Search

BACKGROUND/OBJECTIVES Immune-mediated inflammatory diseases (IMIDs) are prevalent diseases. There is, however, a lack of understanding of the link between diet and IMIDs, how much dietary patterns vary between them and if there are food groups associated with a worsening of the disease. SUBJECTS/METHODS To answer these questions we analyzed a nation-wide cohort of n = 11,308 patients from six prevalent IMIDs and 2050 healthy controls. We compared their weekly intake of the major food categories, and used a Mendelian randomization approach to determine which dietary changes are caused by disease. Within each IMID, we analyzed the association between food frequency and disease severity. RESULTS After quality control, n = 11,230 recruited individuals were used in this study. We found that diet is profoundly altered in all IMIDs: at least three food categories are significantly altered in each disease (P

Few studies have been published focusing on the differences between juvenile idiopathic inflammatory myopathy (JIIM) and adult IIM. This study aimed to describe the characteristics of JIIM main subgroups (juvenile dermatomyositis [JDM] and juvenile polymyositis [JPM]) and to compare their differences with adult IIM subgroups (adult DM and adult PM). Methods This study reviewed the medical records of patients from the REMICAM cohort, a multicentric longitudinal study carried out in patients with IIM, followed up between 1980 and 2014 in 12 hospitals in Madrid, Spain. Patients with definite or probable JPM, JDM, adult DM, and adult PM according to the modified Bohan and Peter criteria were selected. We compared the characteristics between JDM and JPM, and between JIIM and adult IIM. Results Eighty-six juvenile patients (75 JDMs and 11 JPMs) and 283 adult patients (133 DMs and 150 PMs) were included. Compared with patients with JDM, patients with JPM were older at diagnosis, had more fever and arthritis, and were less frequently treated with disease-modifying antirheumatic drugs (these differences were not statistically significant). Compared with patients with adult DM, those with JDM presented more frequently with calcinosis (33.8% vs 6.9%, p Conclusions Our findings confirm that JIIMs are a heterogeneous group of diseases with relevant differences compared with adult IIMs.

espanolObjetivo El objetivo de este estudio es describir los hallazgos de una evaluacion odontologica protocolizada en pacientes con sindrome de Sjogren primario (SSp) atendidos en las consultas de reumatologia de la Comunidad de Madrid. Metodos Estudio descriptivo multicentrico en el que se incluyeron pacientes con SSp clasificados segun criterios del consenso Europeo-Americano de 2002. Se recogieron datos demograficos, clinicos y serologicos. Se realizo una exploracion oral, se recogio el flujo salival y el indice de caries CAOD. Los pacientes rellenaron la escala visual analogica para xerostomia, el cuestionario Oral Health Impact Profile-14 y un cuestionario de salud oral. Resultados Se reclutaron 61 pacientes. El 98% fueron mujeres y la edad media fue de 57 anos. El 52,5% presentaban signos orales patologicos (flujo salival no estimulado/sialografia/gammagrafia de glandulas salivales) y el 90,2% antiRo/antiLa+. El 92% de los pacientes referian xerostomia y un 61% hiposialia. Treinta y cinco pacientes presentaron lesiones de la mucosa oral. El indice CAOD fue de 16,97 ± 7,93 y la escala visual analogica para la xerostomia fue de 46,69 ± 14,43. Los resultados del Oral Health Impact Profile-14 fueron de 23,13 ± 14,16. Los pacientes con signos orales patologicos obtuvieron una puntuacion en el Oral Health Impact Profile-14 significativamente mayor (p = 0,03), al igual que los pacientes con afectacion del sistema nervioso periferico (p = 0,001). Conclusiones La prevalencia de xerostomia en esta cohorte de pacientes con SSp fue muy elevada y el 61% de los enfermos presentaron hiposialia. Mas de la mitad de los pacientes sufrieron lesiones orales. La salud oral presento un impacto negativo en la calidad de vida de los pacientes con SSp, siendo mayor en aquellos pacientes con signos orales objetivos patologicos y en los que presentaban afectacion del sistema nervioso periferico. EnglishObjective The aim of this study is to describe the findings of a protocolised odontological evaluation of patients with primary Sjogren's syndrome (pSS) treated in rheumatology units in the Community of Madrid. Methods Multicentric descriptive study in which pSS patients classified according to the American-European consensus of 2002 criteria were included. We collected the demographic, clinical and serological data of each patient. A complete oral examination was performed and salivary flow and the CAOD caries index were collected. The patients completed the visual analogue scale for xerostomia, the Oral Health Impact Profile-14 questionnaire and an oral health questionnaire. Results Sixty-one patients were recruited. Ninety-eight percent were women and the mean age of the patients was 57 years. Pathological oral signs (unstimulated salivary flow/salivary glands sialography/scintigraphy) were present in 52.5% of the patients, anti-Ro/anti-La were positive in 90.2%. Ninety-two percent of the patients reported xerostomia and 61% of the patients suffered from hyposialia. Thirty-five patients presented oral mucosa lesions. CAOD index was 16.97 ± 7.93 and visual analogue scale for xerostomia was 46.69 ± 14.43. The results of the OHIP-14 questionnaire were 23.13 ± 14.16. Patients with pathological oral signs obtained a significantly higher Oral Health Impact Profile-14 score (P = .03). We also found that patients with peripheral nervous system involvement obtained a significantly higher Oral Health Impact Profile-14 score (P = .001) Conclusions The presence of xerostomia in this cohort of pSS patients was high and hyposialia was present in 61% of the patients. Oral lesions appeared in more than half of the subjects. Oral health had a negative impact on the quality of life of patients with pSS, being higher in those with pathological objective oral signs and in those with peripheral nervous system involvement.

Resumen Objetivo La dificultad para el diagnostico y la variedad de manifestaciones clinicas que pueden determinar la eleccion del tratamiento del sindrome antifosfolipido (SAF) primario ha impulsado a la Sociedad Espanola de Reumatologia (SER) en la elaboracion de recomendaciones basadas en la mejor evidencia posible. Estas recomendaciones pueden servir de referencia para reumatologos y otros profesionales implicados en el manejo de pacientes con SAF. Metodos Se creo un panel formado por 4 reumatologos, una ginecologa y una hematologa, expertos en SAF, previamente seleccionados mediante una convocatoria abierta o por meritos profesionales. Las fases del trabajo fueron: identificacion de las areas claves para la elaboracion del documento, analisis y sintesis de la evidencia cientifica (utilizando los niveles de evidencia de SIGN, Scottish Intercollegiate Guidelines Network) y formulacion de recomendaciones a partir de esta evidencia y de tecnicas de «evaluacion formal» o «juicio razonado». Resultados Se han elaborado 46 recomendaciones que abordan 5 areas principales: diagnostico y evaluacion, medidas de tromboprofilaxis primaria, tratamiento del SAF o tromboprofilaxis secundaria, tratamiento del sindrome antifosfolipido obstetrico y situaciones especiales. Esta incluido tambien el papel de los nuevos anticoagulantes orales, el problema de las recurrencias o los principales factores de riesgo identificados en estos individuos. En este documento se reflejan las ultimas 25, referidas a las areas de: SAF obstetrico y situaciones especiales. El documento contiene una tabla de recomendaciones y algoritmos de tratamiento. Conclusiones Se presentan las recomendaciones de la SER sobre SAF. Este documento corresponde a la parte 2.a relacionada con el SAF obstetrico y las situaciones especiales. Estas recomendaciones se consideran herramientas en la toma de decisiones para los clinicos, teniendo en consideracion tanto la decision del medico experto en SAF como la opinion compartida con el paciente. Se ha elaborado tambien una parte I que aborda aspectos relacionados con el diagnostico, evaluacion y tratamiento.

Objective The difficulty in diagnosis and the spectrum of clinical manifestations that can determine the choice of treatment for primary antiphospholipid syndrome (APS) has fostered the development of recommendations by the Spanish Society of Rheumatology (SER), based on the best possible evidence. These recommendations can serve as a reference for rheumatologists and other specialists involved in the management of APS. Methods A panel of four rheumatologists, a gynaecologist and a haematologist with expertise in APS was created, previously selected by the SER through an open call or based on professional merits. The stages of the work were: identification of the key areas for drafting the document, analysis and synthesis of the scientific evidence (using the Scottish Intercollegiate Guidelines Network [SIGN] levels of evidence) and formulation of recommendations based on this evidence and formal assessment or reasoned judgement techniques (consensus techniques). Results 46 recommendations were drawn up, addressing five main areas: diagnosis and evaluation, measurement of primary thromboprophylaxis, treatment for APS or secondary thromboprophylaxis, treatment for obstetric APS and special situations. These recommendations also include the role of novel oral anticoagulants, the problem of recurrences or the key risk factors identified in these subjects. This document reflects the first 21, referring to the areas of: diagnosis, evaluation and treatment of primary APS. The document provides a table of recommendations and treatment algorithms. Conclusions An update of the SER recommendations on APS is presented. This document corresponds to part I, related to diagnosis, evaluation and treatment. These recommendations are considered tools for decision-making for clinicians, taking into consideration both the decision of the physician experienced in APS and the patient. A part II has also been prepared, which addresses aspects related to obstetric SAF and special situations.

The aims of this study were to describe the long-term retention rate of golimumab (GLM) treatment in patients with rheumatoid arthritis (RA), psoriatic arthritis (PsA), and spondyloarthritis (SpA) in real life, and to analyze determinants of discontinuation. METHODS We conducted a single-center, medical records review of all patients with RA, PsA, and SpA on GLM treatment attending a large rheumatology department from 2010 to 2017. Times from start to end of GLM treatment were collected, as well as sociodemographic, clinical, and safety variables. Golimumab retention rate was estimated by the Kaplan-Meier method, and comparison across diseases was analyzed with the Mantel-Haenszel statistic (log-rank test). Cox proportional hazards regression models were used to identify factors associated with GLM discontinuation. RESULTS In the study period, a total of 212 patients (61 RA, 48 PsA, 103 SpA) were prescribed GLM. Retention rates were 72% in the first year, 61% in the second, 56% in the third, and 38% at 5 years. Differences were statistically significant across diseases (median times to GLM discontinuation were 50.2, 46.0, and 38.7 months for RA, SpA, and PsA, respectively) and according to the number of previous biologic therapies (55.2 months in biologic-naive patients vs 14.0 months in patients with ≥2 previous biologics; p < 0.001). The use of concomitant conventional synthetic disease-modifying antirheumatic drugs was associated with a lower probability of discontinuation (hazards ratio [HR], 0.57; 95% confidence interval [CI], 0.33-0.97). Female sex (HR, 1.84; 95% CI, 1.07-3.17) and having used 2 biologics before GLM (HR, 2.99; 95% CI, 1.76-5.06) were associated with increased discontinuation rates. Twenty-three patients (10.9%) had at least 1 serious adverse event. CONCLUSIONS In a real-life setting, GLM shows appropriate long-term safety-effectiveness ratio.

Objective To investigate the human leukocyte antigen (HLA) association with anti-synthetase syndrome (ASSD). Methods We conducted the largest immunogenetic HLA-DRB1 and HLA-B study to date in a homogeneous cohort of 168 Caucasian patients with ASSD and 486 ethnically matched healthy controls by sequencing-based-typing. Results A statistically significant increase of HLA-DRB1*03:01 and HLA-B*08:01 alleles in patients with ASSD compared to healthy controls was disclosed (26.2% versus 12.2%, P = 1.56E–09, odds ratio–OR [95% confidence interval–CI] = 2.54 [1.84–3.50] and 21.4% versus 5.5%, P = 18.95E–18, OR [95% CI] = 4.73 [3.18–7.05]; respectively). Additionally, HLA-DRB1*07:01 allele was significantly decreased in patients with ASSD compared to controls (9.2% versus 17.5%, P = 0.0003, OR [95% CI] = 0.48 [0.31–0.72]). Moreover, a statistically significant increase of HLA-DRB1*03:01 allele in anti-Jo-1 positive compared to anti-Jo-1 negative patients with ASSD was observed (31.8% versus 15.5%, P = 0.001, OR [95% CI] = 2.54 [1.39–4.81]). Similar findings were observed when HLA carrier frequencies were assessed. The HLA-DRB1*03:01 association with anti-Jo-1 was unrelated to smoking history. No HLA differences in patients with ASSD stratified according to the presence/absence of the most representative non-anti-Jo-1 anti-synthetase autoantibodies (anti-PL-12 and anti-PL-7), arthritis, myositis or interstitial lung disease were observed. Conclusions Our results support the association of the HLA complex with the susceptibility to ASSD.

We aimed to identify the key players in the prothrombotic profile of patients with systemic lupus erythematosus (SLE) not mediated by antiphospholipid antibodies, as well as the potential utility of global coagulation tests to characterize hemostasis in these patients. Patients with SLE without antiphospholipid antibodies and without signs of thrombosis were included. The kinetics of clot formation were determined by ROTEM&reg, Platelet activation markers were determined by flow cytometry. Thrombin generation associated with Neutrophil Extracellular Traps (NETs) and microparticles (MPs) was measured by calibrated automated thrombogram (CAT). The plasma levels of PAI-1 were also determined. ROTEM&reg, showed a procoagulant profile in SLE patients. SLE patients had activated platelets and more leukocyte/platelet aggregates at basal conditions. The plasma PAI-1 and platelet aggregates correlated with several ROTEM&reg, parameters. The thrombin generation associated withthe tissue factor (TF) content of MPs and with NETs was increased. Our results suggest the utility of global tests for studying hemostasis in SLE patients because they detect their procoagulant profile, despite having had neither antiphospholipid antibodies nor any previous thrombotic event. A global appraisal of hemostasis should, if possible, be incorporated into clinical practice to detect the risk of a thrombotic event in patients with SLE and to consequently act to prevent its occurrence.

Background:Tocilizumab (TCZ) showed trends for improving skin fibrosis and prevented progression of lung fibrosis in patients with systemic sclerosis (SSc) in placebo-controlled randomised clinical trials (RCTs). However, safety and effectiveness of TCZ beyond these selected and enriched clinical trial populations in SSc is still unknown.Objectives:To assess safety and effectiveness of TCZ treatment compared to standard of care in SSc patients from the large, multicentre, observational, real-life EUSTAR network/database using propensity score matching.Methods:SSc patients from the EUSTAR network/database, who fulfilled the ACR/EULAR 2013 classification criteria, with a baseline and a follow-up visit at 12±3 months, receiving TCZ or standard of care (controls), were selected. The following variables were used for the propensity score matching (1:1): age at diagnosis, gender, disease subtype, baseline modified Rodnan skin score (mRSS), forced vital capacity (FVC), and diffusing capacity for carbon monoxide (DLCO), co-therapy with immunosuppressives, disease duration, and year of treatment. Primary endpoints were mRSS and FVC at 12±3 months follow-up compared between the groups, using paired t-tests. Secondary endpoints were the percentage of progressive/regressive patients for skin and lung at 12±3 months follow-up according to standard definitions (1,2). Sensitivity analyses assessed pre-processing decisions (selection of most recent vs. random observation for control patients with multiple suitable time intervals), as well as the matching method (optimal vs. exact matching). Missing values were addressed with 100-fold multiple imputation using chained equations. Safety data were analysed in all patients. The study including the statistical analysis plan was pre-registered at www.drks.de (DRKS-ID: DRKS00015537).Results:We identified 93 SSc patients treated with TCZ and 2370 SSc patients with standard of care who fulfilled the inclusion criteria. Forty nine (57.7%) of the TCZ treated patients were diffuse, eight patients were not classified, disease duration was (mean±SD) 6.35±5.40 years, their baseline mRSS was 15.05±10.85, and 76 (81.7%) received immunosuppressive therapy in addition to TCZ.Through multiple imputation and propensity score matching, 100 imputed sets of 93 pairs of TCZ/controls were generated. Comparison between groups showed consistent effects of TCZ across all pre-defined primary and secondary endpoints: mRSS was lower in the TCZ group (mean difference (95% confidence interval (CI)) -1.8 (-4.79 to 1.19), p=0.24, Figure 1A). Similarly, FVC % predicted was higher in the TCZ group mean difference (2.25, 95% CI -4.57 to 9.06), p=0.51, Figure 1B). Considering secondary endpoints, the percentage of skin progressors as well as lung progressors at follow up was lower in the TCZ group (odds ratio OR 0.67 (95% CI 0.07 to 6.41), p=0.74 and OR 0.53 (95% CI 0.16 to 1.7); p=0.2, respectively. Consistently, the percentage of regressors for skin (OR 1.6 (95% CI 0.56 to 4.54), p=0.38) and for lung (OR 1.74 (95% CI 0.66 to 4.58), p=0.26) was higher in TCZ. These results were robust regarding the sensitivity analyses. Safety analysis confirmed previously reported adverse event profiles.Conclusion:In this large, observational, controlled, real-life EUSTAR study, effectiveness of TCZ did not reach statistical significance compared to standard of care treatment but showed consistent positive effects of TCZ on skin and lung fibrosis across all pre-defined primary and secondary endpoints confirming data from recent RCTs.References:[1]Prediction of improvement in skin fibrosis in diffuse cutaneous systemic sclerosis: a EUSTAR analysis. Ann Rheum Dis 2016:1743-8.[2]Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database. Ann Rheum Dis 2021:219-227.Disclosure of Interests:Simon Kuster: None declared, Suzana Jordan: None declared, Muriel Daniele Elhai: None declared, Ulrike Held: None declared, Klaus Steigmiller: None declared, Cosimo Bruni: None declared, Florenzo Iannone: None declared, Serena Vettori: None declared, Elise Siegert: None declared, Simona Rednic: None declared, Veronica Codullo: None declared, Paolo Airò Consultant of: Dr. Airo’ reports personal fees (consultancies) from Bristol Myers Squibb, Bohringer Ingelheim, non-financial support from CSL Behring, SOBI, Janssen, Roche, Sanofi, Pfizer, Yolanda Braun-Moscovici: None declared, Nicolas Hunzelmann: None declared, Maria Joao Salvador: None declared, Valeria Riccieri: None declared, Ana Maria Gheorghiu: None declared, Juan Jose Alegre Sancho: None declared, Katarzyna Romanowska-Prochnicka: None declared, Ivan Castellví: None declared, Ina Koetter: None declared, Marie-Elise Truchetet Consultant of: Marie-Elise Truchetet has had consultancy relationships and/or has received research funding from Boehringer Ingelheim, Genentech/Roche, and Sanofi in the area of potential treatments of scleroderma and its complications., Grant/research support from: Marie-Elise Truchetet has had consultancy relationships and/or has received research funding from Boehringer Ingelheim, Genentech/Roche, and Sanofi in the area of potential treatments of scleroderma and its complications., Francisco J López-Longo: None declared, Pavel Novikov: None declared, Alessandro Giollo: None declared, Yuichiro Shirai: None declared, Laura Belloli: None declared, Elisabetta Zanatta: None declared, Eric Hachulla: None declared, Vanessa Smith: None declared, Christopher Denton: None declared, Ruxandra Ionescu: None declared, Tim Schmeiser: None declared, Jörg H.W. Distler: None declared, Armando Gabrielli: None declared, Anna-Maria Hoffmann-Vold Consultant of: AMHV has received research funding and/or consulting fees and/or other remuneration from Actelion, Boehringer Ingelheim, Roche, Bayer, Merck Sharp & Dohme, ARXX, Lilly and Medscape, Grant/research support from: AMHV has received research funding and/or consulting fees and/or other remuneration from Actelion, Boehringer Ingelheim, Roche, Bayer, Merck Sharp & Dohme, ARXX, Lilly and Medscape. Masataka Kuwana: None declared, Yannick Allanore: None declared, Oliver Distler Speakers bureau: Oliver Distler has/had consultancy relationship and/or has received research funding in the area of potential treatments for systemic sclerosis and its complications from (last three years): Abbvie, Acceleron Pharma, Amgen, AnaMar, Arxx Therapeutics, Baecon Discovery, Blade Therapeutics, Bayer, Boehringer Ingelheim, ChemomAb, Corbus Pharmaceuticals, CSL Behring, Galapagos NV, Glenmark Pharmaceuticals, GSK, Horizon (Curzion) Pharmaceuticals, Inventiva, iQvia, Italfarmaco, iQone, Kymera Therapeutics, Lilly, Medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Roche, Sanofi, Serodapharm, Topadur, Target Bioscience and UCB. Patent issued “mir-29 for the treatment of systemic sclerosis” (US8247389, EP2331143)., Consultant of: Oliver Distler has/had consultancy relationship and/or has received research funding in the area of potential treatments for systemic sclerosis and its complications from (last three years): Abbvie, Acceleron Pharma, Amgen, AnaMar, Arxx Therapeutics, Baecon Discovery, Blade Therapeutics, Bayer, Boehringer Ingelheim, ChemomAb, Corbus Pharmaceuticals, CSL Behring, Galapagos NV, Glenmark Pharmaceuticals, GSK, Horizon (Curzion) Pharmaceuticals, Inventiva, iQvia, Italfarmaco, iQone, Kymera Therapeutics, Lilly, Medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Roche, Sanofi, Serodapharm, Topadur, Target Bioscience and UCB. Patent issued “mir-29 for the treatment of systemic sclerosis” (US8247389, EP2331143)., Grant/research support from: The study was partially supported by a grant from Roche. Roche was not involved in analysis or interpretation of the results.

Resumen Objetivos Describir las caracteristicas clinicas, mortalidad y causas de muerte de una serie de pacientes diagnosticados de miositis inflamatoria idiopatica del registro REMICAM de la Sociedad de Reumatologia de la Comunidad de Madrid (SORCOM). Metodos Estudio descriptivo retrospectivo multicentrico de una cohorte de pacientes con diagnostico de miositis inflamatoria idiopatica en seguimiento en servicios de reumatologia de hospitales de la Comunidad de Madrid entre enero de 1980 y diciembre de 2014. Se han recogido hasta un total de 313 variables acerca de aspectos demograficos, clinicos y de morbimortalidad, y se ha realizado una comparacion entre subgrupos clinicos. Resultados Se han reclutado 479 pacientes procedentes de 12 centros, con un 14% de perdidas durante el periodo de seguimiento. El 74% de los casos eran mujeres, una edad al diagnostico de 44 ± 23 anos, y una media de seguimiento de 10 ± 8 anos. Los subgrupos clinicos mas frecuentes fueron las formas primarias (PM 29%, DM 22%), seguidas de sindrome de solapamiento (20,5%), miopatias juveniles (18%), miopatias asociadas a cancer (8%), miopatias necrosantes inmunomediadas (1%) y miositis por cuerpos de inclusion (1%). Durante el periodo de seguimiento se produjeron un total de 114 fallecimientos (28%), siendo las principales causas el cancer (24%), las infecciones (23%) y los eventos cardiovasculares (21%). Conclusiones En el registro REMICAM de miopatias inflamatorias de la Comunidad de Madrid se han reclutado 479 casos de miositis inflamatoria idiopatica con datos sociodemograficos, clinicos y pronosticos, suponiendo el mayor registro multicentrico espanol en el ambito de la Reumatologia hasta la fecha, y constituyendo una fuente importante para la realizacion de posteriores subestudios.

The present study was undertaken to assess mortality, causes of death, and associated prognostic factors in a large cohort of patients diagnosed with idiopathic inflammatory myositis (IIM) from Spain. A retrospective longitudinal study was carried out in 467 consecutive patients with IIM, identified from 12 medical centers. Patients were classified as primary polymyositis, primary dermatomyositis (DM), overlap myositis, cancer-associated myositis (CAM), and juvenile idiopathic inflammatory myopathies. A total of 113 deaths occurred (24%) after a median follow-up time of 9.7 years. In the overall cohort, the 2-, 5-, and 10-year survival probabilities were 91.9, 86.7, and 77%, respectively. Main causes of death were infections and cancer (24% each). Multivariate model revealed that CAM (HR = 24.06), OM (HR = 12.00), DM (HR = 7.26), higher age at diagnosis (HR = 1.02), severe infections (HR = 3.66), interstitial lung disease (HR = 1.61), and baseline elevation of acute phase reactants (HR = 3.03) were associated with a worse prognosis, while edema of the hands (HR = 0.39), female gender (HR = 0.39), and longer disease duration (HR = 0.73) were associated with a better prognosis. The standardized mortality ratio was 1.56 (95% CI 1.28-1.87) compared to the Spanish general population. Our findings indicate that IIM has a high long-term mortality, with an excess of mortality compared to the Spanish population. A more aggressive therapy may be required in IIM patients presenting with poor predictive factors. Sin financiación 1.952 JCR (2017) Q3, 24/32 Rheumatology 0.906 SJR (2017) Q2, 25/66 Rheumatology No data IDR 2017 UEM

espanolObjetivo Desarrollar recomendaciones basadas en la mejor evidencia y experiencia sobre el manejo de pacientes con enfermedades reumaticas inflamatorias y autoinmunes durante la edad fertil, el embarazo, posparto y lactancia. Metodos Se siguio la metodologia de grupos nominales. Se selecciono un grupo nominal de expertos (12 reumatologos). Se realizo una actualizacion de una revision sistematica de la literatura, una revision literaria, asi como una encuesta a nivel nacional sobre el manejo de estos pacientes. El grupo de expertos se encargo de definir el alcance, usuarios, apartados del manuscrito y posibles recomendaciones. El GA con las recomendaciones se voto siguiendo la metodologia Delphi segun una escala de 1 (total desacuerdo) a 10 (total acuerdo), definiendose el acuerdo como una puntuacion ≥ 7 por al menos el 70% de los participantes. El NE y GR se clasificaron segun el modelo del Center for Evidence Based Medicine de Oxford. El documento completo inicial fue revisado por los expertos y el proyecto estuvo coordinado, en todo momento, por un metodologo experto. Resultados Se generaron 14 recomendaciones sobre el periodo preconcepcional (anticoncepcion, reproduccion asistida), el embarazo (planificacion, manejo farmacologico y seguimiento) y lactancia (manejo farmacologico y seguimiento). Incluye recomendaciones especificas sobre situaciones de especial riesgo como el lupus eritematoso sistemico y el sindrome antifosfolipido. Existio acuerdo > 90% con todas las recomendaciones menos en una de ellas. Conclusiones En los pacientes con enfermedades inflamatorias y autoinmunes estas actuaciones pueden mejorar los resultados y el pronostico de los mismos. EnglishObjective To develop recommendations on the evaluation and management of patients with rheumatic autoimmune and inflammatory diseases during the reproductive age, pregnancy, post-partum and breastfeeding based on the best evidence and experience. Methods Recommendations were generated using nominal group and Delphi techniques. An expert panel of 12 rheumatologists was established. A systematic literature review and a narrative review (websites, clinical guidelines and other relevant documentation) were performed and presented to the panel in its 1st meeting to be discussed and to help define recommendations. A first draft of recommendations was generated and circulated for comments and wording refinement. A national survey analyzing different aspects of this topic was undertaken separately, followed by a Delphi process (2 rounds). Agreement with each recommendation was ranked on a scale of 1 (total disagreement) to 10 (total agreement), and was considered to be achieved if at least 70% voted ≥ 7. The level of evidence and grade of recommendation were assessed using the Oxford Centre for Evidence-based Medicine Levels of Evidence. Results A total of 14 recommendations were generated for the preconception period (oral and hormonal contraception, reproductive techniques), pregnancy (planning, treatment and follow-up), and breastfeeding (treatment and follow-up). High-risk situations such as lupus or antiphospholipid syndrome were included. A consensus > 90% was reached for all but one recommendation. Conclusions These recommendations are intended to provide rheumatologists, patients, families and other stakeholders with a consensus on the evaluation and management of patients with autoimmune and inflammatory diseases during the reproductive age, pregnancy, postpartum and breastfeeding.

Objective To develop recommendations on the evaluation and management of patients with rheumatic autoimmune and inflammatory diseases during the reproductive age, pregnancy, post-partum and breastfeeding based on the best evidence and experience. Methods Recommendations were generated using nominal group and Delphi techniques. An expert panel of 12 rheumatologists was established. A systematic literature review and a narrative review (websites, clinical guidelines and other relevant documentation) were performed and presented to the panel in its 1st meeting to be discussed and to help define recommendations. A first draft of recommendations was generated and circulated for comments and wording refinement. A national survey analyzing different aspects of this topic was undertaken separately, followed by a Delphi process (2 rounds). Agreement with each recommendation was ranked on a scale of 1 (total disagreement) to 10 (total agreement), and was considered to be achieved if at least 70% voted ≥7. The level of evidence and grade of recommendation were assessed using the Oxford Centre for Evidence-based Medicine Levels of Evidence. Results A total of 14 recommendations were generated for the preconception period (oral and hormonal contraception, reproductive techniques), pregnancy (planning, treatment and follow-up), and breastfeeding (treatment and follow-up). High-risk situations such as lupus or antiphospholipid syndrome were included. A consensus >90% was reached for all but one recommendation. Conclusions These recommendations are intended to provide rheumatologists, patients, families and other stakeholders with a consensus on the evaluation and management of patients with autoimmune and inflammatory diseases during the reproductive age, pregnancy, postpartum and breastfeeding.

Objective Arthritis, myositis and interstitial lung disease (ILD) constitute the classic clinical triad of anti-synthetase syndrome (ASSD). These patients experience other accompanying features, such as Raynaud's phenomenon, fever or mechanic's hands. Most ASSD patients develop the complete triad during the follow-up. In the present study we aimed to determine whether the subsequent appearance of accompanying features may suggest the development of triad findings lacking at the onset in anti-Jo1 positive ASSD patients. Methods Anti-Jo1 positive patients presenting with incomplete ASSD (no >2 classic triad features) were assessed. Clinical characteristics and clusters of disease manifestations were retrospectively collected and analyzed in a large international multicenter cohort of ASSD patients. Results 165 patients (123 women) with incomplete ASSD were identified. Ninety-five patients (57.5%) developed new classic triad manifestations after 15months median (IQR 9–51) and 40 (24%) developed new accompanying features after 19months median (IQR 6–56) from disease onset. During the follow-up, the ex-novo occurrence of triad features was observed in 32 out of 40 patients (80%) with new accompanying findings and in 63 out of 125 patients (50.5%) without new accompanying findings (p=0.002). In patients with at least one new accompanying feature the odds ratio for the occurrence of new triad manifestations was 3.94 with respect to patients not developing ex-novo accompanying findings (95% CI 1.68–9.21, p=0.002). Conclusion Anti-Jo1 ASSD patients with incomplete forms at disease onset are at high risk for the subsequent occurrence of lacking classic triad findings. Although all ASSD patients should be carefully assessed for the occurrence of new triad features, a closer follow-up should be considered in the subgroup of patients developing ex novo accompanying findings. These patients, indeed, have near four-fold increased risk for new classic triad manifestation occurrence with respect to patients not presenting ex novo accompanying findings.

Objective Salivary gland ultrasound (SGU) is a reliable technique for assessing the salivary glands in patients with primary Sjögren’s syndrome (pSS). The aim of this study was to elucidate the relationship between SGU findings and autoimmunity in patients with pSS. Methods Patients with pSS underwent an SGU assessment. The patients were classified into three groups according to their autoimmunity profile: the complete positive group (positive rheumatoid factor, antinuclear antibodies, and anti-Ro/anti-La antibodies), the partial seropositive group (positivity of at least one autoantibody but not all), and the seronegative group. Results In total, 93 patients were evaluated. Eighty-six (92.5%) were female, and their median age was 49.5 years. The median disease duration was 12.3 years. Pathological SGU findings were present in 32 (34.4%) patients [25 of 36 (78.1%) in the complete positive group and 7 of 44 (21.9%) in the partial positive group]. Patients with pathological SGU findings had a shorter disease duration and slightly higher European League Against Rheumatism Sjögren’s syndrome disease activity index. Conclusions The autoimmunity profile and pathological SGU findings are strongly associated with each other in patients with pSS. However, the disease duration does not seem to be related to pathological SGU findings.

This study aimed at determining socio-demographic and clinical factors of primary Sjögren syndrome (pSS) associated with osteoporosis (OP) and fragility fracture. SJOGRENSER is a cross-sectional study of patients with pSS, classified according to American European consensus criteria developed in 33 Spanish rheumatology departments. Epidemiological, clinical, serological and treatment data were collected and a descriptive analysis was conducted. Bivariate and multivariate analyses were performed using a binomial logistic regression to study the factors associated with OP and fragility fracture in pSS. 437 patients were included (95% women, with a median age of 58.6 years). 300 women were menopausal (76.4%). Prevalence of OP was 18.5% [in men (N = 21) this measured 19%]. A total of 37 fragility fractures were recorded. In the multivariate analysis, there was an association between OP and age: in the 51-64 age range (menopausal women), the OR measured 9.993 (95% CI 2301-43,399, p = 0.002); In the age 64 years group, OR was 20.610 (4.679-90.774, p 0.001); between OP and disease duration, OR was 1.046 (1.008-1085, p = 0.017); past treatment with corticosteroids, OR 2.548 (1.271-5.105, p = 0.008). Similarly, an association was found between fragility fractures and age: in the 51-64 age group, OR measured 5.068 (1.117-22,995, p = 0.035), age 64 years, OR was 7.674 (1.675-35,151, p 0.009); disease duration, OR 1.049 (CI 1.003-1097, p 0.036) and the ESSDAI index, OR 1.080 (1.029-1134, p = 0.002). Patients with pSS can develop osteoporosis and fragility fractures over the course of the disease. Age, corticosteroids treatment and disease duration were associated with the development of OP. Disease duration and ESSDAI were associated with the development of fractures in patients with pSS.

Objective: To describe the incidence of bacteremia in a large multicentric cohort of patients with systemic lupus erythematosus (SLE) and their clinical characteristics and to identify risk factors. Methods: All bacteremic episodes from the Spanish RELESSER registry were included. Clinical and laboratory characteristics concerning bacteremia and SLE status, as well as comorbidities at the time of infection, were retrospectively collected. A comparison with sex- and age-matched SLE controls without bacteremia was made. A logistic regression was conducted. Results: The study included 114 episodes of bacteremia in 83 patients. The incidence rate was 2.7/1000 patient-years. At the time of bacteremia, the median age was 40.5 (range: 8-90) years, and 88.6% of patients were female. The Safety of Estrogens in Lupus Erythematosus National Assessment-Systemic Lupus Erythematosus Disease Activity Index was 4 [interquartile range (IQR) 8]; 41% had an SLE flare (66% severe); Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index was 3 (IQR 4). A comorbidity was recorded in 64% of cases. At the time of bacteremia, 88.6% received corticosteroids (68.6% > 10 mg/day) and 57% immunosuppressors. Gram-negative bacilli, most frequently Escherichia coli (29.8%), caused 52.6% of the episodes. The bacteremia-related mortality was 14% and bacteremia was recurrent in 27.2% of cases. A dose-response relationship was found between corticosteroids and bacteremia risk. In the multivariate analysis, these factors were associated with bacteremia: elevated creatinine (OR 1.31, 95% CI 1.01-1.70; p = 0.045), diabetes (OR 6.01, 95% CI 2.26-15.95; p < 0.001), cancer (OR 5.32, 95% CI 2.23-12.70; p < 0.001), immunosuppressors (OR 6.35, 95% CI 3.42-11.77; p < 0.001), and damage (OR 1.65, 95% CI 1.31-2.09; p < 0.001). Conclusion: Bacteremia occurred mostly in patients with active SLE and was frequently associated with severe flares and corticosteroid use. Recurrence and mortality were high. Immunosuppressors, comorbidities, and disease-related damage were associated with bacteremia. This work was supported by the Spanish Foundation of Rheumatology. JMPR is supported by grant 316265 (BIOCAPS) from the European Union 7th Framework Programme (FP7/ REGPOT-2012-2013.1) and FIS/ISCIII-Fondo Europeo de Desarrollo regional (FEDER) (Grant number PI11/02857).

MUC5B rs35705950 (G/T) is strongly associated with idiopathic pulmonary fibrosis (IPF) and also contributes to the risk of interstitial lung disease (ILD) in rheumatoid arthritis (RA-ILD) and chronic hypersensitivity pneumonitis (CHP). Due to this, we evaluated the implication of MUC5B rs35705950 in antisynthetase syndrome (ASSD), a pathology characterised by a high ILD incidence. 160 patients with ASSD (142 with ILD associated with ASSD [ASSD-ILD+]), 232 with ILD unrelated to ASSD (comprising 161 IPF, 27 RA-ILD and 44 CHP) and 534 healthy controls were genotyped. MUC5B rs35705950 frequency did not significantly differ between ASSD-ILD+ patients and healthy controls nor when ASSD patients were stratified according to the presence/absence of anti Jo-1 antibodies or ILD. No significant differences in MUC5B rs35705950 were also observed in ASSD-ILD+ patients with a usual interstitial pneumonia (UIP) pattern when compared to those with a non-UIP pattern. However, a statistically significant decrease of MUC5B rs35705950 GT, TT and T frequencies in ASSD-ILD+ patients compared to patients with ILD unrelated to ASSD was observed. In summary, our study does not support a role of MUC5B rs35705950 in ASSD. It also indicates that there are genetic differences between ILD associated with and that unrelated to ASSD, This study was partially supported by grants from the Foundation for Research in Rheumatology (FOREUM). RL-M is a recipient of a Miguel Servet type I programme fellowship from the ‘Instituto de Salud Carlos III’ (ISCIII), cofunded by the European Social Fund (ESF, ‘Investing in your future’) (grant CP16/00033). SR-M is supported by funds of the RETICS Program (RD16/0012/0009), co-funded by the European Regional Development Fund (ERDF). VP-C is supported by a pre-doctoral grant from IDIVAL (PREVAL 18/01). VM is supported by funds of a Miguel Servet type I programme (grant CP16/00033) (ISCIII, co-funded by ESF). LL-G is supported by funds of PI18/00042 (ISCIII, co-funded by ERDF). OG is Staff Personnel of Xunta de Galicia (Servizo Galego de Saude, SERGAS) through a research-staff stabilization contract (ISCIII/SERGAS). OG,is member of RETICS Programme, RD16/0012/0014 (RIER: Red de Investigación en Inflamación y Enfermedades Reumáticas) via Instituto de Salud Carlos III (ISCIII) and FEDER. The work of OG (PI17/00409), was funded by Instituto de Salud Carlos III and FEDER. OG is a beneficiary of a project funded by Research Executive Agency of the European Union in the framework of MSCA-RISE Action of the H2020 Programme (Project number 734899). OG is beneficiary of a grant funded by Xunta de Galicia, Consellería de Educación, Universidade e Formación Profesional and Consellería de Economía, Emprego e Industria (GAIN), GPC IN607B2019/10

Background Little is known about the patient related outcomes and disease activity in systemic lupus erythematosus. Damage and impact are issues that have not been associated with activity in lupus previously. Objectives In a large cohort of systemic lupus erythematosus (SLE) patients, to evaluate the association between a Patient Acceptable Symptom State (PASS) and three different domains of the disease: activity, damage and impact of SLE on patients’ lives Methods Cross-sectional study of 1,364 SLE (ACR 97 Criteria) patients from the Spanish Society of Rheumatology Lupus Registry (RELESSER). Patients were clinically assessed by the physicians, who collected data about demographics, clinical manifestations, laboratory features and therapies. SLE activity was assessed by using the SLEDAI (SLE Disease Activity Index). The Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR DI) was used to measure damage accrual. The impact of SLE on patients’ lives was evaluated by using the Lupus Impact Tracker (LIT). The PASS question “Considering all the different ways your disease is affecting you, if you would stay in this state for the next months, do you consider that your current state is satisfactory?” was answered in a dichotomized way: yes/no. Measures of central tendency and spread were used to describe responses and T-test was used to evaluate the independence between PASS and the other questionnaires and indices. Results Mean age (years) ± S.D. of the 1,364 patients included in the study was 49.9 ± 13.3; 90.3% were female and 94.9% were Caucasian. In the total of patients, 979 (71.8%) were in PASS. Patients in PASS had significantly lower activity (SLEDAI Score) than those not in PASS (2.4 ± 3.2 vs. 3.0 ± 3.9, respectively; p=0.007). The SLICC/ACR DI was significantly lower in the group of patients in PASS compared to those without PASS (0.64 ± 1.1 vs. 0.81 ± 0.21, respectively; p=0.022). SLE had a significantly lower impact on patients in PASS in comparison to those without PASS (LIT Score: 8.47 ± 7.5 vs. 19.8 ± 8.34, respectively; p=0.001). Conclusion In the largest observational European Registry of SLE patients, PASS was associated with other domains of the disease: activity, damage and impact on patients’ life. PASS is a simple and reliable patient-reported outcome that can be a useful tool to evaluate patients’ well-being. Disclosure of Interests Tomas Vazquez Rodriguez: None declared, Inigo Rua Figueroa: None declared, Victor Del Campo Perez: None declared, J. Narvaez Consultant for: Bristol-Myers Squibb, Francisco J Lopez-Longo: None declared, Maria Galindo-Izquierdo: None declared, Jaime Calvo-Alen: None declared, Antonio Fernandez-Nebro: None declared, Juan Ovalles: None declared, Pilar Rubio Munoz: None declared, Eva Tomero Muriel: None declared, Coral Mourino Rodriguez: None declared, Esther Uriarte Isacelaya: None declared, Angela Pecondon-Espanol: None declared, Mercedes Freire Gonzalez: None declared, Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Marian Gantes Mora: None declared, Monica Ibanez Barcelo: None declared, Carlos A. Montilla-Morales: None declared, Jose M Pego-Reigosa: None declared

Background: In inflammatory diseases, such as rheumatoid arthritis, early treatment has demonstrated to improve disease control and decrease mortality. It is not known if an early treatment could have similar effects on inflammatory myositis (IM) patients. Objectives: To analyze if the delay between initial symptoms and diagnosis or therapy initiation influences disease control or mortality in IM patients. Methods: All patients from REMICAM were included (1). The influence of time elapsed between initial symptoms and diagnosis or start of therapy, and the year of diagnosis (before or after 2000), on mortality and disease control (defined as the possibility of glucocorticoid (GC) withdrawal due to IM improvement), was studied by means of survival analysis and regression proportional hazard bi and multivariate Cox models. All factors with p Results: From 479 patients (74% females, 52% polimiositis, 44±22 years at diagnosis, 10±8 years follow up), 208 (43%) were diagnosed before 2000, and 473 (99%) received any treatment: 473 (99%) oral GC, 50/344 (15%) GC pulses, 78 (16%) HCL, 355 (74%) immunosuppressants (IS) (MTX 228, LF 10, AZA 190, CYP 44, MP 38, CyS 32), 55 (12%) biologics (RTX 45, abatacept 2, aTNF 16) and 79 (17%) IVIG. Disease control was more frequent in younger patients, diagnosed after year 2000, and was independently associated with less ILD presence, lower n° of therapies and a shorter time between the initial symptoms and the start of IS therapy (Table 1). Risk factors for mortality were age, male sex and cancer, but not the delay in diagnosis or in the start of therapy (Table 2) Conclusion: In the REMICAM cohort, disease control is more frequent in younger patients, diagnosed after 2000, with early initiation of IS therapy. Mortality was not associated with the delay in diagnosis or therapy in our study. Our data support the hypothesis that, in IM, as in other inflammatory diseases, an early treatment is essential to decrease inflammation and achieve disease control. Reference: [1] Nuno L, Rheumatol Clin 2017; 13:331-337 Disclosure of Interests: Beatriz Joven-Ibanez Speakers bureau: Celgene, Novartis, MSD, Pfizer, AbbVie, and Janssen, Laura Nuno: None declared, Francisco J Lopez-Longo: None declared, Julia Martinez-Barrio: None declared, Carmen Larena: None declared, Valentina Maldonado : None declared, Carmen Barbadillo: None declared, Paloma Garcia de la Pena: None declared, Irene Llorente : None declared, Eva Tomero Muriel: None declared, Ana Perez Gomez: None declared, Henry Moruno : None declared, Tatiana Cobo-Ibanez: None declared, RAQUEL ALMODOVAR: None declared, LETICIA LOJO : None declared, Maria Jesus Garcia de Yebenes: None declared, Patricia Carreira: None declared

Background: Juvenile idiopathic inflammatory myopathies (JIIM) are a heterogeneous group of autoimmune diseases affecting children, characterized by symmetric muscular weakness, cutaneous rash and systemic organ involvement. Given its low incidence, there are few studies describing the characteristics of this disease and its subtypes in Spanish patients1. Objectives: To describe the demographic, clinical and analytical characteristics of patients with JIIM from the registry of inflammatory myopathies in Madrid community (REMICAM), and to compare those measures between polymyositis (PM) and juvenile dermatomyositis (JDM) subgroups. Methods: A multicentre retrospective study from the REMICAM registry was performed. Patients were selected if they were 18 years or younger at onset of JIIM and met definite or probable criteria for IIM by the modified Bohan and Peter criteria. We included patients with JDM or PM subgroups, overlap myositis patients were excluded. Results: 86 patients were included, 12 classified as PM and 74 as JDM. 70% were women and 96% were Caucasian. Mean age at diagnosis was 11.8 years in PM group vs 7.2 years in JDM group. 44% presented arthritis and 93% presented muscular weakness. Gottron sign was present in 76% of the patients, and calcinosis was present in 31.4%. Cardiac and pulmonary manifestations were rare ( Conclusion: JDM was the most frequent form of MIIJ in our study (86%). The most frequent manifestations were the muscular and dermatological ones, but an important group also presented arthritis and fever. There was no statistical difference between both groups, regardless, myalgias and dysphagia were more common in JDM group, and they had higher CPK and aldolase values. PM patients were older, had more fever and arthritis, also, cytopenia and ANA positivity were more common. Reference: [1] Shah M, Mamyrova G, Targoff IN, Huber AM, Malley JD, Rice MM, et al. The Clinical Phenotypes of the Juvenile Idiopathic Inflammatory Myopathies. Medicine (Baltimore). 2013 Jan;92(1):25–41. Disclosure of Interests: Jesus Loarce-Martos: None declared, Carmen Larena: None declared, M. Angeles Blazquez: None declared, Beatriz Joven-Ibanez Speakers bureau: Celgene, Novartis, MSD, Pfizer, AbbVie, and Janssen, Patricia Carreira: None declared, Francisco J Lopez-Longo: None declared, Julia Martinez-Barrio: None declared, J.C. Lopez Robledillo: None declared, RAQUEL ALMODOVAR: None declared, Maria Jesus Garcia de Yebenes: None declared, Laura Nuno: None declared

Background Uveitis is one of the major causes of disability of Behcet’s disease (BD). According to the “Expert panel recommendations”, anti-TNF therapy with infliximab (IFX) or adalimumab (ADA) may be considered as first- or second-line therapy for patients with BD-ophthalmic manifestations. Objectives To compare IFX versus ADA as first biologic drug in refractory uveitis due to BD for 1-year period. Methods Multicenter study of BD-associated uveitis refractory to conventional non-biologic treatment. Dosing schedule: IFX 3-5 mg/kg iv at 0, 2 and 6 weeks and then every 4-8 week, and ADA 40 mg/sc/every other week without loading dose. Main comparative outcome measures: safety and efficacy, assessing the intraocular inflammation, macular thickness, visual acuity, degree of immunosuppression load, drug retention, and glucocorticoid-sparing effect. Results 177 patients (316 affected eyes) were included. IFX was used in 103 and ADA in 74. No significant differences at baseline were observed regarding main demographic features, previous therapy and ocular severity. After 1 year of therapy, we observed an improvement in all ocular parameters in IFX vs ADA groups: AC inflammation (78.18% vs 92.31%), vitritis (78.95% vs 93.33%), retinal vasculitis (97% vs 95%), macular thickness (264.89±59.74 vs 250.62±36.85), and BCVA (0.67±0.34 vs 0.81±0.26). Drug withdrawal was observed in 57 (55.33%) of IFX group and in 21 (28.37%) of ADA group. Conclusion After 1 year of therapy in refractory BD-associated uveitis, ADA showed a statistically better outcome than IFX in improvement of BCVA, vitritis and drug retention. References [1] Levy-Clarke G, et al. Expert panel recommendations for the use of anti-tumor necrosis factor biologic agents in patients with ocular inflammatory disorders. Ophthalmology. 2014; 121:785-96. [2] Santos-Gomez M, et al. The effect of biologic therapy different from infliximab or adalimumab in patients with refractory uveitis due to Behcet’s disease: results of a multicentre open-label study. Clin Exp Rheumatol 2016; 34 (6 Suppl 102): S34-40 [3] Atienza-Mateo B, et al. Anti-IL6-Receptor Tocilizumab in Refractory Uveitis Associated With Behcet’s Disease. Multicenter Retrospective Study. Rheumatology (Oxford). 2018 May 1;57(5): 856-864. [4] Martin-Varillas JL, et al. Successful Optimization of Adalimumab Therapy in Refractory Uveitis Due to Behcet’s Disease. Ophthalmology. 2018 Mar 27. Disclosure of Interests Belen Atienza-Mateo: None declared, Jose Luis Martin-Varillas: None declared, Vanesa Calvo-Rio: None declared, Rosalia Demetrio-Pablo: None declared, Emma Beltran: None declared, Juan Sanchez-Burson: None declared, Marina Mesquida: None declared, Alfredo Adan : None declared, Victoria Hernandez: None declared, Marisa Hernandez-Garfella: None declared, Elia Valls-Pascual: None declared, Lucia Martinez-Costa: None declared, Agusti Sellas-Fernandez: None declared, Miguel Cordero-Coma: None declared, Manuel Diaz-Llopis: None declared, Roberto Gallego: None declared, Jose L. Garcia-Serrano: None declared, Norberto Ortego: None declared, Jose M. Herreras: None declared, Alejandro Fonollosa: None declared, Angel Garcia-Aparicio: None declared, Olga Maiz-Alonso Speakers bureau: Pfizer, Ana Blanco: None declared, Ignacio Torre-Salaberri: None declared, Cruz Fernandez-Espartero: None declared, Vega Jovani: None declared, Diana Peiteado: None declared, Esperanza Pato: None declared, Juan Cruz: None declared, Carlos Fernandez-Cid: None declared, Elena Aurrecoechea: None declared, Miriam Garcia-Arias: None declared, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Miguel A. Caracuel-Ruiz: None declared, Carlos A. Montilla-Morales: None declared, Antonio Atanes-Sandoval: None declared, Felix Francisco: None declared, Santos Insua: None declared, Senen Gonzalez-Suarez: None declared, Amalia Sanchez-Andrade: None declared, Fernando Gamero: None declared, Luis F. Linares Ferrando: None declared, Fredeswinda Romero: None declared, A. Javier Garcia-Gonzalez: None declared, RAQUEL ALMODOVAR: None declared, Enrique Minguez: None declared, Carmen Carrasco-Cubero: None declared, Alejandro Olive: None declared, Julio Vazquez: None declared, Oscar Ruiz-Moreno: None declared, Fernando Jimenez-Zorzo: None declared, Javier Manero: None declared, Santiago Munoz Fernandez: None declared, Cristina Fernandez-Carballido: None declared, Esteban Rubio-Romero: None declared, Fred Anton Pages: None declared, Francisco J. Toyos Saenz de Miera: None declared, Myriam Gandia Martinez: None declared, David Diaz Valle: None declared, Francisco J Lopez-Longo: None declared, Joan Miquel Nolla: None declared, Enrique Raya: None declared, Marcelino Revenga: None declared, J. Luis Hernandez: None declared, Monica Calderon-Goercke: None declared, D. Prieto-Pena: None declared, Inigo Gonzalez-Mazon: None declared, Lara Sanchez Bilbao: None declared, Miguel A. Gonzalez-Gay: None declared, Ricardo Blanco: None declared

Background Inflammatory myositis (IM) are heterogeneous autoimmune diseases, characterized by muscular inflammation, which can associate systemic manifestations. In other inflammatory diseases, as rheumatoid arthritis, inflammation is a cardiovascular risk factor (CVRF), with clear influence on cardiovascular events and mortality (CVE, CVM). Objectives To analyze the influence of IM severity in the development of CVE and CVM in the REMICAM registry. Methods All patients from REMICAM were included(1). REMICAM is a retrospective multicentric registry of IM patients (n=479), performed in Madrid between 2012 and 2014, containing demographic, clinical and outcome data (1). The presence of extra muscular features (cardiac, pulmonary, cutaneous and systemic involvement), number of therapies, immunosuppressants (IS) need, and glucocorticoids (GC) withdrawal due to IM remission defined IM severity. Bi and multivariate logistic regression analysis were used to determine the association between these factors and CVE. Survival analysis and regression proportional hazard bi and multivariate Cox models were used to determine the effect of these factors on CVM. All factors with p Results From 479 patients (74% females, 52% polymyositis, 44±22 years at diagnosis, 10±8 years follow up), 104/467 (22%) presented CVE and 24/409 (6%) died due to CVE. CVE were associated to age, male sex, CVRF number, diagnosis before year 2000, presence of arrhythmia or Raynaud (Table 1). Arrhythmia was the only independent risk factor for CVM. GC withdrawal due to IM remission, and a smaller number of therapies, were protective factors for CVM (Table 2). Conclusion In the REMICAM registry, extra muscular manifestations such as arrhythmia, were associated to CVE and CVM, as independent risk factors. A smaller number of treatment or GC withdrawal due to remission were protective factors for CVM. Our results support the hypothesis that more severe IM, with extra muscular involvement and persistent inflammation, could favor atherosclerosis and CVE development. Reference [1] Nuno L, Rheumatol Clin2017; 13:331-337 Disclosure of Interests Beatriz Joven-Ibanez Speakers bureau: Celgene, Novartis, MSD, Pfizer, AbbVie, and Janssen, Laura Nuno: None declared, Francisco J Lopez-Longo: None declared, Julia Martinez-Barrio: None declared, Carmen Larena: None declared, Valentina Maldonado : None declared, Carmen Barbadillo: None declared, Paloma Garcia de la Pena: None declared, Irene Llorente : None declared, Eva Tomero Muriel: None declared, Ana Perez Gomez: None declared, Henry Moruno : None declared, Tatiana Cobo-Ibanez: None declared, RAQUEL ALMODOVAR: None declared, LETICIA LOJO : None declared, Maria Jesus Garcia de Yebenes: None declared, Patricia Carreira: None declared

Background Although glucocorticoids (GC) remain the cornerstone in the treatment of inflammatory myositis (IM), the management of these processes is not yet standardized. Experts recommend early use of immunosuppressants (IS), in order to prevent GC side effects. It is not known what is the actual management in clinical practice. Objectives To describe the management of IM in REMICAM registry, and to identify differences according to disease subtype (polymyositis (PM), or dermatomyositis (DM)), and to the year of diagnosis (before or after 2000). Methods All patients from REMICAM were included (1). A descriptive analysis of the different therapies (GC, IS, biologics, IV immunoglobulins (IVIG), and hydroxychloroquine HCL) was performed, attending to possible differences between IM subtype and the year of diagnosis. T student and Chi square tests were used for quantitative and qualitative variables, respectively. Results From 479 patients (74% females, 52% PM, 44±22 y at diagnosis, 10±8 y follow up), 473 (99%) received oral GC, 50/344 (15%) pulses GC, 78 (16%) HCL, 355 (74%) IS (methotrexate MTX 228, leflunomide 10, azathioprine AZA190, cyclophosphamide 44, mycophenolate MP 38, cyclosporine 32), 55 (12%) biologics (rituximab 45, abatacept 2, aTNF 16) and 79 (17%) IVIG. Differences according to IM subtype (1), and to the year of diagnosis (2), are shown in Tables. Conclusion The therapeutic management of IM has changed over the last few years, with a clear increase and earlier use of IS in patients diagnosed after 2000. In spite of a more aggressive approach in recent years, severe infections are less frequent, perhaps as a consequence of lower GC doses. The management of PM and DM is similar. Reference [1] Nuno L, Rheumatol Clin2017; 13:331-337 Disclosure of Interests Beatriz Joven-Ibanez Speakers bureau: Celgene, Novartis, MSD, Pfizer, AbbVie, and Janssen, Laura Nuno: None declared, Francisco J Lopez-Longo: None declared, Julia Martinez-Barrio: None declared, Carmen Larena: None declared, Valentina Maldonado : None declared, Carmen Barbadillo: None declared, Paloma Garcia de la Pena: None declared, Irene Llorente : None declared, Eva Tomero Muriel: None declared, Ana Perez Gomez: None declared, Henry Moruno : None declared, Tatiana Cobo-Ibanez: None declared, RAQUEL ALMODOVAR: None declared, LETICIA LOJO : None declared, Maria Jesus Garcia de Yebenes: None declared, Patricia Carreira: None declared

Background The DAS28 forms the mainstay of current RA and other chronic inflammatory arthritis (CIA) [i.e. peripheral spondyloarthritis (SpA)] management in clinical practice. DAS28 has certain limitations. Some joints especially the shoulders are difficult to evaluate correctly using this method. The shoulder is deep and highly affected by rotator cuff tendon lesions either due to degenerative or inflammatory arthritis This structural damage can produce pain and swelling. A swollen shoulder joint can be difficult to palpate by physical examination. Using a clinical approach can be inaccurate to ascertain whether shoulder involvement is actually due to true RA-derived inflammatory activity or other degenerative or structural causes. Objectives To investigate the added value of adding a US assessment of shoulders to DAS28 scores and on disease activity status, in patients with CIA (RA or peripheral SpA) in either disease remission or low disease states who had shoulder pain. Methods Patients were recruited prospectively over a 3-month period. Each patient had a standard DAS28 performed followed by a formal physical shoulder examination, of both shoulders, including testing active and passive range of movements. A complete US examination of both shoulders was carried out by a rheumatologist experienced in this technique. All patients were examined using the same real-time US machine (Esaote MyLab Twice) using a linear probe, 3-13 MHz frequency and 57% gain. The original DAS28 was recalculated (i.e. US-modified DAS28) according to the presence/absence of inflammatory findings on shoulder US. Results Thirty-eight patients [82% females; mean (± SD) age 60.3 (11.96) years] were included. In 33 out of 38 (87% [CI95%: 76-98%]) patients the original DAS28 was greater than the US-modified DAS28. This percentage was significantly greater than 50% (p Conclusion This study showed that patients improved their disease activity scores and status after an US assessment of their shoulders. A low percentage increased their disease status after shoulder US which can be also important for local or systemic therapeutic decisions. Based on this study, we suggest that shoulder US can provide valuable additional information to the DAS28 when evaluating disease activity in CIA patients. References [1] Mierau M, Schoels M, Gonda G, Fuchs J, Aletaha D, Smolen JS. Assessing remission in clinical practice. Rheumatology (Oxford)2007;46: 975-9. [2] Naredo E, Aquado P, De Miguel E, Uson J, Mayordomo L, Gijon-Banos J, Martin-Mola E. Painful shoulder: a comparison of physical examination and ultrasonagraphic findings. Ann Rheum Dis2002; 61:132-6.. [3] Micheroli R, Kyburz D, Ciurea A, Dubs B, Bisig SP, Tamborrini G. Correlation of findings in clinical and high resolution ultrasonography examinations of the painful shoulder. J Ultrason2015; 15: 29–44. Acknowledgement N/A Disclosure of Interests Qasim Akram Consultant for: Roche/Chugai, Lara Valor : None declared, Iustina Janta: None declared, Juan Carlos Nieto: None declared, Letitia Garcia: None declared, Belen Serrano: None declared, Teresa Pedrosa: None declared, Francisco J Lopez-Longo: None declared, Indalecio Monteagudo Saez: None declared, Esperanza Naredo Consultant for: Abbvie, Speakers bureau: AbbVie, Roche, Bristol-Myers Squibb, Pfizer, UCB, Lilly, Novartis, Janssen, and Celgene GmbH

Background Primary Sjogren’s syndrome (pSS) is a systemic autoimmune disease characterized by the involvement of the exocrine glands, mainly salivary and lacrimal glands. Approximately half of patients will experience extraglandular complications throughout their evolution. Objectives To characterize the involvement of the nervous system (NS) in patients with pSS. Methods Multicenter cross-sectional study of a cohort of patients with pSS, constructed by random selection of pSS patients fulfilling the 2002 American-European Consensus Criteria for pSS1 from 33 rheumatology units. Through review of clinical records and interview with patients, demographic, clinical, analytical, therapeutic data and disease activity indexes were collected. Univariate analysis was done by Chi square test, Mann-Whitney U test and Student’s t test. Multivariate analysis was done by linear logistic regression. A p Results 437 patients were included (95% women, median age of 58 years). 65 patients developed NS involvement: 26 patients central NS, 31 peripheral NS and 8 both. Multivariate analysis showed association between NS involvement and atherosclerotic stroke (OR 10.4, 95% CI 2.8-38.5), ear disease (OR 2.1, 95% CI 1.1-4.2) and myopathy (OR 6.5, 95% CI 1.3-31,6). Patients with NS involvement had higher probability of being treated with glucocorticoids (OR 3.3, 95%CI 1.5-7.2) and cyclophosphamide (OR 6.8, 95% CI 1.5-31). Conclusion 15% of patients with pSS develope NS involvement that is associated with atherosclerotic stroke, ear disease and myopathy. Reference [1] Vitali C. et al. Ann Rheum Dis 2002; 61:554 Disclosure of Interests Jose Luis Andreu: None declared, Carlos Sanchez-Piedra: None declared, Monica Fernandez Castro: None declared, Victor Martinez Taboada: None declared, Alejandro Olive: None declared, Jose Rosas Consultant for: Abbvie, Amgen, Bristol, Janssen, Lilly, Merck Sharp & Dohme, Pfizer, UCB Pharma, Speakers bureau: Abbvie, Amgen, Bristol, Janssen, Lilly, Merck Sharp & Dohme, Pfizer, UCB Pharma, Raul Menor-Almagro: None declared, Beatriz Rodriguez Lozano: None declared, Angel Garcia-Aparicio: None declared, Francisco J Lopez-Longo: None declared, Sara Manrique Arija Speakers bureau: ABBvie, MSD, Janssen, Lillly, Roche, Pfyzer, Novartis., Jesus ALberto Garcia Vadillo: None declared, Susana Gil: None declared, Ruth Lopez Gonzalez: None declared, J. Narvaez Consultant for: Bristol-Myers Squibb, Carles Galisteo: None declared, Jorge Juan Gonzalez Martin: None declared, M E Ruiz: None declared, Inigo Rua-Figueroa: None declared, Oscar Illera: None declared, Lurdes Romani: None declared, Sheila Melchor: None declared, Begona Moreira: None declared, Enrique Raya: None declared, Jose M Pego-Reigosa: None declared, Natalia Cid Boza: None declared, Enrique Judez: None declared, Clara Moriano: None declared, Vicente Torrente: None declared, Hector Corominas: None declared, Blanca Garcia-Magallon: None declared, Carlos Guillen-Astete: None declared, Ivan Castellvi Consultant for: I received fees less than 5000USD as a consultant for Kern and Actelion, Paid instructor for: I received fees less than 2000USD as a instructor for Boehringer -Ingelheim, Novartis and Gebro, Speakers bureau: ND, Cristina Bohorquez: None declared, Javier Loricera: None declared, Joaquin Belzunegui: None declared

Background: In the last years multiple genetic variants have been associated with autoimmune disease (AD) risk. However, much less is known on the association with important environmental factors like diet. Objectives: The objective of the present study is to characterize the dietary patterns of six prevalent ADs, including rheumatoid arthritis (RA), psoriatic arthritis (PsA) and systemic lupus erythematosus (SLE). Methods: A cross-sectional cohort of 11,621 individuals from Spain encompassing 1,949 RA, 2,186 psoriasis (PS), 1,437 PsA, 699 SLE, 1,415 ulcerative colitis (UC), 1,952 Crohn’s disease (CD) and 1,983 healthy controls, was recruited by the IMID-Consortium. The weekly consumption frequency of main food categories was recorded, together with drinking habits and smoking status. Multivariate linear models were used to test for association while adjusting for confounders. Results: Patients with AD targeting the digestive system showed the largest differences in comparison to controls. In the rheumatic ADs several food categories were also found to be consumed differently compared to controls. SLE had a high consumption of sweets and pastries, and RA ate more fruit and bread/wholegrain foods and less meat and cold cuts compared to controls. In PsA the dietary pattern differences with controls where milder, and non-overlapping with psoriasis. Disease activity was significantly associated with specific food categories in CD, UC and PS, mildly in RA and SLE, and PsA showed no association. High smoking cessation rates were observed in UC, CD and SLE, but not in Ps, RA and PsA. Tea/coffee and alcohol consumption was low in all ADs compared to controls. Conclusion: Our results show previously unreported associations with dietary habits in rheumatic ADs. Disclosure of Interests: Antonio Julia: None declared, Sergio Hilario Martinez: None declared, Jesus Tornero: None declared, Juan D. Canete: None declared, Antonio Fernandez-Nebro: None declared, Francisco Blanco: None declared, Jesus Rodriguez: None declared, Francisco J Lopez-Longo: None declared, Benjamin Fernandez-Gutierrez: None declared, Jordi Gratacos: None declared, Jose Javier Perez Venegas: None declared, Carolina Perez: None declared, Ruben Queiro Silva: None declared, Alejandro Olive: None declared, Mercedes Alperi-Lopez: None declared, Carlos A. Montilla-Morales: None declared, Jose Luis Andreu: None declared, Juan Carlos Torre-Alonso: None declared, M Angeles Aguirre-Zamorano: None declared, Hector Corominas: None declared, Paloma Vela Casasempere: None declared, Victor Martinez Taboada : None declared, Sara Manrique Arija Speakers bureau: ABBvie, MSD, Janssen, Lillly, Roche, Pfyzer, Novartis., Joan Miquel Nolla: None declared, Isidoro Gonzalez-Alvaro: None declared, Santiago Munoz-Fernandez: None declared, Jose Luis Marenco Speakers bureau: abbie, pfizer, novartis, janmsen, Carlos M. Gonzalez: None declared, Antonio Zea: None declared, Maria Lopez Lasanta: None declared, Daniel Roig: None declared, Jose M Pego-Reigosa: None declared, Mireia Lopez Corbeto: None declared, Pedro Zarco-Montejo: None declared, Mercedes Freire Gonzalez: None declared, Alba Erra: None declared, Elvira Diez Alvarez: None declared, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Esther Rodriguez Almaraz: None declared, Alicia Garcia: None declared, Patricia Carreira: None declared, GEORGINA SALVADOR ALARCON: None declared, Cesar Diaz Torne Consultant for: Grunenthal, Speakers bureau: Grunenthal, Ricardo Blanco: None declared, Alfredo Willisch Dominguez: None declared, Jose Antonio Mosquera Martinez: None declared, Simon Sanchez Fernandez: None declared, Julio Ramirez: None declared, Sara Marsal: None declared

Background: Inflammatory idiopathic myositis (IIM) comprises a heterogeneous group of systemic muscular diseases that can occur together with other connective tissue diseases (CTD), named overlap myositis (OM). The question of whether OM is a distinct entity still remains controversial. Aim: The present study was conducted to assess the clinical and prognostic differences between patients diagnosed with OM, primary polymyositis (PM) and primary dermatomyositis (DM). Method: The study consists of a retrospective longitudinal and multicenter series of IIM patients. Patients were classified as OM, PM and DM. Overlap myositis was defined as patients fulfilling criteria for IIM plus criteria for other CTD (namely systemic sclerosis, systemic lupus erythematosus, mixed connective tissue disease, rheumatoid arthritis and primary Sjögren's syndrome). Result: A total of 342 patients were included (98 OM, 137 PM and 107 DM). Overlap myositis patients, in comparison with PM and DM, showed significant differences, with more extramuscular involvement, particularly more arthritis (66%, 34.6% and 48.1%, respectively), puffy fingers (49.5%, 11.1% and 24.3%), sclerodactyly (45.4%, 2.2% and 2%), dysphagia (41.8%, 18.2% and 26.4%), Raynaud phenomenon (65.3%, 16.9% and 19.8%), leucopenia (28.9%, 2.2% and 8.4%), thrombocytopenia (8.2%, 2.2% and 1.9%), interstitial lung disease (ILD) (48%, 35% and 30.8%), renal manifestations (13.4%, 3.7% and 1.9%), and more severe infections (41.3%, 26.7% and 21%). No significant differences were found in survival between groups in log rank test (P = 0.106). Multivariate adjusted survival analyses revealed a worse prognosis for severe infections, ILD and baseline elevation of acute phase reactants. Conclusion: Overlap myositis stands out as a distinct entity as compared to PM and DM, featuring more extramuscular involvement and more severe infections. Close monitoring is recommended in this subset for early detection and treatment of possible complications. Sin financiación 1.980 JCR (2019) Q1, 25/32 Rheumatology 0.649 SJR (2019) Q3, 33/64 Rheumatology No data IDR 2019 UEM

Background Remission and Low Disease activity state (LDAS) are the treat to target (T2T) outcomes used Systemic Lupus Erythematosus (SLE). Lupus Impact Tracker (LIT), a ten item unidimensional patient reported (PRO) tool with good psychometric properties and responsiveness including to composite response Index (SRI) and T2T outcomes. SLE patients often fibromyalgia (FM). We report discriminant validity of LIT to T2T outcomes in SLE patients stratified by FM from the largest European SLE registry- cohort. Methods Observational, multi-center data from 1364 adult patients with SLE meeting 1997 ACR criteria were obtained. This included demographics, LIT, disease activity-DA (SLEDAI) and medications. Remission off therapy (ROFT) was defined as SLEDAI=0 without prednisone or Immunosuppressive/s. Remission on-therapy (RONT) was SLEDAI=0 and a prednisone dose 5 mg/day and/or Immunosuppressive/s. LDAS (modified) was SLEDAI 4, prednisone dose 9 mg/day and/or maintenance immunosuppressive/s. Non-optimal (NO) disease status was SLEDAI >4 and/or prednisone dose >9 mg/day and/or immunosuppressive/s in induction dose. LIT values were compared between T2T groups using mixed models among (a) all patients, (b) with and (c) without FM. For Model 1 : RONT and LDAS were combined (given relatively very low DA and lower prevalence of steroid use in LDAS) and compared with NO and ROFT. For model 2, all 4 T2T categories were used. Results 1232/1364 (90%) were women, and 95% were Caucasian,76/1364 had FM. Median LIT (Q1, Q3) was 25 (10,46.3). LIT scores were significantly different among T2T categories (table 1). Significant relevant differences were not evident between RONT and LDAS groups: 1) RONT had greater medication use than LDAS (prednisone 5–10 mg/day use of 17.2% vs 8.3%)). 2) In LDAS, mean SLEDAI was 2, which can occur without clinical DA. RONT thus fared slightly worse than LDAS in patients evaluation of SLE impact. LIT was able to differentiate between T2T outcomes, and the scores moved in the anticipated direction. Though LIT scores for RONT were lower than NO in those with FM, they did not reach significance. This could potentially be secondary to small number of FM patients and use of 4 comparator categories. Conclusions LIT can differentiate between T2T outcomes in SLE patients with and without FM. Given its extensive validation in various languages, regions, measurement equivalence, ease of use, acceptability by patients and physicians, and responsiveness to changes in assessments by physician (SLEDAI, PGA, SRI, T2T) and patient (HRQOL, SLAQ), the tool is indicated for routine patient care, research and clinical trials. Funding Source(s): None

Background Remission and Low Disease activity state (LDAS) are physician assessed treat to target-T2T outcomes for Systemic Lupus Erythematosus (SLE). Lupus Impact Tracker (LIT), a ten-item unidimensional patient reported tool has good psychometric properties and responds to patient reported changes in health, physician based disease activity (DA) and composite response Index (SRI). Herein we report responsiveness of LIT to changes in physician (T2T) and patient assessed outcomes (DA by SLAQ and health status (EQ5D)) among SLE patients from the largest European SLE registry- cohort. Methods One-year longitudinal, observational, multi-center data from 1364 adult patients with SLE meeting 1997 ACR criteria were obtained from baseline and year 1 visit. This included demographics, patient reported tools (LIT, EQ5D VAS, SLAQ), SLE (activity-SLEDAI) and medications. Remission off therapy (ROFT) was defined as SLEDAI=0 without prednisone or Immunosuppressive/s. Remission on-therapy (RONT) was SLEDAI=0 and a prednisone dose 5 mg/day and/or Immunosuppressive/s (maintenance dose). LDAS (modified) was defined as SLEDAI 4, prednisone dose 9 mg/day and/or maintenance immunosuppressive/s. Non-optimal (NO) disease status was SLEDAI >4 and/or prednisone dose >9 mg/day and/or immunosuppressive/s in induction dose. Use of hydroxychloroquine was permitted in all groups. LIT values were compared using mixed models. Responsiveness was evaluated by standard response means (SRM) in groups with changes in DA (T2T, SLAQ) and EQ5D VAS as anchors. We did not have enough observations for stratified analysis for SLE patients with fibromyalgia. Results 1232/1364 (90%) were women, and 95% were Caucasian. Mean (SD) SLEDAI and SDI were 2.6 (3.5) and 0.7 (1.1) respectively. As (i) DA was low (median 2) in LDAS, (ii) steroid use was more prevalent in RONT than LDAS, we combined RONT and LDAS into one category to analyse patient relevant differences in LIT. LIT was responsiveness in the appropriate direction with improvement and worsening in disease activity (T2T and SLAQ) and health status (EQ5D VAS) over time. Mean LIT changes to and from NO to RONT/LDAS ranged from 3–5 (table 1), while it declined by over 8.5 with change from NO to ROFT. We had limited observations for ROFT to NO change. Mean change in LIT ranged from −3 to 3 with improvement and worsening in SLAQ, and from −7.6 to 6 with improvement and worsening in EQ5D VAS. Conclusions LIT responds appropriately in both directions to changes in physician (T2T) as well as patient relevant (DA and health status) outcomes among Spanish SLE patients. Funding Source(s): None

Background The clinical coexistence of Systemic Lupus Erythematosus (SLE) and Sjogrens Syndrome (SS) was recognized in 1959. The prevalence of SS among patients with SLE varies considerably among the published studies (10%–30%). There is still controversy as to whether or not SLE patients with overlapping SS have a distinct and significantly milder lupus. To address the clinical and serologic features of SLE and differences from SLE that occurs in overlap with SS. Methods This is a multicenter, descriptive, cross-sectional study of 3575 patients from the Spanish Society for Rheumatology Lupus Registry (RELESSER). Unselected SLE patients from 45 Rheumatology Departments across Spain were evaluated for the presence of overlapping SS using the American-European consensus criteria. Cumulative clinical data were collected at the moment of the last assessment. Clinical and laboratory parameters in SLE patients with SS (SLEwSS) were compared with those in SLE patients without SS (SLEwoSS). Results SS was identified in 516 SLE patients (14.4%). Compared with the SLEwoSS group, patients with SLEwSS were significantly older, had a higher frequency of mucocutaneous manifestations, Raynauds phenomenon, peripheral neuropathy, anti-Ro/SSA, anti-La/SSB, neoplasia, and older age at death, but had a significantly lower frequency of renal involvement, thrombocytopenia, anti-dsDNA, anti-2-GPI IgM and complement consumption. Both groups displayed a clinically similar presentation of lymphadenopathy, systemic vasculitis, serositis, damage accrual, mortality, musculoskeletal and CNS manifestations. Conclusions SLEwSS appears to constitute a subgroup of SLE patients with distinct clinical and serologic features, in whom SS is expressed as an overlapping entity. A particular cluster of clinical variables, namely, mucocutaneous manifestations, Raynauds phenomenon, peripheral neuropathy, renal involvement and thrombocytopenia, was found to be important overall for discriminating SLE patients with or without SS. SLEwSS patients constitute a subgroup of patients with SLE characterized by milder lupus: older age at death, similar rates of mortality and SLICC-ACR damage index, less renal and immunological manifestations. Funding Source(s): None

Background:Radiosynovectomy (RS) is a useful for treating inflammatory arthritis that fail conventional treatments. The main isotope used is Yttrium-90 on large joints as knees, whereas Erbium-169 and Renium-186 are more common in small and medium sized joints respectively.RS is a safe procedure since the isotopes cannot escape the synovial capsule or be absorbed into circulation. It is, however, lethal against cells within the inflamed joint.The most common rheumatic disease treated with RS is rheumatoid arthritis (RA), followed by axial spondyloarthritis (SpA) and idiopathic juvenile arthritis (JIA). It has also been used on persistent synovitis after joint replacements, pigmented villonodular synovitis (PVNS) and undifferentiated arthritis.Objectives:To describe the experience in RS of a tertiary rheumatology center and compare patients with and without clinical response to treatment in the following 12 months.Methods:Observational retrospective study between May 31st 2013 and October 31st 2019. We collected demographic variables, data about the disease of the patient, the joints affected, isotope utilized, presence of Baker’s cyst, systemic treatment received, need of additional infiltrations (before and after), complications and any changes in medication up to a year after the procedure.All the RS were performed ambulatory and the radioisotope infiltration was guided by ultrasound, with 40mg of triamcinolone infiltrated after.SPSS v23 was used for statistical analysis; with Chi2 for qualitative variables and Student’s T distribution for quantitative variables.Results:We evaluated 67 joints in 49 patients in total. All of them were refractory to conventional treatment. 44 patients (65.7%) were women, median of 53.4 years of age (IQ 43.4-67.1).The median disease duration was 12.5 years and RS seemed to fare better the longer the patient had the disease (median of 13.5 years vs 6.5 years pThe joints infiltrated where 46 (68.6%) knees, 14 (20.9%) wrists and 7 (15.2%) elbows. Out of the knees, 16 (34.8%) belonged to RA patients with effective response in 14 (87,5%). 100% of elbows had an effective response, of them 6 (85.7%) had RA. However, even when 9 (64.2%) wrists also had RA as diagnosis, only 3 (21.4%) were effective.Of the PVNS, 6 out of 8 (75%) had no clinical response, as shown in Table 1.Table 1.RS response compared to clinical diagnosis.TOTALEFFECTIVEINEFFECTIVEp 67 (100%)46 (68.6%)21 (31.3%)Inflammatory Arthritides(RA + PsA + SpA + sJIA), (%)52 (77.6)39 (75%)13 (25%)RA (%)30 (44.7)22 (73.3)8 (26.6)RA positive ACPA/FR21 (70)15 (71.4)6 (28.6)Psoriasic arthritis (PsA) (%)6 (9)4 (66.6)2 (33.3)0.42SpA (%)10 (14.9)8 (80)2 (20)0.45sJIA (%)6 (9)5 (83.3)1 (16.6)0.55PVNS (%)8 (11.9)2 (25)6 (75)Inespecific monoarthritis (%)3 (4.4)3 (100)0 (0)0.23OA + Calcium Pyrophosphate Deposition (CPPD) (%)4 (5.9)2 (50)2 (50)0.33Intra articular corticosteroids were needed before RS, with no differences in effective and ineffective joints; however after RS it was significantly lower in effective joints in the first six months (0% vs 43% pOnly 13 (28%) patients with effective RS needed to change systemic treatment compared to 10 (43%) of those ineffective (pConclusion:Our study showed that knees were the main joint infiltrated and they had an overall good response to treatment, especially if the diagnosis was RA.Patients with effective procedures needed leest treatment changes and significantly less corticosteroids infiltrations.In our study, RS in PVNS was significantly less effective than in inflammatory arthritis (25% vs 75% pReferences:[1]Liepe K. Efficacy of radiosynovectomy in rheumatoid arthritis. Rheumatol Int. 2012 Oct; 32(10):3219-24.[2]Ćwikła JB, Żbikowski P, Kwiatkowska B, Buscombe JR, Sudoł-Szopińska I. Radiosynovectomy in rheumatic diseases. J Ultrason. 2014 Sep; 14(58):241-51.Disclosure of Interests:None declared

Hemophagocytic lymphohistiocytosis (HLH) is a very rare syndrome with a mortality up to 95% of cases if not treated. It is characterised by an excessive activation of the immune system that leads to a disproportionate and destructive inflammatory response. The high mortality rates are in part due to a delay in the diagnosis, and therefore clinicians must maintain a high index of suspicion. When the treatment is started early, the survival rate reaches around 55% of cases. HLH usually presents with persistent fever, pancytopenia, and organomegaly and is associated with very high levels of serum ferritin. In this manuscript, we present the case of a patient with primary Sjögren's syndrome who developed HLH after an acute infection by Cytomegalovirus. We will describe and discuss the pathogenesis, differential diagnosis and a pragmatic approach to the treatment for this critically important and, when diagnosed early, potentially curable syndrome.

Objective The objective of this study was to describe the demographic, clinical, and immunological manifestations of systemic lupus erythematosus (SLE) in male patients. Methods A cross-sectional, multicenter study was carried out of 3651 patients (353 men, 9.7%, and 3298 women, 90.2%) diagnosed with SLE, included in the Spanish Rheumatology Society SLE Registry (RELESSER). Results Mean ages (18–92 years) of symptom onset were 37 (SD 17) years (men) and 32 (SD 14) years (women). Male/female ratio was 1/9. Age of onset of symptoms and age at diagnosis were higher in men than in women ( p 50 years had a higher mortality (odds ratios 3.6 and 2.1, respectively). Furthermore, SLE patients who developed pulmonary hemorrhage, pulmonary hypertension, psychiatric involvement, complement deficiency, and hemophagocytic syndrome also had higher mortality, regardless of gender. Conclusion Patients with SLE over the age of 50 years have an increased risk of mortality. In Caucasians, age at diagnosis and symptom onset is higher in men than in women. The diagnostic delay is shorter in men. Male SLE patients present more cardiovascular comorbidities, and also more serositis, adenopathies, splenomegaly, renal involvement, convulsion, thrombosis, and lupus anticoagulant positivity than women.

Objective.To evaluate the multiobserver reliability of salivary gland ultrasonography (SGUS) for scoring greyscale (GS) parenchymal inhomogeneity and parenchymal color Doppler (CD) signal in patients with established primary Sjögren syndrome (pSS).Methods.The study comprised 2 multiobserver reliability assessments in patients with pSS in 2 European centers. The first reliability exercise was performed on 24 patients with pSS and 8 controls who were independently evaluated with GS and CD US by 5 observers at the Institute of Rheumatology, Belgrade, Serbia. The second reliability exercise was carried out on 10 patients with pSS who were independently assessed with GS and CD US by 8 observers at the Hospital G.U. Gregorio Marañón, Madrid, Spain. SGUS parenchymal inhomogeneity and parenchymal CD signal were semiquantitatively scored using a 4-grade scoring system. The multiobserver agreement was calculated by the overall agreement and Light’s κ statistics.Results.A total of 640 SGUS examinations were performed in the first reliability exercise and a total of 320 examinations in the second reliability exercise. Multiobserver reliability was good (κ = 0.71–0.79) to excellent (κ = 0.81–0.82) for GS parenchymal inhomogeneity in both exercises. There was a moderate (κ = 0.53–0.58) to good (κ = 0.70) multiobserver reliability for parenchymal CD signal in the first exercise. However, there was no agreement or only a fair agreement (κ = 0.03–0.29) for parenchymal CD signal in the second exercise.Conclusion.US may be a reliable technique in the multiobserver scoring of GS parenchymal inhomogeneity of major SG in patients with established pSS. CD scoring of SG needs further standardization to be used in multicenter studies.

OBJECTIVE To compare structural damage assessed by conventional radiography and tendon damage assessed by musculoskeletal US (MSUS) at wrist and ankle in RA patients. METHODS We evaluated 72 consecutive patients [56 (77.8%) females] with RA. The MSUS evaluation consisted in a B-mode examination of bilateral extensor carpi ulnaris and tibialis posterior tendons. Tendon damage was defined and scored according to OMERACT. A total score for the tendon damage score (TDS) was calculated by summing the grades for each tendon. For the radiographic evaluations we used the van der Heijde score; a total radiographic score (RTS) was calculated by summing a bone erosion score (ERS) and a joint space narrowing score (JSNS). RESULTS We evaluated 288 tendons. The mean (s.d.) of TDS was 2.3 (1.8). Fifty-four (75%) patients presented tendon damage of at least one tendon. From all evaluated tendons, 134 (46.5%) had no tendon damage, 146 (50.7%) had grade 1 and 8 (2.8%) had grade 2 tendon damage. The mean (s.d.) for RTS was 91.4 (97), for ERS was 47.3 (61.9) and for JSNS was 44.1 (37.2). We found a significant correlation between disease duration and both TDS and RTS (r = 0.413 and r = 0.560, respectively; P < 0.0001). We found a good significant correlation between TDS and all variables of radiographic structural damage (RTS, r = 0.65; ERS, r = 0.637; JSNS, r = 0.618; P < 0.001). CONCLUSION The MSUS assessment of only four tendons can be an additional feasible method to assess structural damage in RA patients.

Background:The mortality in Systemic Lupus Erythematosus (SLE) varies largely across different countries most probably due to social, healthcare and ethnic differences.Objectives:To analyze the causes and identify predictive factors of mortality of SLE in Spain in the present century.Methods:We performed a cross-sectional and retrospective study analyzing data from the RELESSER cohort (Spanish Registry of Systemic Lupus Erythematosus of the Spanish Society of Rheumatology). We included all patients diagnosed with SLE since the year 2000 and recorded sociodemographic, clinical and serological variables, comorbidities and treatments, as well as indicators of disease activity, damage and severity. The characteristics of the deceased patients were compared with those of the survivors, and variables with clinical significance or statistical significance were grouped into multivariate models to determine which ones were independently associated with the outcome of the disease.Results:A total of 2004 patients were included, 88.6% female, the mean age at diagnosis was 38.3 (± 15.3) years, with a mean delay in diagnosis of 28.9 (± 52.6) months. Up to 2.84% of the individuals had died. The leading cause of death was SLE activity (n=16), followed by infections (n=14), vascular events (n=7) and cancer (n=6). The mean age of death was 54.68 (± 20.13) years, and neither age, sex nor delay in diagnosis was independently associated with mortality. The presence of nephritis, depression, severe infections, organ damage (SLICC/ACR DI) or disease activity (SLEDAI), as well as the use of cyclophosphamide, rituximab or high doses of corticosteroids, were predictors of mortality in our cohort. Antimalarial treatment and skin manifestations were linked to improved survival.Conclusion:In the RELESSER cohort, clinical factors, co-morbidities, as well as therapeutic attitudes were associated with a significant increase in mortality in SLE. Interestingly, depression was independently associated to mortality. The activity of the disease and infections continue to be the main causes of death at the beginning of the 21st century amongst our patients.Disclosure of Interests:Clara Moriano: None declared, Jaime Calvo Grant/research support from: Lilly, UCB, Consultant of: Abbvie, Jansen, Celgene, Iñigo Rua-Figueroa: None declared, Elvira Diez Alvarez: None declared, Cristina Bermúdez: None declared, Francisco J López-Longo Grant/research support from: AbbVie and GSK, Speakers bureau: AbbVie, Actelion, Bristol Myers Squibb, GSK, MSD, Pfizer, Roche, and UCB Pharma, María Galindo-Izquierdo: None declared, Alejandro Olive: None declared, Eva Tomero Muriel: None declared, Antonio Fernandez-Nebro: None declared, Mercedes Freire González: None declared, Olaia Fernández- Berrizbeitia: None declared, Ana Pérez Gómez: None declared, Esther Uriarte Isacelaya: None declared, Carlos Marras Fernandez Cid: None declared, Carlos A. Montilla-Morales: None declared, Gregorio Santos Soler: None declared, Ricardo Blanco Grant/research support from: AbbVie, MSD, and Roche, Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, and MSD, M. Rodíguez-Gómez: None declared, Paloma Vela-Casasempere: None declared, Alina Boteanu: None declared, J. Narváez: None declared, Victor Martinez Taboada: None declared, Blanca Hernández-Cruz Speakers bureau: Abbvie, Lilly, Sanofi, BMS, STADA, José Luis Andreu: None declared, José A Hernandez Beriain: None declared, Lorena Expósito: None declared, Raúl Menor-Almagro: None declared, Mónica Ibañez Barceló: None declared, Ivan Castellví Consultant of: Boehringer Ingelheim, Actelion, Kern Pharma, Speakers bureau: Boehringer Ingelheim, Actelion, Bristol-Myers Squibb, Roche, Carles Galisteo: None declared, Enrique Raya: None declared, Víctor Quevedo Vila: None declared, Tomas Vazquez Rodriguez: None declared, Jesus Ibañez: None declared, Jose M Pego-Reigosa: None declared

Objective High frequency ultrasound allows visualization of epidermis, dermis and hypodermis, precise measurement of skin thickness, as well as assessment of skin oedema, fibrosis and atrophy. The aim of this pilot cross-sectional observational study was to assess the performance and multiobserver variability of ultra-high-frequency (UHF) (50 MHz) ultrasound (US) in measuring skin thickness as well as the capacity of UHF-derived skin features to differentiate SSc patients from healthy controls. Methods Twenty-one SSc patients (16 limited and five diffuse SSc) and six healthy controls were enrolled. All subjects underwent US evaluation by three experts at three anatomical sites (forearm, hand and finger). Dermal thickness was measured and two rectangular regions of interest, one in dermis and one in hypodermis, were established for texture feature analysis. Results UHF-US allowed a precise identification and measurement of the thickness of the dermis. The dermal thickness in the finger was significantly higher in patients than in controls (P < 0.05), while in the forearm it was significantly lower in patients than in controls (P < 0.001). Interobserver variability for dermal thickness was good to excellent [forearm intraclass correlation coefficient (ICC) = 0.754; finger ICC = 0.699; hand ICC = 0.602]. Texture computed analysis of dermis and hypodermis was able to discriminate between SSc and healthy subjects (area under the curve >0.7). Conclusion These preliminary data show that skin UHF-US allows a very detailed imaging of skin layers, a reliable measurement of dermal thickness, and a discriminative capacity between dermis and hypodermis texture features in SSc and healthy subjects.

Objective To investigate the genetic background influencing the development of cardiovascular (CV) disease in patients with rheumatoid arthritis (RA). Methods We performed a genome-wide association study (GWAS) in which, after quality control and imputation, a total of 6,308,944 polymorphisms across the whole genome were analyzed in 2,989 RA patients of European origin. Data on subclinical atherosclerosis, obtained through assessment of carotid intima-media thickness (CIMT) and presence/absence of carotid plaques by carotid ultrasonography, were available for 1,355 individuals. Results A genetic variant of the RARB gene (rs116199914) was associated with CIMT values at the genome-wide level of significance (minor allele [G] beta coefficient 0.142, P = 1.86 x 10(-8)). Interestingly, rs116199914 overlapped with regulatory elements in tissues related to CV pathophysiology and immune cells. In addition, biologic pathway enrichment and predictive protein-protein relationship analyses, including suggestive GWAS signals of potential relevance, revealed a functional enrichment of the collagen biosynthesis network related to the presence/absence of carotid plaques (Gene Ontology no. 0032964; false discovery rate-adjusted P = 4.01 x 10(-3)). Furthermore, our data suggest potential influences of the previously described candidate CV risk loci NFKB1, MSRA, and ZC3HC1 (P = 8.12 x 10(-4), P = 5.94 x 10(-4), and P = 2.46 x 10(-4), respectively). Conclusion The present findings strongly suggest that genetic variation within RARB contributes to the development of subclinical atherosclerosis in patients with RA., Supported in part by the European Union FEDER fund and the Instituto de Salud Carlos III (ISCIII) (Fondo de Investigacion Sanitaria grants PI06/0024, PS09/00748, PI12/00060, PI15/00525, and CP16/00033), the ISCIII RETICS programs (RD12/0009 and RD16/0012), the European IMI BTCure Program. Dr. Lopez-Mejias is recipient of the Miguel Servet type I Program Fellowship from the ISCIII and the European Social Fund (ESF) (grant CP16/00033). Dr. Carmona's work was supported by the Spanish Ministry of Economy and Competitiveness (Ramon y Cajal Program grant RYC-2014-16458). Dr. Genre is recipient of a Sara Borrell postdoctoral fellowship from the ISCIII and ESF (grant CD15/00095). Dr. Remuzgo-Martinez' work was supported by the ISCIII and European Regional Development Fund (ERDF) (RETICS Program RD16/0012/0009). Ms Pulito-Cueto's work was supported by a predoctoral grant from the Instituto de Investigacion Sanitaria Valdecilla (PREVAL 18/01). Ms Mijares' work was supported by the Miguel Servet type I Program from the ISCIII and ESF (grant CP16/00033). Dr. Gualillo is staff personnel of Xunta de Galicia (Servizo Galego de Saude [SERGAS]) through a research-staff stabilization contract (ISCIII/SERGAS) and his work is funded by ISCIII and FEDER (grants RD16/0012/0014 [RIER] and PI17/00409). He is beneficiary of project funds from the Research Executive Agency (REA) of the European Union in the framework of MSCA-RISE Action of the H2020 Programme, project 734899-Olive-Net.

The difficulty in diagnosis and the spectrum of clinical manifestations that can determine the choice of treatment for primary antiphospholipid syndrome (APS) has fostered the development of recommendations by the Spanish Society of Rheumatology (SER), based on the best possible evidence. These recommendations can serve as a reference for rheumatologists and other specialists involved in the management of APS.A panel of four rheumatologists, a gynaecologist and a haematologist with expertise in APS was created, previously selected by the SER through an open call or based on professional merits. The stages of the work were: identification of the key areas for drafting the document, analysis and synthesis of the scientific evidence (using the Scottish Intercollegiate Guidelines Network [SIGN] levels of evidence) and formulation of recommendations based on this evidence and formal assessment or reasoned judgement techniques (consensus techniques).46 recommendations were drawn up, addressing five main areas: diagnosis and evaluation, measurement of primary thromboprophylaxis, treatment for APS or secondary thromboprophylaxis, treatment for obstetric APS and special situations. These recommendations also include the role of novel oral anticoagulants, the problem of recurrences or the key risk factors identified in these subjects. This document reflects the first 21, referring to the areas of: diagnosis, evaluation and treatment of primary APS. The document provides a table of recommendations and treatment algorithms.An update of the SER recommendations on APS is presented. This document corresponds to partI, related to diagnosis, evaluation and treatment. These recommendations are considered tools for decision-making for clinicians, taking into consideration both the decision of the physician experienced in APS and the patient. A partII has also been prepared, which addresses aspects related to obstetric SAF and special situations.

Objective: To study prognostic factors in different types of idiopathic inflammatory myopathies (IIM) associated with interstitial lung disease (ILD). Patients and methods: Multicenter retrospective study of a Spanish cohort of patients diagnosed with IIM. Patients were classified into four categories: polymyositis (PM), dermatomyositis (DM), antisynthetase syndrome (ASS), and overlap myositis (OM). Sociodemographic data, clinical characteristics, antibodies, and treatments were collected. Cox regression models were calculated to identify factors associated with mortality, the necessity for long-term oxygen therapy (LTOT), and deterioration in respiratory function tests (RFT). Results: The number of patients included was 478, of whom 112 (23.4%) suffered from ILD: 17% PM, 16% DM, 45% ASS, and 22% OM. Factors associated with mortality in the multivariate analysis were clinically meaningful progression of ILD after 3 months (CMP 3m) (hazard ratio (HR) 9.48, p = 0.005), severe infections (HR 6.41, p = 0.016), heliotrope erythema (HR 31.1, p = 0.002), delay in diagnosis (HR 1.29; p = 0.011), and Raynaud's phenomenon (HR 11.9, p = 0.007). However, being female (HR 0.19, p = 0.044) and positivity solely for ANAs (HR 0.08, p = 0.008) presented a protective effect. CMP 3m (HR 22.7, p = 0.027) was associated with the need for LTOT, while basal aldolase (HR 0.90; p = 0.049) had a protective effect. Likewise, joint manifestations (HR 0.04, p = 0.034) were shown to reduce risk of deterioration in RFT. Conclusions: CMP 3m, severe infections, delay in diagnosis, heliotrope erythema, and Raynaud's phenomenon were identified as factors of poor prognosis in different IIM associated with ILD. Sin financiación 2.394 JCR (2019) Q3, 20/32 Rheumatology 0.755 SJR (2019) Q2, 27/64 Rheumatology No data IDR 2019 UEM

Background It is accepted that donor-derived immunity is transferred with allogeneic bone marrow transplantation (BMT)1. Objectives To show evidence of the transfer of systemic sclerosis by allogeneic BMT. Methods In this report we describe a pacient with T acute lymphoblastic leukaemia who underwent BMT and developed systemic sclerosis. Results 34-year-old man in complete remission from a T acute lymphoblastic leukaemia treated with allogeneic BMT from his mother in february of 2012. First seen in november 2017 for digital ulcers that appeared one year before. He presented two necrotic ulcers: one on the second finger of the left hand and other on the third finger of the right hand (IMAGE 1). He was admited to receive intravenous prostaglandins and complete the study. After the BMT he developed Raynaud’s phenomenon and in the examination he only presented facial and corporal telangiectasia, attributed before to chronic graft versus host disease (cGVHD). The analysis showed ANA 1/640 centromere pattern, anticentromere antibodies, reumatoid factor (RF) (652 UI/mL) and C3 of 86.4 mg/dL. Previously to the BMT he had negative ANA, but we do not know the rest of the previous autoimmunity. On the videocapillaroscopy we observe an active scleroderma pattern (IMAGE 2–3). He was diagnosed with systemic sclerosis based on Raynaud’s phenomenon, digital ulcers, anticentromere antibodies and abnormal nailfold capillaries. He had not familiar background of connective tissue diseases, but his mother presented Raynaud’s phenomenon since she was thirty. So we studied her. She presented facial telangiectasia, puffy fingers and fingertip pitting scars and the same autoantibodies: ANA 1/320 centromere pattern, anticentromere antibodies, RF (159 UI/mL) and consumption of C3 (74.3 mg/dL) and C4 (7.6). We do not have previous autoimmune studies of her. On the videocapillaroscopy we observed a late scleroderma pattern (IMAGE 4–5). She was also diagnosed with systemic sclerosis, based on Raynaud’s phemomenon, puffy fingers, fingertip pitting scars and facial telangiectasia, anticentromere antibodies and abnormal naifold capillaries. Conclusions Experimental animal studies and human clinical reports have described the transfer of immune-mediated diseases from affected donors to unaffected recipients, because of that the importance of screening this diseases in the donor before a BMT2. To our knowledge, this will be the first described case of transmission of systemic sclerosis by this mechanism. However, other explanations should also be taken into consideration. This include recipient’s own persistent intrathymic lymphocyte population that may produce autoantibodies and autoimmunity in the context of cGVHD. However, this last hypothesis is not supported since there is a familiar background and presence of anticentromere antibodies. References [1] Sherer, Y. and Shoenfeld, Y. (1998). Autoimmune diseases and autoimmunity post-bone marrow transplantation. Bone Marrow Transplantation. [2] Hough, R.(2005). Haemopoietic stem cell transplantation in autoimmune diseases: a European perspective. British Journal of Haematology. Disclosure of Interest None declared

Background Among the subtypes of idiopathic inflammatory myopathy (IIM), it has been recognised that primary polymyositis (PM) and primary dermatomyositis (DM) have a worse prognosis than overlap syndrome (OM). However, the benign course of OM has not been confirmed in other studies. The identification of specific characteristics that could allow a correct classification may be important for its prognosis. Objectives To analyse clinical characteristics and survival of patients diagnosed with OM, and to compare if there are differences with other IIM subgroups in a multicentric cohort. Methods Retrospective, multicentric longitudinal observational study of a cohort of patients followed between January 1980 and December 2014, with a diagnosis of MII. The patients were divided into 2 groups: OM and primary idiopathic inflammatory myositis (PIIM), which included PM and DM. Patients classified as OM had to meet IIM criteria and criteria for rheumatoid arthritis (RA), systemic sclerosis (SSc), systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD) or primary Sjogren’s syndrome (SS). Results The present study included a total of 348 patients (98 OM, 250 PIIM). The connective tissue diseases associated with OM were: MCTD in 32 (32.7%), SSc in 29 (29.6%), SLE in 21 (21.4%), RA in 8 (8.2%) and SS in 8 (8.2%). Patients with OM showed remarkable differences compared to PIIM. In general, OM exhibited more extramuscular involvement and more complications, with more serious infections (41% vs. 24%, p Conclusions In the REMICAM registry of 348 inflammatory myopathies, 98 cases of myositis with overlap syndrome have been included, presenting with more extramuscular complications, more severe infections and higher mortality than other myopathies. It would be important to identify these patients at the onset of the disease, in order to closely monitor for development of possible complications. Disclosure of Interest None declared