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espanolIntroduccion La capilaroscopia periungueal (CP) es util en la evaluacion del fenomeno de Raynaud tanto primario como secundario, y en el seguimiento de los pacientes con esclerosis sistemica. Nuestro estudio evalua el impacto de la CP en el diagnostico, en funcion del motivo de solicitud y el perfil de autoanticuerpos en la practica clinica diaria. Material y metodos Se incluyeron todos los pacientes con al menos una CP entre junio de 2012 y diciembre de 2017. Se revisaron las historias clinicas y se determino de forma dicotomica (si/no) si la CP contribuyo al diagnostico realizado en la consulta posterior a la realizacion de la CP. Se recogieron datos demograficos, clinicos y de laboratorio, motivo de solicitud de la CP y su relacion con los patrones CP. Resultados De 530 pacientes con una primera CP, 266 se realizaron como estudio de un fenomeno de Raynaud. De estos, en 20 pacientes (3,8%) se realizo un diagnostico de enfermedad del tejido conectivo en la consulta posterior a la CP; 15 fueron diagnosticados de esclerosis sistemica, 4 de conectivopatia indiferenciada y uno de enfermedad mixta. Salvo un paciente diagnosticado de conectivopatia indiferenciada, el resto tenia anticuerpos antinucleares positivos y 11 de ellos, ademas, anticuerpos especificos (9 anticentromero, uno anti-Scl70 y otro, anti-RNPC). La positividad de anticuerpos antinucleares se asocio con una mayor probabilidad de presentar una CP de esclerodermia, y ningun paciente diagnosticado de una enfermedad reumatica tras la CP tenia un patron normal. Conclusion La CP es una tecnica util, pero con impacto limitado en el diagnostico de enfermedades del tejido conectivo. La positividad de los anticuerpos se relaciona con una mayor probabilidad de presentar patrones patologicos en la CP. EnglishIntroduction Nailfold capillaroscopy (NC) is useful in the evaluation of Raynaud's phenomenon, associated with some connective tissue diseases and in the follow-up of patients with systemic sclerosis. Our study evaluates the impact of NC in the diagnosis, according to the reason for the request and profile of autoantibodies in daily clinical practice. Material and methods All patients that undergone at least one NC between June 2012 and December 2017 were included. Clinical records were reviewed and analysed in a dichotomous way (yes/no), to see whether the NC contributed to a change of diagnosis in subsequent consultations. In addition, demographic, clinical and laboratory data were collected, and the relationship with NC patterns evaluated. Results Of the 530 patients who had undergone at least one NC, 266 had Raynaud's phenomenon as primary indication for the technique. Of those, 20 patients (3.8%) had a diagnostic change in the post-NC consultation; 15 were diagnosed with systemic sclerosis, 4 with undifferentiated connective tissue disease and one with mixed connective tissue disease. All patients had, except for one patient diagnosed with undifferentiated connective tissue disease, positive antinuclear antibodies titres, 11 of them had disease specific antibodies (9 anti-centromere, one anti-Scl70 and other anti-RNPC). The positivity of antinuclear antibodies titres was associated with a higher probability of presenting a scleroderma pattern in the NC, and all patients with a specific rheumatological diagnosis had an abnormal NC. Conclusion NC is a useful technique, but with limited impact in the diagnosis of connective tissue diseases. Autoantibody positivity is associated with a greater likelihood of presenting pathological NC patterns.

Objective SLE can affect any part of the gastrointestinal (GI) tract. GI symptoms are reported to occur in >50% of SLE patients. To describe the GI manifestations of SLE in the RELESSER (Registry of SLE Patients of the Spanish Society of Rheumatology) cohort and to determine whether these are associated with a more severe disease, damage accrual and a worse prognosis. Methods We conducted a nationwide, retrospective, multicentre, cross-sectional cohort study of 3658 SLE patients who fulfil ≥4 ACR-97 criteria. Data on demographics, disease characteristics, activity (SLEDAI-2K or BILAG), damage (SLICC/ACR/DI) and therapies were collected. Demographic and clinical characteristics were compared between lupus patients with and without GI damage to establish whether GI damage is associated with a more severe disease. Results From 3654 lupus patients, 3.7% developed GI damage. Patients in this group (group 1) were older, they had longer disease duration, and were more likely to have vasculitis, renal disease and serositis than patients without GI damage (group 2). Hospitalizations and mortality were significantly higher in group 1. Patients in group 1 had higher modified SDI (SLICC Damage Index). The presence of oral ulcers reduced the risk of developing damage in 33% of patients. Conclusion Having GI damage is associated with a worse prognosis. Patients on a high dose of glucocorticoids are at higher risk of developing GI damage which reinforces the strategy of minimizing glucocorticoids. Oral ulcers appear to decrease the risk of GI damage.

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.

Background Antiphospholipid antibodies (aPL) have been associated with organ damage and certain features in systemic lupus erythematosus (SLE) patients. Our aim was to investigate the differences between SLE patients according to the presence of aPL and/or clinical antiphospholipid syndrome (APS). Methods Patients from the RELESSER-T registry were included. RELESSER-T is a multicenter, hospital-based registry, with retrospective cross-sectional collection of data from a large representative sample of adult non-selected patients with SLE attending Spanish rheumatology services from the public national health system. Results We included 3651 SLE patients and 1368 were positive for aPL (44.9% of patients were positive for anticardiolipin antibodies, 27.3% showed positivity for anti b2glycoprotein I and 24% for lupus anticoagulant). Overall 2283 patients were classified as SLE no aPL, 528 as SLE-APS and 840 as SLE-aPL. Demographic data, clinical and laboratory features in the different groups are showed in table 1. Regarding cardiovascular risk factors, SLE-APS patients had higher rates of hypertension, dyslipidemia and diabetes than SLE-aPL and SLE no aPL patients (p 0.5 grs), urinary cell casts, seizures and psychosis (p0.001). Overall, SLE-APS patients showed a lower rate of cutaneous manifestations and higher rates of neuropsychiatric, cardiac, pulmonary, renal, joint and ophthalmological manifestations (table 1). In accordance with a more severe clinical profile, higher frequency of anti-DNA antibodies and hypocomplementemia were observed in the SLE-APS group (p Conclusions SLE-APS patients show a more severe clinical profile with higher frequency of major organ involvement and more damage accrual than SLE-aPL and SLE no APL. 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

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 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.

Introduction Nailfold capillaroscopy (NC) is useful in the evaluation of Raynaud's phenomenon, associated with some connective tissue diseases and in the follow-up of patients with systemic sclerosis. Our study evaluates the impact of NC in the diagnosis, according to the reason for the request and profile of autoantibodies in daily clinical practice. Material and methods All patients that undergone at least one NC between June 2012 and December 2017 were included. Clinical records were reviewed and analysed in a dichotomous way (yes/no), to see whether the NC contributed to a change of diagnosis in subsequent consultations. In addition, demographic, clinical and laboratory data were collected, and the relationship with NC patterns evaluated. Results Of the 530 patients who had undergone at least one NC, 266 had Raynaud's phenomenon as primary indication for the technique. Of those, 20 patients (3.8%) had a diagnostic change in the post-NC consultation; 15 were diagnosed with systemic sclerosis, 4 with undifferentiated connective tissue disease and one with mixed connective tissue disease. All patients had, except for one patient diagnosed with undifferentiated connective tissue disease, positive antinuclear antibodies titres, 11 of them had disease specific antibodies (9 anti-centromere, one anti-Scl70 and other anti-RNPC). The positivity of antinuclear antibodies titres was associated with a higher probability of presenting a scleroderma pattern in the NC, and all patients with a specific rheumatological diagnosis had an abnormal NC. Conclusion NC is a useful technique, but with limited impact in the diagnosis of connective tissue diseases. Autoantibody positivity is associated with a greater likelihood of presenting pathological NC patterns.

Background The efficacy of Golimumab treatment in psoriatic arthritis (PsA) patients has been widely documented. Objectives The aim of this study was to analyse the long-term retention rate of golimumab and to identify independent predictors of drug retention in patients with PsA including concomitant synthetic disease-modifying antirheumatic drugs (sDMARD) Methods Prospective monocentric cohort of PsA patients treated with golimumab according to clinical practice. Study was approved by local Ethics Committee. Demographic and clinical variables were analysed with Cox proportional hazard regression model. Results 48 patients were included, 20/48 (41.7%) oligoarticular, 19/48 (39.6%) polyarticular and 9/48 (18.7%) with peripheral and axial PsA. The baseline characteristics of the patients are shown in table 1. Follow-up time was 89.25 patients-year. Mean survival time was 40.3 months (95% CI: 32.0–48.5). Age, mean evolution time and previous biological use were significant in the univariate analysis. Concomitant sDMARD had no influence on golimumab retention rate (HR: 1.3; 95% CI: 0.5–3.2; p: 0.6). Figure 1. When golimumab was used as first or second biologic treatment, it had a better retention rate than when it was used as third or fourth, but did not reach statistical significance (HR: 2.3; IC 95%: 0.8–6.2; p=0.1). 18/48 patients (37.5%) withdrew golimumab treatment. 13/18 (72.2%) due to lack of efficacy, 1/18 (0.6%) due to adverse events and 4/18 (22.2%) due to other reasons. Conclusions Real-world Golimumab retention rate in patients with PsA was good and did not depend on concomitant treatment with sDMARD. When used as first or second biologic, Golimumab retention rate tended to be better. Disclosure of Interest None declared

Background The clinical coexistence of Systemic Lupus Erythematosus (SLE) and Sjogren’s Syndrome (SS) was recognised 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. Objectives To address the clinical and serologic features of SLE and differences from SLE that occurs in overlap with SS. Methods A retrospective cohort of 3575 unselected SLE patients from 45 Rheumatology Units across Spain was 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, Raynaud’s 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-DNA, anti-β2-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, Raynaud’s 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 characterised by milder lupus: older age at death, similar rates of mortality and SLICC-ACR damage index, less renal and immunological manifestations. Disclosure of Interest None declared

Background The major SARD have an increased mortality compared to the general population. It is well known that the main causes of death in Systemic Lupus Erythematosus (SLE) are infections (INF), cardiovascular events (CV), neoplasia (NEO) and disease activity. However, the compared mortality of Mixed Connective Tissue Disease (MCTD), Systemic Sclerosis (SSc), Poly/Dermatomyositis (PM/DM), overlap syndromes (OS), Sjogren9s syndrome (SS), Antiphospholipid syndrome (APS), systemic vasculitis (SV), and undifferentiated or incomplete Connective Tissue Disease (UCTD) is poorly described. Objectives To analyze the causes of death and the autoantibodies (AAB) profile among the SARD. Methods This was a single center, prospective and observational study. Mortality by all causes and relationship with AAB profile were analyzed in patients diagnosed of SLE, MCTD, SSc, PM/DM, OS (simultaneous or sequential criteria of 2 or more SARD), SS, APS, SV and UCTD or incomplete SARD (at least one clinical criterion of the classification criteria and a related antibody of any of the SARD). Data were obtained from the “Systemic Autoimmune Rheumatic Diseases Registry” of a tertiary referral hospital from 1986 to 2016. Patients with rheumatoid arthritis were excluded. The SARD registry counts with the institutional review board approval. Results 1750 patients were included, of whom 1453 (83%) were women. Five hundred fifty six SLE, 125 SSc, 111 PM/DM, 91 OS, 90 MCTD, 250 SS, 71 APS, 211 SV, 117 UCTD and 128 losses to follow-up, the global follow up rate was 92.7%. A global mortality of 350 (20%) cases was observed: 101 INF (28,8%), 89 CV (25,4%), 51 NEO (14,5%), 45 due to disease activity (12,8%), 41 other causes (11,7%) and 23 from unknown causes (6,5%). Table 1 shows detailed mortality causes compared by diseases. A higher mortality was associated (p Conclusions The main causes of death among SARD patients are CV (MCTD, SLE, and SSC), severe infections (OS, SV, and PM/DM), disease activity (APS) and neoplasia (SS). A higher mortality is observed among ANCA positive SV, anti-topoisomerase I positive SSC, MCTD, OS, anticardiolipin and myositis-specific positive patients. Disclosure of Interest None declared

Background Lung fibrosis (LF) is a type of interstitial disease that leads to lung scarring, respiratory failure and later on, death. There are 2 main types of LF: idiopathic and secondary; and the prognosis is very different. LF becomes relevant in connective tissue diseases (CTD) and some studies have suggested that there could be an association between anti-Ro/TRIM21 antibodies and the development of interstitial lung disease in these patients. Objectives The aim of this study was to assess if the presence of anti-Ro52/TRIM21 antibodies is an independent risk factor for developing CTD-associated LF. We also aimed to evaluate the initial manifestations of systemic diseases and clinical characteristics linked to certain antibodies. Methods It is a prospective, observational, longitudinal, single-center study conducted among unselected patients with CTD (rheumatoid arthritis, systemic lupus erythematosus (SLE), systemic sclerosis (SSc), polymyositis (PM), overlap CTD syndrome (OCTD), MCTD, primary Sjogren9s sd (PSS), primary antiphospholipid sd, systemic vasculitis, and undifferentiated connective tissue disease). We analysed data from 1,432 caucasian patients included in the “Systemic Autoimmune Diseases (SAD) Registry” from 1988 to 2014. They were all checked at least biannually and blood samples were taken according to clinical practice. Exclusion criteria were LF as the initial manifestation of the SAD, LF already diagnosed at the first visit and also LF secondary to drugs or a specific environment. Results 10% of patients included in the study developed LF. The OR for LF in patients with anti-Ro52/TRIM21 antibodies was 1.757 (95%CI=1.1–2.7). The OR for LF increased with every year of age (OR=1.03, 95%CI=1.02–1.04). Only 9 out of 146 patients with LF were positive for Anti-La/SS-B antibodies, and the OR for males was 8.6 compared to women (95%CI=1.8–39.5). Patients with SSc and PM showed a higher OR for the development of LF (6.9 95%CI=4.6–10.4 and 2.0, 95% CI=1.2–2.8 respectively) compared to those diagnosed with other CTD. The time passed from the first symptoms to the diagnosis of LF was inversely related to the age of onset (r=-.230; p=0.013). The average time was 60 months for patients with anti-Ro52/TRIM21 antibodies (29 patients) and 138 months for patients without them. Conclusions Anti-Ro52/TRIM21 antibodies have been proved to be a risk factor for developing LF. The earlier the age of onset, the slower the progression to fibrosis. However, patients with anti-Ro52/TRIM21 antibodies tend to have a faster development, independent of the age of onset. Anti-La-SS-B antibodies seem to be a protective factor for the development of LF in both genders; but the association is stronger in women. Disclosure of Interest None declared

Background Mixed Connective Tissue Disease (MCTD) is a systemic autoimmune rheumatic disease (SARD) characterized by clinical manifestations of systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and polymyositis (PM) and the presence of anti-U1-RNP antibodies. Objectives To determine whether there are patients with symptoms of MCTD in the absence of anti-U1-RNP antibodies. Methods This was a monocentric, prospective, observational study of patients with SARD. All patients diagnosed of MCTD according to Kasukawa and/or Alarcόn-Segovia9s criteria, SLE, SSc, PM, overlap syndromes (simultaneous or sequential criteria of 2 or more SARD), Sjogren9s syndrome, Antiphospholipid syndrome, systemic vasculitis and undifferentiated or incomplete SARD (at least one clinical criterion of the classification criteria and a related antibody of any of the SARD) were included in the “Autoimmune Systemic Rheumatic Diseases Registry” of the Hospital General Universitario Gregorio Maranon Rheumatology Department from 1986 to 2012. The registry includes 2406 patients diagnosed with SARD. Patients with rheumatoid arthritis were excluded. Patients with clinical MCTD criteria were divided into seropositive (MCTD, with anti-U1RNP) and seronegative (possible MCTD, without anti-U1RNP). The registry counts with the local Institutional Ethics Board approval. Results A total of 692 patients were recruited, 608 women (87.9%). Seventy (70, 10.1%) patients were classified as seropositive and 75 (10.8%) as seronegative by Kasukawa9s criteria. Sixty-two (62, 8.9%) patients were classified as seropositive and 54 (7.8%) as seronegative according to Alarcόn-Segovia9s criteria. There were no significant differences in age at disease onset, age at diagnosis or disease duration (p>0.05) between seropositive and seronegative patients. Seropositive patients with Kasukawa9s criteria presented more frequently: lymphadenopathy, malar rash, leukopenia, Raynaud9s phenomenon, muscle weakness and increase of muscle enzymes (Table 1). By Alarcόn-Segovia9s criteria, patients who developed myositis were more frequent in the seropositive group (p=0.007, OR 3.25, 95% CI, 1.44–7.32). Conclusions Some patients with SARD manifestations fulfill MCTD clinical criteria, both Kasukawa9s and Alarcόn-Segovia9s, in the absence of anti-U1-RNP antibodies from the onset of the disease and throughout its evolution (seronegative MCTD). The frequency of seronegative MCTD was similar to the frequency of seropositive MCTD. Patients with seropositive MCTD presented more frequently manifestations of SLE (lymphadenopathy, malar rash and leukopenia) when using Kasukawa9s criteria and of PM when using both criteria. Disclosure of Interest None declared

This cross-sectional observational study aimed to explore the relationship between B cell count and ultrasound (US)-detected synovitis, in patients with rheumatoid arthritis treated with rituximab. Thirty-seven consecutive RA patients treated with RTX were recruited for the study. The patients underwent clinical [i.e., Disease Activity Score 28 joints (DAS28)], laboratory, and US assessment of 12 joints. Each joint was semiquantitatively (0-3) scored on B-mode and power Doppler mode. The scores were summed, and a global index was created for BM (BMS) and PD scores (PDI) synovitis. BM subclinical synovitis was evident in all patients, with PD synovial signal detected in 16 patients (43.2 %). No correlation was found between DAS28 and US scores. B cells were detected in 27 (72.9 %) patients, but there was no association in the mean B cell count and disease activity as measured by DAS28 (DAS28 2.6 = 34.53, DAS28 2.6 = 49.45, p = 0.52) and PDI score (PDI 1 = 49.48, PDI 1 = 35.44, p = 0.54). There was no correlation between the B cell count and DAS28, BMS, and PDI (r = 0.020, p = 0.907; r = -0.151, p = 0.371; r = -0.099, p = 0.558, respectively). In RTX-treated RA patients, no relationship could be established between US-detected synovitis and peripheral blood B cell count.

The aim of the study was to investigate the predictive value of different reduced joint ultrasound (US) assessments of synovitis and tenosynovitis in relation to unstable remission in a cohort of rheumatoid arthritis (RA) patients on methotrexate therapy. Forty-seven RA patients (38 women, 9 men), being treated with methotrexate (MTX), in clinical remission as judged by their consultant rheumatologist were evaluated for disease activity according to the Disease Activity Score (DAS) 28 at baseline and 6 months. Sustained remission and unstable remission were defined according to the baseline and 6-month DAS28 and changes in RA therapy during the follow-up. Each patient underwent at baseline a B-mode and power Doppler (PD) assessment of 44 joints and 20 tendons/tendon compartments by a rheumatologist blinded to the clinical and laboratory data. B-mode synovial hypertrophy (SH), synovial PD signal, B-mode tenosynovitis, and Doppler tenosynovitis were scored 0–3. The presence and index of synovial PD signal in 44 joints [odds ratio (OR) 8.21 (p = 0.016) and OR 2.20 (p = 0.049), respectively] and in 12 joints [OR 5.82 (p = 0.041) and OR 4.19 (p = 0.020), respectively], the presence of SH in wrist and MCP joints [OR 4.79 (p = 0.045)], and the presence of synovial PD signal in wrist–MCP–ankle–MTP joints [OR 4.62 (p = 0.046)] were predictors of unstable remission. The 12-joint or wrist–hand–ankle–MTP US assessments can predict unstable remission in RA patients in apparent clinical remission being treated with MTX.

Objective To investigate the predictive value of synovitis detected by Doppler US in relation to failed tapering of biologic therapy (BT) in RA patients in sustained clinical remission. Methods A total of 77 RA patients (52 women, 25 men) in sustained clinical remission, treated with a stable dosage of BT were prospectively recruited. BT was tapered according to an agreed strategy implemented in clinical practice (i.e. increasing the interval between doses for s.c. BT and reducing the dose for i.v. BT). BT tapering failure was assessed at 6 and 12 months. Doppler US investigation of 42 joints for the presence and grade (0-3) of B-mode synovial hypertrophy and synovial power Doppler signal (i.e. Doppler synovitis) was performed at baseline by a rheumatologist blinded to clinical and laboratory data. Hand and foot radiographs were obtained at baseline and at 12-month follow-up. Results Of the 77 patients, 46 (59.7%) were on s.c. BT and 31 (40.3%) on i.v. BT. At 12 months, 35 patients (45.5%) presented BT tapering failure, 23 of them (29.9% of all patients) in the first 6 months of BT tapering. In logistic regression analysis, the baseline DAS28 and the global score of Doppler synovitis were identified as independent predictors of BT tapering failure at 12 and 6 months. The presence of Doppler synovitis was the strongest predictor for BT tapering failure. No patient showed radiographic progression. Conclusion Our results suggest that the presence of Doppler-detected synovitis may predict BT tapering failure in RA patients in sustained clinical remission.

The primary objective of this study was to describe and compare clinical and musculoskeletal (MS) ultrasound (US) features between psoriatic arthritis (PsA) patients treated with full and tapered dosage of biologic (b) disease-modified antirheumatic drugs (DMARDs). The secondary objective was to compare clinical and MSUS features between PsA patients treated with bDMARDs with and without concomitant synthetic (s) DMARDs. We evaluated 102 patients with PsA treated with bDMARDs. The bDMARD dosage tapering had been made in patients with a maintained remission or minimal disease activity (MDA) according to their attending rheumatologist and with the patient acceptance. The bDMARD tapering consisted of the following: increase the interval between doses for subcutaneous bDMARDs or reduction of the dose for intravenous bDMARDs. The clinical evaluation consisted of a dermatologic and rheumatologic assessment of disease activity. The presence of B-mode and Doppler synovitis, tenosynovitis, enthesopathy, and paratenonitis was investigated by a rheumatologist blinded to drug dosage, clinical assessments, and laboratory results. Seventy-four (72.5 %) patients received full dosage of bDMARDs and 28 (27.5 %) received tapered dosage. The duration with biologic therapy and with current biologic therapy was significantly higher in patients with tapered dosages (p = 0.008 and p = 0.001, respectively). We found no significant differences between clinical, laboratory, and US variables, both for BM and CD between patients with full and tapered dosage and between patients with and without concomitant sDMARD. Clinical assessment, MSUS variables, and MDA status are similar in patients receiving full and tapered dosage of bDMARDs.

Background Rituximab (RTX) is a chimeric monoclonal anti-CD20 antibody. Several studies have proved its safety when used in different autoimmune chronic rheumatic diseases (ACRD). However, the real conditions of daily practice are not comparable to the ideal frame of a clinical trial. Objectives The aim of this study is to describe the profile of safety of RTX in patients with ACRD 12 and 24 months after the administration of the first cycle. Methods It is an observational retrospective study of patients diagnosed with ACRD who received treatment with RTX. Data were collected on demographic, clinical and biochemical profiles right before the first infusion, 12 and 24 months later. Other parameters were concomitant medication, prednisone dose, previous biologic treatment, accumulated RTX dose, infusion and anaphylactic reactions, infections, tumor relapses.Comparison was done using Wilcoxon non parametric test. Results Data were collected from 50 patients that received RTX. 24 months after treatment no major cytopenias or hypogammaglobulinemia were found. Hepatic and lipid profile did not show any alterations and complement levels did not experiment relevant changes. The average dose of prednisone was reduced significantly 1 year after the treatment. A decline in levels of acute phase reactants and antibodies was observed. This effect was maintained in every parameter with the exception of the rheumatoid factor; which continued presenting a downwards trend after 24 months. Concerning the infections, 3 patients were hospitalised (because of severe infections): two of them suffered from pneumonia and the other one required a surgical intervention in order to replace an infected prosthetic knee. 14 mild infections were registered: 8 mucocutaneous, 2 gastrointestinal, 2 respiratory and 2 genitourinary. There were no cases of primoinfection, reactivation of hepatic viruses or mycobacteria. No tumoral relapses, new diagnosis of tumors or demyelinising diseases were observed. 5 patients experimented mild infusion reactions but none of them had an anaphylactic reaction or died. Conclusions RTX has reduced the average maintenance dose of prednisone and also lowered the levels of serum immunoglobulins (including antibodies) without inducing hypogammaglobulinemia. It has proved to be a relatively safe treatment that does not cause hematic, hepatic toxicity or deep immunosuppresion after 12 and 24 months of follow up in patients with ACRD. Further studies are needed in order to evaluate long term safety of RTX. References Becerra E et al; Long-term Safety of Rituximab in Patients With Rheumatoid Arthritis. Int J Clin Rheumatol. 2012;7(4):383–390. Disclosure of Interest None declared

Background Biologicals therapies have been used for the treatment of Systemic Lupus Erythematosus (SLE) but only Belimumab has been approved by the European Medicines Agency. Abatacept (ABA), a T-cell co-stimulation blocker, has been proposed as an effective treatment for SLE due to its mechanism of action. Three randomised clinical trials have been performed with ABA in patients with SLE. Although any of these trials met the primary outcome of efficacy, it is still considered as an “off-label” option when none of the other conventional therapies have worked. Objectives To assess effectiveness and safety of ABA in patients diagnosed of SLE in a Rheumatology service from a tertiary care hospital in Madrid, Spain. Methods We performed an observational retrospective study in patients with SLE (according to the 1997 American College of Rheumatology classification criteria) who received treatment with ABA. We recorded demographic, clinical and laboratory parameters at the beginning of treatment, and after 6 and 12 months of treatment. We also recorded adverse events. Results We included 8 patients, all of them women. The median age was 45.13 years (range 30–67 years) and the median time from SLE diagnosis to ABA treatment was 12.12 years (range 3–21 years). Three patients had an overlap disease with Rheumatoid Arthritis. The most frequent symptoms were arthritis (n=5), cutaneous (n=3) and neurological symptoms (n=5), specifically organic brain syndrome (n=4) and lupus headache (n=3). None of the patients had renal disease. In all patients ABA was use after anti-TNFα or Rituximab treatment. One patient discontinued ABA after 2 months due to inefficacy, so she was excluded from statistical analysis. Table 1 shows clinical and laboratory parameters at the beginning of treatment and after 6 and 12 months of treatment. After 6 months of treatment, disease activity according to SLEDAI decreased. One patient persisted with organic cerebral syndrome. After 12 months of treatment neurological symptoms showed a substantial improvement in every patient that presented them at the beginning. Four patients showed significant improvement of arthritis. Two patients experienced serious infections that required hospitalization. Conclusions ABA was effective in our SLE patients. Clinical response was evident at 6 months and persisted at 12 months. ABA may be a therapeutic option in SLE patients with inadequate response to conventional therapies, specially if arthritis or neurological symptoms are present. References Aringer M. Expert Opin Drug Saf. 2015;14:243–51. Ikeda K. Clin Dev Immunol. 2013;2013:697525. Disclosure of Interest None declared

Objective To investigate the sensitivity for detecting subclinical synovitis of different reduced joint ultrasound (US) assessment models as compared with a comprehensive US assessment in rheumatoid arthritis (RA) patients in clinical remission. Methods Sixty-seven RA patients (50 women, 17 men) in clinical remission as judged by their consultant rheumatologist and treated with methotrexate were prospectively recruited. Patients were evaluated for disease activity according to the Disease Activity Score in 28 joints (DAS28) and the Simplified Disease Activity Index (SDAI) by the same investigator. Each patient underwent a 44-joint B-mode and power Doppler (PD) assessment by a rheumatologist blinded to the clinical and laboratory data. B-mode synovial hypertrophy (SH) and synovial PD signal were scored from 0–3 at each joint. Global indices for SH and PD signal were calculated for the 44-joint and different joint combination models for each patient. Results SH was detected in 87.8% of patients with a DAS28

Background Psoriatic arthritis (PsA) is an inflammatory spondyloarthritis associated with psoriasis. Over the recent years the treatment of PsA has changed dramatically with the use of synthetic (s) disease modified anti-rheumatic drugs (DMARDs) as well as biologic (b) DMARDs, particularly tumor necrosis factor inhibitors. The goal of all these therapies is to achieve remission or minimal disease activity (MDA) [1]. Once remission is achieved and sustained the question is whether the treatment should be continued at full dosage, at a tapered dosage or should be discontinued. For PsA there are no recommendations for dosage tapering or discontinuation of biologic therapy and literature data regarding maintained clinical remission is conflicting. B-mode (BM) musculoskeletal (MS) ultrasound (US) has been widely shown to be more sensitive than clinical assessment in detecting joint synovitis and enthesitis [2,3] and Doppler technique is able to identify inflammatory activity [3,4]. Objectives To describe and compare clinical and MSUS features between PsA patients treated with full and tapered dosage of bDMARDs. The secondary objective was to compare clinical and MSUS features between PsA patients treated with bDMARDs with and without concomitant sDMARDs. Methods We evaluated 102 patients with PsA treated with bDMARDs. The bDMARD dosage tapering had been made in patients with a maintained remission or MDA according to their attending rheumatologist and with the patient acceptance. The bDMARDs tapering consisted of the followings: increase the interval between doses for subcutaneous bDMARDs or reduction of the dose for intravenous bDMARDs. The clinical evaluation consisted of a dermatologic and rheumatologic assessment of disease activity. The presence of BM and colour Doppler (CD) synovitis, tenosynovitis, enthesopathy and paratenonitis was investigated by a rheumatologist blinded to drug dosage, clinical assessments and laboratory results. Results Seventy four (72.5%) patients received full dosage of bDMARDs and 28 (27.5%) received tapered dosage. The duration with biologic therapy and with current biologic therapy were significantly higher in patients with tapered dosages (p=0.008 and p=0.001, respectively). We found no significant differences between clinical, laboratory and US variables, both for BM and CD between patients with full and tapered dosage and between patients with and without concomitant sDMARD. Conclusions Clinical assessment, MSUS variables, and MDA status are similar in patients receiving full and tapered dosage of bDMARDs. References Gossec L et al. Ann Rheum Dis. 2012 Jan;71(1):4-12 Balint PV et al. Ann Rheum Dis 2002;61:905-10 D9Agostino MA et al. Arthritis Rheum 2003;48:523-33 Naredo E et al. Arthritis Rheum 2008;58:2248-56 Disclosure of Interest None declared

Background Several studies have shown the relationship between anti-SSA-Ro/SSB-La antibodies and Systemic Lupus Erythematosus (SLE), Sjogren Syndrome (SS) and other autoimmune diseases in adult population. However, the expression of these autoantibodies and clinical correlation in juvenile patients is poorly described. Objectives To characterize the clinical and serological profile and primary rheumatic diseases in pediatric patients with positive anti-SSA-Ro and/or anti-SSB-La antibodies. Methods The data was obtained from a long term prospective cohort of patients under age 18 diagnosed with rheumatic diseases in a tertiary hospital in Spain. Demographic, clinical, and laboratory data were collected from 1986 to 2010. Patients were divided into 2 groups: anti-SSA-Ro/SSB-La positive and anti-SSA-Ro/SSB-La negative. Results A total of 187 patients were tested for anti Extractable Nuclear Antigens (ENA), with a following mean time of 11 years. Mean age at disease onset was 12.6 years and 77% were female. Fifty-four (28.9%) anti-SSA-Ro/SSB-La positive subjects were compared against 133 (71.1%) anti-SSA-Ro/SSB-La negative subjects. Among positive cases, 13 (24.1%) patients were double-positive for anti-SSA-Ro and anti-SSB-La, 51 (94.4%) were positive for anti-SSA-Ro and 3 (5.5%) were single-positive for anti-SSB-La. The anti-SSA-Ro/SSB-La antibodies were found less frequently (p=0.003) in the overlapping syndromes, and more frequently in SLE (p=0.007). In addition rheumatoid factor (p Conclusions Similarly to adults, we observed a relationship between anti-SSA-Ro/SSB-La antibodies and SLE in pediatric patients. However a low proportion of childhood primary SS exists in our anti-SSA-Ro/SSB-La positive cases. This could be explained by underdiagnoses related to the atypical clinical presentation of SS in pediatric population. Single-positive anti-SSB-La patients are uncommon, the clinical significance of this serological result remains uncertain in children. References Rheumatol Int (2014) 34:1123–1127. Disclosure of Interest None declared

Background Musculoskeletal ultrasound (MSU) is a useful, non-invasive, nonionizing, quick and well-tolerated imagine technique, appropriate for assessing children. In the last years, MSU has been increasingly used in pediatric rheumatology. Most studies have been on juvenile idiopathic arthritis (JIA) patients, showing more sensitivity of MSU for detecting synovitis than clinical examination. The impact of MSU in adult patients with rheumatic diseases have been studied, but there is a lack of studies on the impact of MSU in pediatric rheumatology. Objectives To investigate the impact of MSU in diagnosis and therapeutic decisions in a pediatric rheumatology outpatient clinic. Methods We included patients who attended consecutively our pediatric rheumatology unit in 11 random days during a 4-month period (September-December 2013). A consultant rheumatologist with 25 years of experience in pediatric rheumatology decided whether MSU was indicated or not, and completed a standard questionaire. This questionnaire was composed of clinical data, previous diagnosis, current global and local diagnosis, global and local treatment decisions and disease activity assessment. The level of Indication of MSU was measure by a Likert scale from 0 (not neccesary) to 5 (very neccesary). MSU of selected joints was performed by a rheumatologist ultrasonographer. MSU findings were provided to the consultant rheumatologist immediately after the MSU examination. The consultant rheumatologist completed a second questionaire with global and local changes in diagnosis and treatment decisions, and changes in disease activity. Results The study included 111 patients (38 (34,2%) male and 73 (65,8%) female). Fifteen (13,5%) were new patients and 96 (86,5%) were follow-up patients. Fifty-one (45,8%) patients had already been diagnosed with JIA. The consultant rheumatologist asked for a MSU in 66 patients (59,5%) with a mean indication level of 3,48 (SD 1,25) (range 1-5). A clinical joint examination was performed in 67 patients with a total of 107 joints assessed. From these joints, in 92 (86%) a MSU examination was also performed. Table 1 shows number and percentage of changes in diagnosis and treatment decisions, and changes in disease activity. From 51 patients with JIA diagnosis, 42 (82,4%) were assessed with MSU for disease activity with changes in 19 (45,2%) patients. Conclusions MSU changed global and local diagnosis, treatment decisions, and disease activity in a relevant number of patients in our pediatric rheumatology unit. References Micu MC, Alcalde M, Saenz JI, Crespo M, Collado P, Bolboaca SD, Naredo E. Impact of musculoskeletal ultrasound in an outpatient rheumatology clinic. Arthritis Care Res 2013;65:615-21. Disclosure of Interest J. C. Nieto-Gonzalez: None declared, I. Monteagudo Consultant for: Abbvie, Roche Farma, Bristol- Myers Squibb, Pfizer, UCBL, E. Naredo Consultant for: Abbvie, Roche Farma, Bristol- Myers Squibb, Pfizer, UCB, General Electric Healthcare, and EsaoteL, L. Vargas-Henny: None declared, I. Janta: None declared, M. Hinojosa-Davila: None declared, L. Martinez-Estupinan: None declared, M. Montoro: None declared, J. G. Ovalles-Bonilla: None declared, L. Carreno: None declared DOI 10.1136/annrheumdis-2014-eular.4206

Background Many observational studies have shown responsiveness of ultrasound (US)-detected synovitis in rheumatoid arthritis (RA) patients, most of them using biologic therapies. However, these studies have not taken into consideration in their design the pharmacokinetic characteristics of the drug administered. Objectives To investigate whether the pharmacokinetics (i.e. time point of US assessment) of subcutaneous anti-Tumour Necrosis Factor (anti-TNF) agents influences the grade of US-detected synovitis in RA patients treated with these drugs. Methods Fifty consecutive RA patients (31 women, 18 men) were prospectively recruited from the Biologic Therapy Unit of our hospital. Inclusion criteria consisted of being in treatment with subcutaneous anti-TNF agents and having had neither changes in therapy nor local corticosteroid injections in the previous 3 months. Patients underwent clinical, laboratory and US assessment at two time points, i.e., at peak plasma drug concentration and at trough plasma drug concentration. Patients were assessed at both time points for disease activity according to the Disease Activity Score in 28 joints (DAS28) and the Simplified Disease Activity Index (SDAI) by the same investigator. US assessments were performed by a rheumatologist expert in musculoskeletal US who was blinded to the anti-TNF agent, the administration time, and the clinical and laboratory data. Twenty-eight joints were investigated for the presence and grade (0-3) of B-mode synovitis and synovial PD signal (i.e. Doppler synovitis). A global index for B-mode synovitis (BSI), joint count for B-mode synovitis (BSC), a global index for Doppler synovitis (DSI), and joint count for Doppler synovitis (DSC) were calculated for a 12-joint US scoring model (31) and a wrist-hand-ankle-foot US scoring model. B-mode US remission was defined as a BSI Results Twenty (40.8%) patients were treated with etanercept, 19 (38.8%) with adalimumab, 6 (12.2%) with golimumab, and 4 (8.2%) with certolizumab. There were no significant differences between the clinical, laboratory and B-mode and Doppler US indices and joint counts for both the 12-joint US model and the wrist-hand-ankle-foot model at peak time and trough time (p=0.132-0.986). There were no significant differences between the proportion of patients with active disease and those in remission according to DAS28, SDAI, B-mode US, and Doppler US at peak time and trough time assessments (p=0.070-1). DAS28, Disease Activity Score in 28 joints; SDAI, Simplified Disease Activity Index; B-mode synovitis index; BSC, B-mode synovitis count; DSI, Doppler synovitis index; DSC, Doppler synovitis count; W, wrist; A, ankle; Diff, values at peak time–values at trough time. Conclusions Our results suggested that subcutaneous anti-TNF pharmacokinetics does not significantly influence US-scored synovitis in RA patients. Disclosure of Interest : M. Hinojosa-Davila: None declared, E. Naredo Grant/research support: Esperanza Naredo has received speaker fees from Abbvie, Roche Farma, Bristol-Myers Squibb, Pfizer, UCB, General Electric Healthcare, and Esaote. Esperanza Naredo has received research funding from UCB and MSD., L. Valor: None declared, D. Hernandez: None declared, C. Mata-Martinez: None declared, B. Serrano-Benavente: None declared, N. Bello: None declared, J. G. Ovalles-Bonilla: None declared, J. Martinez-B.: None declared, L. Martinez-E.: None declared, J. C. Nieto: None declared, T. Del Rio: None declared, C. Gonzalez: None declared, J. Lόpez-Longo: None declared, M. Montoro: None declared, I. Monteagudo: None declared, L. Carreno: None declared DOI 10.1136/annrheumdis-2014-eular.2966

Objectives To investigate the predictive value of synovitis detected by Doppler ultrasound (DUS) in relation to successful tapering of BT in RA patients in sustained clinical remission. Methods Seventy-seven RA patients (52 women, 25 men) treated with BT were prospectively recruited from the Biologic Therapy Unit of our hospital. Inclusion criteria were as follows: 1) being in treatment with stable doses of BT and synthetic disease modifying antirheumatic drugs (DMARDs) in the previous 12 months; 2) being in sustained clinical remission judged by their usual consultant rheumatologist and according to Disease Activity Score in 28 joints (DAS28) or Simplified Disease Activity Index (SDAI) criteria in the previous 12 months; 3) being in treatment with ≤5 mg/day of prednisone in the previous 6 months; 4) having needed neither nonsteroidal anti-inflammatory drugs (NSAIDs) for more than 1 week nor local corticosteroid injections in the previous 6 months. BT was tapered at baseline according to an agreed strategy implemented in clinical practice (i.e. increasing the interval between doses for subcutaneous BT and reducing the dose for intravenous BT). Patients underwent clinical and laboratory assessment every 3 months. DUS investigation of 42 joints for the presence and grade (0-3) of B-mode synovitis and synovial power Doppler (PD) signal (i.e. Doppler synovitis) was performed at baseline by a rheumatologist blinded to therapy, clinical and laboratory data. A global index for B-mode synovitis (BSI) and a global index for Doppler synovitis (DSI) were calculated. Hand and foot radiographs were obtained at baseline and at 12-month followup. BT tapering failure was assessed at 6 and 12 months. BT tapering failure was defined as an increase in BT doses by their usual consultant rheumatologist and/or the presence of clinical disease activity according to both DAS28 and SDAI criteria. Results Twenty-three (29.9%) patients were treated with adalimumab, 21 (27.3%) with etanercept, 18 (23.4%) with infliximab, 7 (9.1%) with tocilizumab, 6 (7.8%) with abatacept, and 2 (2.6%) with golimumab. Thirty-five (45.5%) patients presented tapering failure at 12 months, 23 (29.9%) of them before 6 months of BT tapering. Longer duration of RA (p=0.009), higher number of previous synthetic DMARDs (p=0.003), higher DAS28 at baseline (p=0.011), higher SDAI at baseline (p=0.003), higher DSI at baseline (p 0 at baseline (p 0 at baseline (p Conclusions Our results suggested that the presence and grade of Doppler-detected synovitis may predict BT tapering failure in RA patients in sustained clinical remission. Disclosure of Interest : E. Naredo Grant/research support: UCB and MSD, Consultant for: Abbvie, Roche Farma, Bristol-Myers Squibb, Pfizer, UCB, General Electric Healthcare, and Esaote, L. Valor: None declared, I. De la Torre: None declared, M. Montoro: None declared, N. Bello: None declared, J. Martinez-Barrio: None declared, L. Martinez-Estupinan: None declared, J. C. Nieto: None declared, J. G. Ovalles-Bonilla: None declared, D. Hernandez: None declared, C. M. Gonzalez: None declared, J. Lόpez-Longo: None declared, I. Monteagudo Consultant for: Abbvie, Roche Farma, Bristol-Myers Squibb, Pfizer, UCB, L. Carreno: None declared DOI 10.1136/annrheumdis-2014-eular.3013

Background Antisynthetase syndrome (ASS) is a rare condition characterized by the presence of one or more of the following clinical features: arthritis, Raynaud9s phenomenon, “mechanic9s hands”, interstitial lung disease and polymyositis, in patients with antisynthetase antibodies which target aminoacyl-tRNA synthetases enzymes, being Anti-Jo1 the most common one. Objectives To investigate the clinical features of ASS at disease onset and their influence on the disease clinical course. Methods 32 consecutive patients diagnose with ASS, were prospectively enrolled between 1988 and 2013. Inclusion criteria consisted of having a positive test for antisynthetase antibodies, regardless of fulfilling classification criteria for other rheumatic diseases. Serological, clinical and demographic features were registered both at clinical onset and during the course of the disease. Anti-synthetase antibodies were determined by LIA or ELISA. Results From 32 patients, 21 (65.6%) were females and 11 (34.4%) were males. Mean age at clinical onset was 47.4 years (SD 18.8) and the mean time of disease course was 11.1 years (SD 10.2). The most common feature at disease onset was arthritis in 50% of the patients (8 of them presenting arthritis>6 weeks), followed by constitutional syndrome (31.3%) and muscular symptoms (25%). Table 1 shows the different clinical features and frequency of occurrence at onset. During the course of disease 28 patients (87.5%) developed musculoskeletal manifestations, 26 (81.3%) arthritis, 21 (65.6%) cutaneous manifestations, 20 (62.5%) interstitial lung disease and 13 (40.6%) Raynaud9s phenomenon. Associations between the presence of Anti-Ro52 and arthritis at disease onset (p=0.009), interstitial lung disease (p=0.002) and low complement levels (p≤0.001) were found. Conclusions In our study, clinical features at onset were very heterogeneous. Patients showed neither all clinical features at the same time nor during the disease course. In our cohort, Anti-Ro52 antibody was associated to both the presence of arthritis at onset and the development of interstitial lung disease, which could be relevant for differential diagnosis and prognosis. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.3113

Background Currently data regarding long-term outcome of juvenile systemic sclerosis (jSSc) is scarce. Objectives To describe the differences between patients with jSSc versus adult onset evaluated at a single medical center. Methods Patients with disease onset before the age of 17 years were included in the jSSc group, while subjects with SSc onset after age 18 formed the adult-onset cohort. The 2 groups were compared with respect to disease classification, clinical, serological data, and mortality. Results Seventeen jSSc cases seen between 1986 and 2011 were compared with 120 adult onset SSc cases. Mean age at onset in the jSSc group was 11.7±3.9 years, and the mean disease duration at the last follow-up was 19.3±7.8 years. The sex distribution was 7.5:1 (female:male). Among juvenile cases, 58.8% had diffuse SSc, while 41.2% had limited SSc. The clinical features seen in juvenile patients were: articular manifestations (arthralgia, arthritis, contractures and weakness) in the 100% of cases, Raynaud’s phenomenon in 94%, digital ulcers and telangiectasia in 53%, interstitial lung disease in 11.8%. Antinuclear antibody was positive in 100%, and anti-U1RNP in 64.7%. A global mortality of 17.6% was seen. Articular manifestations, anti-DNA and anti-U1RNP were seen more frequently in the jSSc group. Adult onset SSc develop a higher frequency of sclerodactyly and interstitial lung disease. Conclusions Diffuse SSc, limited SSc, calcinosis, digital ulcers, pulmonary hypertension, anti-centromere, anti-Scl-70 and mortality shows a similar distribution between the 2 groups. Patients with jSSc presented a lower frequency of interstitial lung disease and sclerodactyly, but a higher expression of anti-DNA and anti-U1RNP. Disclosure of Interest None Declared

Background Among the rheumatic diseases, Systemic Sclerosis (SSc) stands out as a severely incapacitating and life-threatening disease. Objectives To analize the causes of death, survival and risk factors for mortality in SSc patients. Methods Demographic, clinical, immunological and mortality data were obtained from a long term prospective cohort of SSc patients recruited between 1986 and 2011 in the Rheumatology Department of Gregorio Maranon Hospital in Madrid, Spain. Patients were divided into 4 groups: limited SSc, diffuse SSc, SSc in overlap and SSc in mixed connective tissue disease (SSc-MCTD). ANOVA, Kruskal-Wallis or χ 2 tests were used to identify differences among groups; Kaplan-Meier and Mantel-Haenszel (log-rank) analysis were used to estimate survival, and Cox proportional hazards regression analysis was used to identify factors associated with mortality. Results A total of 137 patients were included, of whom 122 (89%) were women. Mean age at diagnosis was 43±17.7 years, and the mean follow-up time was 15.2 years. A global mortality of 34 (24.8%) cases was observed: 8 related to cardiac involvement, 8 to serious infections, 7 to pulmonary involvement, 6 to neoplasms, 2 to renal involvement and 3 to other causes. Survival rates from disease onset were 96, 90, 75 and 50% at 5, 10, 20 and 30 years, respectively. Limited SSc and SSc-MCTD show a significantly (p=0.002) higher survival mean time (33.7 years) when compared to diffuse and overlap variants (22.4 years). Poorer survival is associated with interstitial lung disease, pulmonary arterial hypertension, severe infections, cardiac involvement, chronic kidney disease, and Anti-Ro antibodies (see table 1). Independent prognostic factors for mortality were interstitial lung disease (HR 6.8, CI 95% 1.5-30, p=0.012), pulmonary arterial hypertension (HR 5.6, CI 95% 1.2-26.9, p=0.032), and Anti-Ro antibodies (HR 3.9, CI 95% 1.5-9.8, p=0.004). Conclusions A 20-year survival was seen in more than 70% of SSc patients. The main causes of death are cardiac involvement, severe infections and pulmonary manifestations. Diffuse SSc, SSc-MCTD, interstitial lung disease, pulmonary arterial hypertension, severe infections, cardiac involvement, chronic kidney disease, and Anti-Ro antibodies are the main risk factors for mortality. Disclosure of Interest None Declared

Objectives To describe the arthritic patterns in Systemic Lupus Erythematosus (SLE) patients and their relationship with clinical and immunological features. Methods Data was obtained from a long term prospective cohort of SLE patients recruited between 1986 and 2010 in the Rheumatology Department of Gregorio Maranon Hospital in Madrid, Spain. Demographic, clinical, and laboratory data were collected at disease onset and during it’s course. Patients were divided into 4 groups according to arthritic pattern: acute oligoarthritis, chronic oligoarthritis, acute polyarthritis and chronic polyarthritis. Organ damage was scored using the SLICC/ACR Damage Index. Results A total of 522 patients were included, of whom 465 were women. Arthritis was present at diagnosis in 29.7% of patients. The chronic variants (oligo and poly) presented more frequently in association with Sjogren syndrome. Other manifestations like bony erosions, hypogammaglobulinemia and osteoporosis were considerably more frequent in the chronic polyarthritis group. A higher frequency of renal manifestations was observed in the acute arthritis groups, both at disease onset and during evolution. During the follow-up period 68.4% of patients developed arthritis. The chronic polyarthritis group shows a statistically significant association with morning stiffness, bony erosions, Sjogren syndrome, Jaccoud’s arthropathy, osteoporosis, hypogammaglobulinemia, orthopedic interventions, drug side effects and higher serum levels of rheumatoid factor. A trend towards higher titers of ACPA and anti-modified citrullinated vimentin was also seen in the chronic polyarthritis group, although the differences among groups did not reach statistical significance. Mortality, damage accrual, and systemic manifestations are not related to any arthritic pattern in particular. Conclusions At disease onset 30% of patients presented with arthritis, and 70% of all studied patients developed some kind of arthritic pattern during the course of the disease. Renal manifestations are related to acute oligo and polyarthritis. Damage accrual, systemic manifestations and mortality are not associated with any particular arthritic pattern. Disclosure of Interest None Declared

Objectives To describe the clinical and immunological features, the damage accrual and mortality of late onset compared with adult onset SLE. Methods The data was obtained from a long term prospective cohort of 353 patients diagnosed with SLE in the Rheumatology Department of Gregorio Maranon Hospital in Madrid, Spain. Demographic, clinical, and laboratory data were collected at disease onset and during it’s course from 1986 to 2006. Patients were divided into 2 groups: adult onset 19-49 years (n=276) and late onset ≥50 years (n=77). Organ damage was scored using the Systemic Lupus International Collaborative Clinics/American College of Rheumatology (SLICC/ACR) Damage Index. Results A total of 353 patients were recruited, with a following mean time of 11 years. The female to male ratio differed significantly (p=0.005) between groups. At diagnosis, the late-onset group presented cutaneous manifestations less frequently (p Conclusions Late onset SLE is clinically different with less arthritis, fever, low serum complement, cutaneous, hematologic and renal manifestations compared with the adult onset group. The higher frequency of damage accrual, mortality and hypertension observed in the late-onset group can be affected by aging-related factors other than disease activity or duration. Disclosure of Interest None Declared