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Articular involvement in Systemic Lupus Erythematosus (SLE) is well recognized as one of the most common manifestations of the disease. This article reviews the recent knowledge of the clinical manifestations, diagnostic techniques and therapies used for the treatment of joint involvement in SLE. The degree of articular involvement is characterized by widespread heterogeneity in terms of clinical presentation and severity. It may range from minor arthralgia without erosions or deformity to erosive arthropathy and severe functional disability. Inflammatory musculoskeletal manifestations are described as a major cause of pain impacting daily activities and as a major determinant of quality-of-life impairment. Thus, physicians must be aware of articular involvement in SLE. Lupus arthritis diagnosis may be challenging, due to the frequently mild synovitis. The introduction of new more sensitive imaging techniques, such as ultrasound, and MRI have contributed significantly to improving the diagnosis of osteoarticular involvement in SLE. There are several treatment options for the management of joint manifestations in patients with SLE. The choice of treatment will depend on the type and pattern of joint involvement, its severity, and the characteristics of the patient.

The initial immune response to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) includes an interferon-dependent antiviral response. A late and uncontrolled inflammatory response characterized by high activity of proinflammatory cytokines and the recruitment of neutrophils and macrophages develops in predisposed individuals and is potentially harmful in some cases. Interleukin (IL)-17 is one of the many cytokines released during coronavirus disease 2019 (COVID-19). IL-17 is crucial in recruiting and activating neutrophils, cells that can migrate to the lung, and are heavily involved in the pathogenesis of COVID-19. During the infection T helper 17 (Th17) cells and IL-17-related pathways are associated with a worse outcome of the disease. All these have practical consequences considering that some drugs with therapeutic targets related to the Th17 response may have a beneficial effect on patients with SARS-CoV-2 infection. Herein, we present the arguments underlying our assumption that blocking the IL-23/IL-17 axis using targeted biological therapies as well as drugs that act indirectly on this pathway such as convalescent plasma therapy and colchicine may be good therapeutic options.

RESUMEN La poliautoinmunidad se define como la presencia de más de una enfermedad autoinmune (EA) bien caracterizada en un mismo paciente. Es una condición frecuente en pacientes con síndrome de Sjögren (SS) y sigue un patrón de agrupamiento. Las EA más frecuentes observadas en el SS son la enfermedad tiroidea autoinmune, la artritis reumatoide y el lupus eritematoso sistémico. El estudio de este fenómeno aporta claves importantes para entender los mecanismos comunes de las EA. ABSTRACT Multiple autoimmunity is defined as the presence of more than one well-defined autoimmune disease (AD) in a single patient. Multiple autoimmunity is a frequent condition in Sjögren's syndrome (SS) and follows a grouping pattern. The most frequent ADs observed in SS are autoimmune thyroid disease, rheumatoid arthritis, and systemic lupus erythematosus. The study of multiple autoimmunity provides important clues for elucidating the common mechanisms of ADs.

Background and aim There is increasing interest regarding SARS-CoV-2 infection in patients with autoimmune and immune-mediated inflammatory diseases (AI/IMID) with some discrepancies in different cohorts about their risk and outcomes. The aim was to describe a multidisciplinary cohort of patients with AI/IMID and symptomatic SARS-CoV-2 infection in a single tertiary center and analyze sociodemographic, clinical, and therapeutic factors associated with poor outcomes. Methods A retrospective observational study was conducted from the 1st of March until May 29th, 2020 in a University tertiary hospital in Barcelona, Spain. Patients with an underlying AI/IMID and symptomatic SARS-CoV-2 infection were identified in our local SARS-CoV-2 infection database. Controls (2:1) were selected from the same database and matched by age and gender. The primary outcome was severe SARS-CoV-2 infection, which was a composite endpoint including admission to the intensive care unit (ICU), need for mechanical ventilation (MV), and/or death. Several covariates including age, sex, and comorbidities among others were combined into a multivariate model having severe SARS-CoV-2 as the dependent variable. Also, a sensitivity analysis was performed evaluating AID and IMID separately. Results The prevalence of symptomatic SARS-CoV-2 infection in a cohort of AI/IMID patients was 1.3%. Eighty-five patients with AI/IMID and symptomatic SARS-CoV-2 were identified, requiring hospitalization in 58 (68%) cases. A total of 175 patients admitted for SARS-CoV-2 (58 with AI/IMID and 117 matched-controls) were analyzed. In logistic regression analysis, a significant inverse association between AI/IMID group and severe SARS-CoV-2 (OR 0.28; 95% CI 0.12–0.61; p = 0.001), need of MV (OR 0.20; IC 95% 0.05–0.71; p = 0.014), and ICU admission (OR 0.25; IC 95% 0.10–0.62; p = 0.003) was found. Conclusions Patients with AI/IMID who require admission for SARS-CoV-2 infection have a lower risk of developing severe disease, including the need to stay in the ICU and MV., Highlights • Patients with AI/IMID fwho required admission for SARS CoV2 infection have a lower risk of developing severe disease. • Among patients with AID and IMID, there were no differences in terms of severity. • According to the 7-category ordinal scale, maximum oxygen requirement was lower among AI/IMID group.

Polyautoimmunity is defined as the presence of more than one well-defined autoimmune disease (AD) in a single patient. Polyautoimmunity is a frequent condition in Sjögren syndrome (SS) and follows a grouping pattern. The most frequent ADs observed in SS are autoimmune thyroid disease, rheumatoid arthritis, and systemic lupus erythematosus. Main factors associated with polyautoimmunity in SS are tobacco smoking and some genetic variants. The study of polyautoimmunity provides important clues for elucidating the common mechanisms of autoimmne diseases (ie, the autoimmune tautology). © 2016 Elsevier Inc.

Guillain–Barré syndrome (GBS) is a rapid-onset muscle weakness disease caused by the immune-mediated damage of the peripheral nervous system. Since there is an increase incidence of GBS cases in Latin America, particularly in Colombia, and most of them are currently preceded by Zika virus (ZIKV) infection, we aimed to assess the available evidence of the disease in Colombia through a systematic literature review. Out of 51 screened abstracts, only 16 corresponded to articles that met inclusion criteria, of which 15 were case reports or case series. A total of 796 cases of GBS were reported in the included articles. The majority of patients were males (66.8 %) and younger than 50 years old (94 %). An infectious disease before the onset of GBS was registered in 31 % of patients, with gastrointestinal or respiratory symptoms being the most frequently observed. In those cases in which electrodiagnostic tests were performed, the most common subphenotype was acute inflammatory demyelinating polyneuropathy (17 %). Death was reported in 15 % of patients. Data regarding GBS in Colombia is scant and heterogeneous. Taking into account the burden of the disease and the recent rise of GBS cases associated with ZIKV, a careful patient evaluation and a systematic collection of data are warranted. A form to data gathering is proposed. © 2016, Springer Science+Business Media New York.

Background Clinical response to biologics varies widely between individuals with rheumatoid arthritis (RA). To date, there are few, and in some cases, conflicting results in the personalized approach of patients with RA treated with abatacept. Only the seropositive subphenotype (anti-cyclic citrullinated peptide, CCP) was validated in several populations, including a real-life registry (ORA) [1], and non-inferiority trial (AMPLE) [2]. Objectives To assess whether baseline anti-CCP3 antibody status and concentration correlated with drug survival of subcutaneous (SC) abatacept among patients with RA in a real-world setting. Methods This was a prospective study in which well-characterized patients with RA (by 1987 ACR criteria) were included from April 2014 to December 2016. Patients were evaluated at a single rheumatology outpatient center in Bogota, Colombia. Baseline anti-CCP3 antibody status (positive/negative) and concentration were determined using an anti-CCP3 IgG ELISA (INOVA Diagnostics). Patients with a baseline anti-CCP3 IgG concentration of ≥20 U were considered to be positive and were further divided into equal quartiles according to concentration [Q1–Q4 (highest concentration)]. The Cox proportional hazards regression model was used to test if there were any differences in drug survival curves according to baseline anti-CCP3 antibody status and concentration. The test was performed by the coxph function of the “survival” R package [3]. Results A total of 129 patients were included. Baseline characteristics: female gender 86%, mean age 52±13 years, median disease duration 10 (IQR 11) years, and erosions 35%. Treatment background was as follows: biologic-naive (n=54), switched from IV to SC abatacept administration (n=24), and inadequate response to at least 1 biologic disease-modifying antirheumatic drug (n=51). Forty-three patients (33%) discontinued treatment. The most frequent reasons for drug suspension were loss of efficacy. Rheumatoid Factor and anti-CCP3 was positive in 94%, and 89%, respectively. Median titre of anti-CCP3 was 248 U (IQR 352), and number of patients in each quartile group were Q1 (22–122)=21; Q2 (123–248)=20; Q3 (249–475)=19; Q4 (476–1544)=19. According to Cox proportional hazards regression model (Fig.1), there were significant differences between survival curves for Q1 (HR 0.1; 0.02–0.60 95% IC; p=0.011), Q2 (HR 0.2; 0.06–0.94 95% IC; p=0.041), and Q3 (HR 0.2; 0.04–0.85 95% IC; p=0.030), compared to negative group at month 32. Conclusions Baseline anti-CCP3 positivity was associated with a better response for SC abatacept in a real-world setting. Patients with lowest baseline anti-CCP3 antibody concentrations had better drug survival than patients with higher concentrations. Our results highlight the importance of identification of factors associated with response to biologics in order to optimize treatment and reduce costs. References Sokolove J, et al. Ann Rheum Dis. 2016;75(4):709–14. Gottenberg JE, et al. Ann Rheum Dis. 2012;71(11):1815–9. Therneau,T, Grambsch P. Modeling Survival Data: Extending the Cox Model. Springer-Verlag, 2000. Disclosure of Interest None declared

Background Survival time or time to discontinuation of medication is a surrogate of the long-term impact on the course of disease in real life. It reflects clinical effectiveness in the absence of significant adverse events [1]. Treatment discontinuation can result from loss of efficacy or safety concerns, but prognostic factors for patient retention have not been explored thoroughly despite data for abatacept and other biologics being available from national registries [2]. Objectives To assess drug survival of subcutaneous (SC) abatacept among patients with rheumatoid arthritis (RA) according to treatment background. Methods This was a prospective study in which well-characterized patients with RA (by 1987 ACR criteria) were assessed from April 2014 to December 2016. Each patient was evaluated by a rheumatologist in a single rheumatology outpatient private center in Bogota, Colombia. Patients were stratified by treatment background: (n=54) biologic-naive, (n=24) switched from IV to SC abatacept administration, and (n=51) inadequate response to at least 1 biologic disease-modifying antirheumatic drug. The Mantel-Haenszel test was used to test if there were any differences in the survival curves among groups. The test was performed by the survdiff function of the “survival” R package [3]. Results A total of 129 patients were included. Baseline characteristics of patients were as follows: female gender 86%, mean age 52±13 years, median disease duration 10 (IQR 11) years, Rheumatoid Factor positive 94%, Anti-Cyclic Citrullinated Peptide Antibodies 89%, and erosive phenotype 35%. At baseline, mean DAS28 and RAPID3 were 5.4±1.3 and 16.6±6.8, respectively. SC abatacept monotherapy was reported in 27%. Demographics and disease characteristics were similar in all groups, except for baseline DAS28 and RAPID3 in switch group (p Conclusions Our results disclose a similar drug survival of SC abatacept regardless of treatment background. Patients switching from IV to SC formulation of abatacept had lower activity and functional impairment at baseline, and survival tends to be higher through follow-up. The insurance-related limitations is a reality in Latin American countries, and could have a negatively impact on survival time of several drugs. References Nuslein HG, et al. BMC Musculoskelet Disord. 2015;16:176. Souto A, et al. Rheumatology (Oxford). 2016;55(3):523–34. Therneau, T. and Grambsch, P.M. 2000. Acknowledgements The authors are grateful to all the members of the Center of Dermatology and Rheumatology (FUNINDERMA) for their contribution to this work. Disclosure of Interest None declared

Objective. This study was performed to determine the prevalence of and associated risk factors for cardiovascular disease (CVD) in Latin American (LA) patients with systemic lupus erythematosus (SLE).Methods. First, a cross-sectional analytical study was conducted in 310 Colombian patients with SLE in whom CVD was assessed. Associated factors were examined by multivariate regression analyses. Second, a systematic review of the literature on CVD in SLE in LA was performed.Results. There were 133 (36.5%) Colombian SLE patients with CVD. Dyslipidemia, smoking, coffee consumption, and pleural effusion were positively associated with CVD. An independent effect of coffee consumption and cigarette on CVD was found regardless of gender and duration of disease. In the systematic review, 60 articles fulfilling the eligibility criteria were included. A wide range of CVD prevalence was found (4%–79.5%). Several studies reported ancestry, genetic factors, and polyautoimmunity as novel risk factors for such a condition.Conclusions. A high rate of CVD is observed in LA patients with SLE. Awareness of the observed risk factors should encourage preventive population strategies for CVD in patients with SLE aimed at facilitating the suppression of cigarette smoking and coffee consumption as well as at the tight control of dyslipidemia and other modifiable risk factors.

Introduction: Guillain-Barré syndrome is a rare disease representing the most frequent cause of acute flaccid symmetrical weakness of the limbs and areflexia usually reaching its peak within a month. The etiology and pathogenesis remain largely enigmatic and the syndrome results in death or severe disability in 9–17% of cases despite immunotherapy. Areas covered: In terms of etiology, Guillain-Barré syndrome is linked to Campylobacter infection but less than 0.1% of infections result in the syndrome. In terms of pathogenesis, activated macrophages and T cells and serum antibodies against gangliosides are observed but their significance is unclear. Expert commentary: Guillain-Barré syndrome is a heterogeneous condition with numerous subtypes and recent data point towards the role of ganglioside epitopes by immunohistochemical methods. Ultimately, the syndrome results from a permissive genetic background on which environmental factors, including infections, vaccination and the influence of aging, lead to disease. © 2016 Informa UK Limited, trading as Taylor and Francis Group.

Personalized medicine encompasses a broad and evolving field informed by a patient distinctive information and biomarker profile. Although terminology is evolving and some semantic interpretations exist (e.g., personalized, individualized, precision), in a broad sense personalized medicine can be coined as: “To practice medicine as it once used to be in the past using the current biotechnological tools.” A humanized approach to personalized medicine would offer the possibility of exploiting systems biology and its concept of P5 medicine, where predictive factors for developing a disease should be examined within populations in order to establish preventive measures on at-risk individuals, for whom healthcare should be personalized and participatory. Herein, the process of personalized medicine is presented together with the options that can be offered in health care systems with limited resources for diseases like rheumatoid arthritis and type 1 diabetes. © 2016 Elsevier B.V.

Septic sacroiliitis is an unusual condition, and due to its non-specific symptoms, the diagnosis is often delayed. It should be suspected in cases in which inflammatory back pain and systemic inflammatory signs co-exist, especially in people with risk factors, such as postpartum. The case is presented of a woman, who in the late postpartum, presented with sacroiliitis and severe sepsis due to Escherichia coli. This is the second report of a case of septic sacroiliitis due to E. coli associated with pregnancy. La sacroiliitis séptica es una condición inusual, a menudo el diagnóstico se hace de forma tardía debido a la poca especificidad de los síntomas. Debe ser sospechada en casos donde coexista dolor lumbar inflamatorio y signos de respuesta inflamatoria sistémica, especialmente en personas con factores de riesgo tales como el puerperio. En este artículo reportamos el caso de una mujer quien durante el puerperio tardío presentó sacroiliitis por Escherichia coli y sepsis grave secundaria, siendo este el segundo caso reportado de sacroiliitis séptica por Escherichia coli asociada al embarazo.

Objectives Familial autoimmunity and polyautoimmunity represent extreme phenotypes ideal for identifying major genomic variants contributing to autoimmunity. Whole exome sequencing (WES) and linkage analysis are well suited for this purpose due to its strong resolution upon familial segregation patterns of functional protein coding and splice variants. The primary objective of this study was to identify potentially autoimmune causative variants using WES data from extreme pedigrees segregating polyautoimmunity phenotypes. Methods DNA of 47 individuals across 10 extreme pedigrees, ascertained from probands affected with polyautoimmunity and familial autoimmunity, were selected for WES. Variant calls were obtained through Genome Analysis Toolkit. Filtration and prioritization framework to identify mutation(s) were applied, and later implemented for genetic linkage analysis. Sanger sequencing corroborated variants with significant linkage. Results Novel and mostly rare variants harbored in SRA1, MLL4, ABCB8, DHX34 and PLAUR showed significant linkage (LOD scores are >3.0). The strongest signal was in SRA1, with a LOD score of 5.48. Network analyses indicated that SRA1 , PLAUR and ABCB8 contribute to regulation of apoptotic processes. Conclusions Novel and rare variants in genetic linkage with polyautoimmunity were identified throughout WES. Genes harboring these variants might be major players of autoimmunity.

La sacroiliitis séptica es una condición inusual, a menudo el diagnóstico se hace de forma tardía debido a la poca especificidad de los síntomas. Debe ser sospechada en casos donde coexista dolor lumbar inflamatorio y signos de respuesta inflamatoria sistémica, especialmente en personas con factores de riesgo tales como el puerperio. En este artículo reportamos el caso de una mujer quien durante el puerperio tardío presentó sacroiliitis por Escherichia coli y sepsis grave secundaria, siendo este el segundo caso reportado de sacroiliitis séptica por Escherichia coli asociada al embarazo. Septic sacroiliitis is an unusual condition, and due to its non-specific symptoms, the diagnosis is often delayed. It should be suspected in cases in which inflammatory back pain and systemic inflammatory signs co-exist, especially in people with risk factors, such as postpartum. The case is presented of a woman, who in the late postpartum, presented with sacroiliitis and severe sepsis due to Escherichia coli. This is the second report of a case of septic sacroiliitis due to E. coli associated with pregnancy.

Studies documenting increased risk of developing autoimmune diseases (ADs) have shown that these conditions share several immunogenetic mechanisms (i.e., the autoimmune tautology). This report explored familial aggregation and segregation of AD, polyautoimmunity, and multiple autoimmune syndrome (MAS) in 210 families. Familial aggregation was examined for first-degree relatives. Segregation analysis was implemented as in S.A.G.E. release 6.3. Data showed differences between late- and early-onset families regarding their age, age of onset, and sex. Familial aggregation of AD in late- and early-onset families was observed. For polyautoimmunity as a trait, only aggregation was observed between sibling pairs in late-onset families. No aggregation was observed for MAS. Segregation analyses for AD suggested major gene(s) with no clear discernible classical known Mendelian transmission in late-onset families, while for polyautoimmunity and MAS no model was implied. Data suggest that polyautoimmunity and MAS are not independent traits and that gender, age, and age of onset are interrelated factors influencing autoimmunity. © 2015 John Castiblanco et al.

Since cardiovascular disease (CVD) is the most common cause of mortality in patients with rheumatoid arthritis (RA), we aimed to determine factors associated with such a complication in a large series of Colombian patients. This was a cross-sectional analytical study in which 800 consecutive Colombian patients with RA were assessed for variables associated with CVD. Furthermore, a systematic literature review was performed to address the state of the art about non-traditional risk factors for CVD in RA. The preferred reporting items for systematic reviews and meta-analyses guidelines were followed in data extraction, analysis, and reporting of articles selected. Hypercholesterolemia, type 2 diabetes mellitus, abnormal body mass index, abdominal obesity, and current smoking were all traditional risk factors significantly associated with CVD in Colombians. As non-traditional risk factors, familial autoimmunity, more than 10 years of duration of the disease, patients working on household duties, use of systemic steroids, and low education level were associated with CVD in the studied population. Out of a total of 9,812 articles identified in PubMed and Scopus databases, 140 fulfilled the eligibility criteria and were included. Through this systematic review, several factors and outcomes related to CVD were confirmed and identified. These were categorized into genetics, RA-related, and others. Traditional risk factors do not completely explain the high rates of CVD in patients with RA; thus, novel risk factors related to autoimmunity are now recognized predicting the presence of CVD as strong as traditional risk factors. Our results may assist health professionals and policymakers in making decisions about CVD in patients with RA. © 2013 Springer Science+Business Media New York.

Background Therapeutic guidelines draw heavily on evidence from randomised controlled trials undertaken in well-characterised, highly-selective populations and managed in tightly-controlled settings. As such, the extent to which therapeutic efficacy in real-life populations and routine care settings is often unclear. Objectives To assess subcutaneous (SC) abatacept (ABA) efficacy and safety in a real-life setting. Methods This was a retrospective and single center study in which 70 patients (1987 ACR) were assessed from April 2014 to December 2014. The patients were stratified according to their treatment background: biologic-naive, switched from IV to SC ABA administration (125mg-wk), and inadequate response to TNF inhibitor (TNF-IR). The primary and secondary endpoints were change in DAS28-CRP, and Routine Assessment of Patient Index Data (RAPID3) from baseline to 9 months, respectively. A linear mixed effects model was made to account for correlation among repeated measures. Adverse events (AEs) were assessed and recorded at each visit to the rheumatology center. Results Baseline characteristics of patients were as follows: female gender 84.3%, mean age 54.9±10.6 years, median disease duration 12.5 (IQR 14) years, Rheumatoid Factor or Anti-Cyclic Citrullinated Peptide Antibodies seropositivity 93.8%, erosive disease 32.9%, SC ABA monotherapy 48.5%, concomitant use of methotrexate (MTX) 38.5%, mean DAS28-CRP 4.61±1.59, and mean RAPID3 15.2±7.1. Demographics and disease characteristics were similar in the three groups (including MTX use), except for baseline DAS28-CRP (p Conclusions Our results disclose an improvement in RA disease activity and physical function, during 9 months administration of SC ABA. Patients switching from IV to SC formulation of ABA had lower activity and functional impairment at baseline, and efficacy was maintained through follow-up. SC ABA demostrated a good safety profile, consistent with previously published data. References Nuslein H, et al. Arthritis Rheum 2012;64(Suppl10):S199. Pincus T, et al. Rheumatology (Oxford). 2008 Mar;47(3):345-9. Reggia R, et al. J Rheumatol. 2014 Dec 15 [Epub ahead of print]. Disclosure of Interest None declared

Background Polyautoimmunity (i.e., the presence of two or more autoimmune diseases in a single patient) and familial autoimmunity (i.e., diverse autoimmune diseases in a nuclear family) represent extreme phenotypes ideal for identifying major genomic variants contributing to autoimmunity (1–3). Whole Exome Sequencing (WES) and linkage analysis are well suited for this purpose due to its strong resolution upon familial segregation patterns of functional protein coding and splice variants (3). Objectives The primary objective of this study was to identify potentially autoimmune causative variants using WES data from extreme pedigrees segregating polyautoimmunity phenotypes. Methods DNA of 47 individuals across 10 extended and multigenerational pedigrees, ascertained from probands affected with polyautoimmunity and familial autoimmunity, were selected for WES. Variant calls were obtained through Genome Alignment Tool Kit (GATK) algorithm; filters for prioritizing major functional were applied and later implemented for genetic linkage analysis. Sanger sequencing corroborated variants with significant linkage. Results Novel and mostly rare variants harbored in SRA1, MLL4, ABCB8, DHX34 and PLAUR genes showed significant linkage (LOD scores are >3.30). The strongest signal was in SRA1 , with a LOD score of 5.48. Network analyses indicated that SRA1 and PLAUR both contribute to regulation of apoptotic processes. Conclusions Novel and rare variants in genetic linkage with polyautoimmunity were identified throughout WES. Genes harboring these variants might be major players of autoimmunity. References Expert Rev Clin Immunol. 2007;3:623–35. BMC Med. 2013;11:73. Autoimmun Rev. 2015;14:204–9. Acknowledgement Supported by Colciencias (Bogota, Colombia) Disclosure of Interest None declared

Background Systemic Lupus Erythematosus (SLE) is a complex disease which can potentially involve any organ and, therefore, has a wide range of clinical manifestations (i.e., subphenotypes) [1]. Objectives To propose a novel methodology for building an index of severity in patients with SLE. Methods A cross-sectional analytical study was done on 319 SLE patients (1997 ACR). A structural model for the latent trait in the 2pl item response theory model was fitted following the Ogasawara9s formulation [2] in order to characterize a severity construct of 17 variables. In addition, the model analyzed possible associations of the severity latent trait with other patient covariates including cardiovascular disease, age at onset of the disease, and expositional factors. Results Baseline characteristics of patients were as follows: female gender 91%, median age 37 (IQR 21) years, median duration of the disease 5 (IQR 10) years, lupus nephritis 37.3%, and central nervous system involvement in 16%. Several symptoms were excluded from the severity construct due to inconsistency according to Cronbach-Mesbah Curve including lupus nephritis, diffuse alveolar hemorrhage, and vasculitis. A total of 11 symptoms were included in the item response theory model showing three levels of disease severity (Figure 1). The only covariate registered that reaches an association with severity was coffee consumption. Conclusions Symptoms excluded due to inconsistency share the same pathogenic mechanism in SLE, which corresponds to autoantibody-driven tissue damage, and are associated with high mortality risk. The remaining symptoms are related to inflammation and vasculopathy, and in most cases, require a less aggressive immunosuppressive treatment [3]. The order of symptoms on the present scale of severity coincides with clinical experience, and the three levels of performance can be associated with low, moderate, and high severity. The present index of severity is useful for SLE patients with less severe phenotype (i.e., without nephritis, diffuse alveolar hemorrhage, and vasculitis). A further replication study is necessary to validate these results. References Tsokos GC. N Engl J Med 2011;365(22):2110–21. Ogasawara, H. Behaviormetrika;1995 22(1): 37–48. D9Cruz DP, Khamashta MA, and Hughes GR. Lancet 2007;369(9561):587–96. Disclosure of Interest None declared

Background Cigarette smoking has been found to play a pathogenic role in several autoimmune diseases. However, data about its influence on Sjogren9s syndrome (SS) is scarce [1,2]. Objectives To assess the impact of cigarette smoking on patients with SS. Methods This was a cross-sectional study in which consecutive patients with SS (2002 AECG) followed at a single center were included. Smoking status (ever) together with additional environmental factors and clinical and laboratory characteristics were assessed. Bivariate analysis and the mixed-cluster methodology based on multivariate descriptive methods were employed to analyze data. Results Out of a total of 277 patients, 94% were women. The mean age of patients was 51.2 ± 12.5 years, and the mean duration of the disease was 7.1 ± 7.1 years. By Chisholm and Mason classification, 93% of patients were positive for a minor salivary gland biopsy. Anti-Ro and anti-La antibodies were present in 65% and 40% respectively (by ELISA). Polyautoimmunity was registered in 43% of patients. Smokers showed a longer duration of the disease and were mostly men. Cigarette smoking was associated with parotidomegaly (OR: 1.9; 95%CI 1.05–4.3; p=0.03), polyautoimmunity (OR: 1.9; 95%CI 1.2–3.3; p=0.009), acid peptic disease (OR: 2.3; 95%CI 1.4–4.0; p=0,0008), and coffee consumption (OR: 1.7; 95%CI 0.98–3.6; p=0.04). In order to clarify whether the observed associations were influenced by gender or coffee consumption, 4 clusters were built: 1) men with a high frequency of smoking and coffee consumption (5.6%), 2) women who exclusively reported coffee consumption (42%), 3) women who all drank coffee and smoked cigarettes (30%) and 4) women with no coffee consumption but a third had been exposed to tobacco (22.4%). Cluster 2 patients were statistically less prone to present factors found in the bivariate analysis suggesting that coffee consumption may counteract the harmful effects of smoking. Conclusions Our results indicate that cigarette smoking has significant effects on SS expression, which could be modified by coffee consumption. References Manthorpe R, et al. Ann Rheum Dis 2000;59(1):54–60. Karabulut G, et al. Mod Rheumatol 2011;21(6):602–7. Disclosure of Interest None declared

Background Age at onset of disease (AOD) refers to the time period at which a patient experiences the first symptom(s). AOD varies among autoimmune diseases (ADs) and has been related to prognosis in some of them. Genetic, environmental and immunological factors may influence the AOD. Sjogren’s syndrome (SS) is considered an AD affecting mostly older women. However, information about how does AOD influence the course of SS is scarce. Objectives To evaluate the factors associated with AOD in SS. Methods This was a two-phase study. First, a cross sectional analytical study was undertaken in 294 consecutive adult patients with SS (by AECG criteria) with a mean duration of disease of 6.4 years. Clinical, laboratory and sociodemographic variables were investigated through a structured medical record review. AOD was categorized in early (17-35 years) and late (>35 years) according to the 25th percentile of AOD in our cohort. Odds Ratios (ORs) with their 95% confidence intervals were calculated, and p-values Results Significant differences were not observed between early-AOD (73/294) and late-AOD (221/294) groups, except for the presence of cutaneous vasculitis (OR= 0.15, 95% CI: 0.07-0.39, p Conclusions In contrast with other ADs, AOD has not a critical effect on disease expression and autoimmune response in patients with SS. Late-onset traits are more sensitive to environmental variation rather than genetic influence. Therefore, these results, together with the fact that SS has a similar clinical and immunological presentation worldwide, may serve to design better strategies aimed to discover the etiological factors of disease. References Amador-Patarroyo MJ, et al. Autoimmune Dis . 2012;1-7. Botsios C, et al. Joint Bone Spine . 2011;78:171-4. Haga HJ, et al. Scand J Rheumatol . 1999;28:227-32. Ramos-Casals M, et al. Lupus . 1998;7;202-6. Disclosure of Interest None Declared