group 3. Retroperitoneal and aortic involvement was infrequent. Clinical and serological features differed according to sex, ethnicity, and clinical phenotype.

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BackgroundA collaborative EULAR/ACR Project has developed new criteria for inflammatory myopathies(IM) and their subgoups1 ObjectivesTo analyse agreement between the 2017 IM classification criteria and the Bohan and Peter(BP) criteria in REMICAM cohort2 MethodsAll patients were included. New criteria were applied to obtain classification as: possible(Pos), probable(Pro) and definitive(Def) IM, and subclassification in 6 subgroups: polymyositis(PM), dermatomyositis(DM), juvenile DM(JDM), amiopathic DM(ADM), inclusion body myositis(IBM) and juvenile myositis(JM). The 7 subgroups in REMICAM were harmonised to fit the 6 subgroups of the 2017 criteria. Agreement between 2017 and BP criteria was analysed in classification/subclassification, calculating the weighted kappa value (k). Subanalysis including only patients with available data on the muscle strength items required for the 2017 criteria, and in those having also muscle biopsy data, were conducted.ResultsFrom 479 REMICAM patients, 477 (99.6%), fulfilled BP criteria (5.9%Pos, 26.8%Pro, 67.4%Def) and 431 (89.9%) 2017 criteria (2.5%Pos, 21.8%Pro, 65.7Def). Global agreement between both criteria was 89.5%. Agreement between subtypes (Pos, Pro, Def) was low (k=0.15). When 399 patients with muscle strength data, and 243 with muscle biopsy data were analysed, results were similar (k=0.17). Disagreement was mainly seen in Pos/Pro subtypes with BP criteria, since 60% classified as Def when the 2017 criteria were applied. Agreement in the different subgroups of IM (PM, DM, JDM, ADM, IBM, JM) between both criteria was very high (k=0.94).ConclusionsThe new 2017 EULAR/ACR criteria for IM classification show good agreement with BP criteria in the REMICAM cohort. New criteria classify 60% of Pos/Pro patients by BP criteria, as Def, and show very high agreement between IM subgroups. Validation studies are needed, but our results in this large cohort suggest the 2017 criteria might be useful for clinical trials and research in IM.References[1] Lundberg IE. Ann Rheum Dis2017;76:1955–64.[2] Nuño L. Rheumatol Clin2017;13:331–7.Disclosure of InterestNone declared

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Background:The mortality rate in patients with systemic lupus erythematosus (SLE) is 2–3 times higher than in the general population. However, survival in these patients has improved significantly and is currently 95% at 5 years according to different studies. Since the last 20 years, there are no new reports on this issue in Argentina.Objectives:To analyze the factors associated with mortality, survival and causes of death in patients with SLE.Methods:Longitudinal - multicenter study, in which 10 rheumatology centers of Argentina participated. Patients with SLE (ACR 1997 and / or SLICC 2012 criteria) with a minimum follow-up of 6 months monitored between January 2008 and December 2018 were included. Demographic, clinical, laboratory, therapeutic variables (treatments received during the evolution of the disease and within 60 days prior to death or last control); mortality, causes of death and survival at 5, 10 and 20 years were evaluated. Statistical analysis: descriptive statistics, Kaplan-Meier survival curves and Cox regression model.Results:Three hundred and eighty two patients were included; 90% women and 82% mestizos. The mean of evolution time of SLE was 4.1 ± 6.7 years. The mean age at the last control or death was 37.2 ± 12.7 years, SLEDAI 3.2 ± 4.2 and SLICC 1.2 ± 1.9.Mortality was 12% (95% CI [8-15]) and the causes of death were: Infections (27), cardiovascular disease (6), SLE activity (3), catastrophic antiphospholipid syndrome (2) and other causes (8). Using the variables associated with mortality in different Cox regression models, the variables that increased the risk of death significantly were: renal involvement (RR 3.3), cardiac involvement (RR 2.7), central nervous system involvement (RR 2.1), arterial thrombosis (RR 2.3), hyperlipemia (RR 2.4), number of infections (RR 1.2) and last SLEDAI (1.1).The time of HCQ use greater than 36 months decreased the risk of death in this cohort by 40% (p 0.03). Prednisone (maximum dose and time) was not associated with mortality (p NS). When analyzing the last treatment and adjusting it for final SLEDAI, HCQ was a mortality protection factor (RR 0.4) while the use of cyclophosphamide alone or associated with prednisone was a risk factor for death (RR 5.2).Significant differences were found when analyzing the causes of death according to the SLE evolution time (p 0.017): patients who died from infection had less evolution time (Me 2.25 years), than those who died due to cardiovascular causes (Me 10 years) or SLE activity (Me 15 years). In this cohort of patients, survival was 93% at 5 years, 88% at 10 years and 72% at 20 years.Conclusion:Mortality in this series of patients was 12% and infection was the leading cause of death. The use of HCQ for a period greater than 36 months, decreased the risk of death 40%.Disclosure of Interests:None declared

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Recent studies on systemic lupus erythematosus (SLE) have reported more favorable outcomes thanks to better knowledge of the disease, more expert management of it, and rational use of treatments. In this study we identified 301 SLE patients, seen between 1988 and 2019. Two hundred and twenty eight patients were treated in the public health system and 73 in private practice. In comparing both groups, we discovered that patients in the public health system were youn-ger at first consultation and at SLE onset and that the mean duration of their disease prior to first consultation was shorter in a statistically significant way. Also, they showed more frequence of leucopenia, Sm antibody, renal involvement and received I.V. corticosteroids. Patients treated in the public system of health showed more accrual damage over the 10 first years of the disease than patients seen in the private system of health, but death in both groups was similar. Patients from both public and private groups were attended by medical specialist practices who made close follow-ups.