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© Copyright Clinical and Experimental Rheumatology 2022., Objectives: To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods: We conducted a multicentre, international, retrospective cohort study. Results: 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusions: The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Background:Neuro-Behçet’s disease (NBD) is a severe complication of Behcet’s disease (BD). Despite well-established therapies with glucocorticoids and conventional immunosuppressants (cIS), a significant proportion of patients are refractory.Objectives:To assess efficacy and safety of biologic therapy (BT) in NBD refractory to glucocorticoids and at least one cIS.Methods:Open-label multicenter study of refractory NBD from 23 different referral Spanish Hospitals. Main outcome was neurological response. Secondarily, analytical efficacy was measured by Erythrocyte Sedimentation Rate (ESR), C-Reactive Protein (CRP) and Hemoglobin (Hb) at baseline, 6 months, 1 year and 2 years.Results:We studied 42 patients (21 women/ 21 men; mean age 40.4±10.8 years). HLA B51 was positive in 15 out of 37 (40.5%) patients tested. Non-neurological manifestations were oral ulcers (n=41, 97.6%), genital ulcers (n=31, 73.8%), skin lesions (n=28, 66.7%), arthralgia (n=27, 64.3%), uveitis (n=21, 50.0%), arthritis (n=9, 21.4%), venous thrombosis (n=9, 21.4%) and arterial thrombosis (n=4, 9.5%). The underlying neurologic manifestation were parenchymal (n=34, 81.0 %) and non-parenchymal (n=17, 40.5%) involvement (Table 1). The first BT used was infliximab (n=20), adalimumab (n=13), golimumab (n=3), tocilizumab (n=3) and etanercept (n=2).After 58.2±51.4 months since initiation of BT, neurological response was complete (n=27; 64.3%), or partial (n=11, 26.1%) (Figure 1). Only 4 (9.5%) patients did not respond. After 6 months of BT, ESR improved from.31.5±25.6 to 15.3±11.9 mm/h (p=0.005), CRP from 1.4 [0.2-12.8] to 0.3[0.1-3] mg/dL (p= 0.002) and Hb from 13.1±1.6 to 13.8±1.3 g/dL (p=0.005).Figure 1.Neurological clinical response to biological therapy.Primary failure was observed in 16 (38.1%) patients due to inefficacy (n=11, 68.8%) or adverse effects (n=5, 31.3%). Similarly, causes of secondary failure (n=6, 14.3%) were inefficacy (n=5, 83.3%) and adverse effects (n=1, 16.7%). No serious adverse effects were observed.Conclusion:BT, especially monoclonal anti-TNF drugs, seems to be effective and safe in refractory NBD.Table 1.Neurologic manifestation of 42 patients with refractory neurobehçet's disease treated with biologic therapy.Parenchymal subtype, n (%)34 (81.0)-Hemiparesis8 (19.1)-Polineuropathy8 (19.1)-Encephalopathy6 (14.3)-Cognitive impairments4 (9.5)-Optic neuropathy4 (9.5)-Ophtalmoparesis4 (9.5)-Other cranial nerve involvement3 (7.1)-Hemihypoesthesia3 (7.1)-Cerebellar dysphasia1 (2.4)-Cerebellar involvement1 (2.4)-Non-steroidal psicosis1 (2.4)Non-parenchymal subtype, n (%)17 (40.5)-Aseptic meningitis12(28.6)-Thrombosis4 (9.5)-Intracranial hypertension1 (2.4)Disclosure of Interests:Alba Herrero-Morant: None declared, José Luis Martín-Varillas Grant/research support from: AbbVie, Pfizer, Lilly, Janssen, and Celgene, Santos Castañeda: None declared, Iñigo González-Mazón: None declared, Olga Maiz: None declared, Ana Blanco Speakers bureau: AbbVie, Julio Sánchez: None declared, Norberto Ortego: None declared, Enrique Raya Speakers bureau: MSD, Grant/research support from: AbbVie, Alejandro Olive: None declared, Anahy Brandy-Garcia: None declared, Águeda Prior-Español: None declared, Clara Moriano: None declared, Elvira Diez Alvarez: None declared, Rafael Melero: None declared, Jenaro Graña: None declared, Álvaro Seijas-López: None declared, ANA URRUTICOECHEA-ARANA: None declared, Angel Ramos Calvo: None declared, Concepción Delgado Beltrán: None declared, Marta Loredo Martínez: None declared, Eva Salgado-Pérez: None declared, Francisca Sivera: None declared, Ignacio Torre-Salaberri: None declared, J. Narváez Speakers bureau: Bristol-Myers Squibb, José Luis Andréu Sánchez: None declared, Olga Martínez González: None declared, Ricardo Gómez de la Torre: None declared, Sabela Fernández: None declared, Susana Romero-Yuste: None declared, Gerard Espinosa: None declared, Miguel Á. González-Gay Speakers bureau: AbbVie, Pfizer, Roche, Sanofi, Lilly, Celgene and MSD, Grant/research support from: AbbVie, MSD, Jansen and Roche, Ricardo Blanco Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, Sanofi, Lilly and MSD, Grant/research support from: AbbVie, MSD, and Roche

Objective: The aim of this study was to estimate the prevalence of ankylosing spondylitis (AS) in Spain.Method: This is a cross-sectional, population-based study of people aged 20 years or older in Spain. Randomly selected individuals were contacted by telephone and rheumatic disease screening was performed. If the first screening was positive, medical records were then reviewed and/or a telephone questionnaire was conducted by a rheumatologist, followed by an appointment if necessary. Cases had to fulfil the modified New York (mNY) criteria.Results: In total, 4916 individuals were included, of whom 355 had a positive screening result for AS. Of these, 11 were classified as AS. An additional individual who reported a prior diagnosis of rheumatoid arthritis had a diagnosis of AS confirmed on review of the medical records. Estimated prevalence was 0.26% (95% CI 0.14-0.49).Conclusion: EPISER2016 is the first population-based study to estimate the prevalence of AS in Spain, which has been estimated as being similar to that in other European countries.

Background Elderly onset rheumatoid arthritis (EORA) has several peculiarities. Which activity index should we use in this age group is still an unresolved issue. The use of ESR in DAS28 is widespread, but it is well known that ESR could increase with age, overestimating activity. DAS28-PCR or SDAI, which uses PCR, or CDAI that does not use analytical data, could be more suitable alternatives. Objectives To describe disease activity scores evolution in EORA and to analyse the correlation and concordance between them. Methods Longitudinal observational study in naive treatment EORA patients (ACR/EULAR 2010 criteria). Study visits were carried out basally (pre-treatment) and at month 1, 3, 6 and 12. DAS28 VSG, DAS28 PCR, SDAI and CDAI were calculated at each visit, and ACR/EULAR remission index at 12 months. The same rheumatologist made all visits and explorations. Correlations were analysed using the Spearman or Pearson correlation index (p value ≤ 0.001). The concordance was evaluated with the kappa index. The statistical study was performed with Stata 15.1. Results 45 patients with EORA were enrolled. Table 1 and 2 summarize the clinical and analytical characteristics. All the scores had a very good linear correlation both at baseline and at follow-up (correlation score: 0.83-0.98). A moderate concordance was observed from the baseline visit between DAS28-VSG and DAS28-PCR (k=0.43-0.52, p Conclusion The correlation between the different scores was good. However, the concordance between all the scores decreases as time passes, according to a higher percentage of patients in remission or low activity. This low-moderate concordance is demonstrated even between DAS28-PCR and DAS28-VSG, and when these are compared with CDAI and SDAI. CDAI and SDAI are the only ones that maintain a good concordance between them, even when the number of patients in remission or low activity increases. ACR/EULAR only maintains an acceptable concordance with the most restrictive scores, SDAI and CDAI. By contrast, agreement is very low with DAS28, especially by DAS-PCR. Disclosure of Interests None declared

Background Myositis specific antibodies have gained special importance in last years. Its knowledge has allowed stratifying patients in different clinical phenotypes, predicting with greater accuracy prognosis and establishing a clinical attitude to follow. SAE1/2 antibody (anti-small ubiquitin-like modifier activating enzyme) was first described in 2007 in patients with amyopathic dermatomyositis with cutaneous and digestive involvement. Its prevalence ranges from 8% in European cohorts to 3% in asians. Objectives Describing myositis specific antibodies (MSA) in a cohort of inflammatory myopathies. To characterize the clinical phenotype of SAE1/2 antibody and to compare it with the rest of MSA. Methods Patients diagnosed of dermatomyositis in a tertiary hospital from 1978-2018, according to the criteria of Bohan and Peter (1975) and according to Dalakas classification criteria (2015). Clinical and analytical data, including the immunological profile were collected, as well as the treatments received and the evolution of the disease. Results Out of 46, 41 dermatomyositis had positive antinuclear antibodies (ANA). 55% percent had aNA titles≥640, being the fine speckled pattern the most frequent (43%) followed by coarse speckled and homogeneous (13.6% each). 72% had MSA, the most frequent being antiJo1 (27.3%) followed by MDA5 (18.2%) and SAE1/2 (15%). Up to 40% had two or more antibodies, being the association with antibodies Ro52 and Ro60, the most frequent. 5 patients presented positivity against SAE antibody. In comparison to the rest of MSA, 80% presented with cutaneous debut (p = 0.00), being the most frequent manifestations heliotrope erythema (p = 0.00), Gottron papules (p = 0.10) and skin rash (p = 0.00). 60% had pathological capillaroscopy compared to 15% (p = 0.00). Muscular balance was preserved in 60% of patients. Sixty percent had dysphagia vs 9% (p = 0.00). Two of them had lung involvement (alveolar hemorrhage and rapidly progressive pneumonitis). In comparison with SAE negative group, patients presented more pulmonary hypertension (44.5vs34 mmHg), without reaching significant differences. No significant differences were found between muscle enzyme levels’ neither acute phase reactants. As complications, one patient presented a myocarditis with quickly rapidly progressive pneumonitis that required high doses of corticotherapy and another one alveolar hemorrhage, which was treated the same way. All patients required corticotherapy at doses of mg/kg, requiring two of them to be treated with DMARDs (methotrexate and dolquine) and one of them with immunoglobulins due to cutaneous involvement. Mortality rate was 40% due to rapidly progressive lung affectation and cardiorespiratory arrest. Conclusion Higher prevalence of SAE1/2 antibody in our cohort may be explained due to the use of amplified myositis antibodies kit that specifically includes this antibody. These patients typically present with cutaneous involvement and dysphagia but also lung affectation as a complication. Studies with larger samples should be performed in order to know the prognosis of this antibody specificity. Disclosure of interests None declared

Background: Two antisynthetase syndrome (ASSD) diagnosis criteria sets have been proposed; both consider mandatory the presence of anti-aminoacyl transfer RNA synthetase (ARS) autoantibodies. Solomon’s criteria consider major clinical criterions (interstitial lung disease (ILD) and fulfillment of Bohan and Peter criteria for DM/PM) and minor criterions (arthritis, Raynaud’s phenomenon (RP) and mechanic’s hands (MH)) (1). In contrast, Connors criteria evaluate the presence of at least one of the previously mentioned clinical features except myositis, and includes the presence of fever without other cause (2). Objectives: 1) to evaluate the performance of Solomon’s and Connors’ criteria in patients with clinical suspicion of aSSD or myositis and positive aRS. 2) to describe their clinical characteristics. Methods: We performed an observational retrospective study in two centers. All patients with clinical suspicion of aSSD or myositis, and positive aRS in the myositis immunoblot (Euroimmun assay) were included. Results: We analyzed 37 patients; 70.3% woman, with a mean age at the moment of the aRS detection of 51.4 (SD±14.0) years, median time from the first symptom to the aRS detection of 4.0 (SD±5.8) years, and time of evolution of 7.69 (SD±6.51) years. The frequency of aRS was: anti-Jo1 (n=17), anti-PL-12 (n=8), anti-PL-7 (n=4), anti-EJ (n=4), and anti-OJ (n=4). Diagnosis criteria fulfillment and clinical manifestations: 1) Patients that met Solomon and Connors’ criteria (n=17, 45.9%): - at disease onset: ILD (n=6, 35.9%), muscle weakness (MW) (n=5, 29.4%), and arthritis (n=4, 23.5%). - During disease development: ILD (n=14, 82.3%); arthritis (n=13, 76.5%); MW (n=10, 58.8%); mechanic hands (n=10, 58.8%); Raynaud phenomenon (n=8, 47.0%); and fever (n=3, 17.5%). 2) Patients that only met Connors’ criteria (n=17, 45.9%): - at disease onset: ILD (n=5, 29.4%), MW (n=3, 17.6%), or arthritis (n=5, 29.4%). - During disease development: arthritis (n=8, 47.0%); ILD (n=6, 35.3%); MW (n=6, 35.3%); Raynaud phenomenon (n=6, 35.3%); fever (n=5, 29.4%); and mechanic hands (n=1, 5.9%). Relative risk (RR) of the different clinical manifestation for Solomon’s criteria fulfillment: - MH RR=2.98 (95%CI 1.5-5.6; P=0.002), ILD RR=3.2 (95%CI 1.1-9.2; P=0.013); other manifestations does not presented significant RR. Conclusion: More than three quarter of all patients presented as first clinical manifestation one of those included in the aSSD classic triad (ILD, MW and arthritis). These manifestations showed increasing rates during the disease development, being more frequent in patients that met Solomon’s criteria than in those who only met Connor’s criteria; more than twice as high in for ILD, and almost twice for MW and arthritis. This suggests that patients who met Solomon’s criteria, at disease onset presented incomplete clinical forms, and their clinical progression favored the criteria fulfillment. On the other hand, we cannot predict if the patients that only met Connors’ criteria are going to fulfill Solomon’s criteria; nevertheless, our results suggests it. To conclude, our results suggest that: 1) Connors criteria might be considered for screening of aSSD; 2) Solomon’s criteria can be considered at the moment the gold standard for the aSSD diagnosis; 3) the presence of mechanic hands or ILD indicates a high probability of Solomon’s criteria fulfillment in patients with positive aRS. References [1] Solomon J, et al. Jornal brasileiro de pneumologia. 2011;37(1):100-9. [2] Connors GR, et al. Chest. 2010;138(6):1464-74. Disclosure of interests: Martin Greco: None declared, Maria Jesus Garcia de Yebenes: None declared, inmaculada alarcon: None declared, anahy Brandy-Garcia: None declared, Inigo Rua-Figueroa: None declared, Estibaliz Loza Grant/research support from: Roche, MSD, Pfizer, abbvie, BMS, UCB, actelion, Celgene, Grunenthal and Sanofi, Teresa Oton: None declared, Juan Carlos Quevedo-Abeledo: None declared, Carlos Rodriguez-Lozano: None declared, Loreto Carmona Grant/research support from: abbvie, actelion, astellas, BMS, Eisay, Gebro Pharma, Grunenthal, Leo Pharma, Lilly, MSD, Novartis, Pfizer, Roche, Sanofi-Aventis and UCB Pharma, Paid instructor for: Novartis

Background: Rheumatoid arthritis (RA) patients have a higher insulin resistance (IR) and some studies report that it is present at diagnosis. Systemic inflammation has been pointed out as the reason. However, data on polymyalgia rheumatica (PMR) are controversial. Objectives: To analyse IR in a group of untreated patients with a recent diagnosis of RA and PMR, and to establish predictive factors related with IR. Methods: Longitudinal observational study of patients older than 60 years, newly diagnosed with elderly-onset AR (ACR/EULAR 2010) and PMR (ACR/EULAR 2012). Comparison with healthy control group of the same age. Inclusion: consecutive and voluntary. Exclusion: patients with insulin-dependent diabetes. Follow-up time: 12 months. The clinical-epidemiological, anthropometric and analytical characteristics were collected. IR was calculated by HOMA-IR [(homeostatic model assessment of insulin resistance) = glucose (mg/dL) * insulin (mUI/L)/405] baseline and at 12 months. HOMA-IR>2.75 was considered IR (according to Spanish data). The statistical study was performed with Stata 15.1. Results: We recruited 42 patients with RA, 18 with PMR and 18 healthy controls. None of them had received treatment with corticosteroids or with DMARD at the baseline visit. Baseline characteristics are summarized in the table. At baseline visit, 66.7% of patients with elderly-onset RA had IR, compared with 33.3% of controls (p=0.024) and 27.8% of PMR (p=0.006). Therefore, the prevalence of IR in patients with RA doubled that of controls and patients with PMR before starting treatment. After 12 months of evolution and treatment, patients with RA and IR decreased from 66.7% to 51.2%, not being statistically significant (p=0.179). On the other hand, the percentage of patients with PMR and IR remained the same (27.8%). The differences in IR between RA and PMR at 12 months remained statistically significant (p=0.048). Given the results we decided to analyze the predictive factors related with IR only in the 42 patients with RA. In the univariate logistic regression analysis, the predictors of presenting IR were the BMI, the abdominal perimeter and the scapular girdle involvement. Specifically for BMI, for each of 2 kg/m2 the probability of having IR was 1.24 times higher (OR=1.24, IC95%: 1.12-1.37). Patients with scapular girdle involvement had a 6-fold increased risk of developing IR (OR = 6.0, 95% CI: 1.3-26.6). And for every 5 centimetres of abdominal perimeter the risk increased almost 4 times more (OR=3.9, 95% CI: 2.9-5.1). In the multivariate analysis, the only independent factor to increase the IR was the abdominal perimeter (aOR = 1.23, IC95%: 1.07-1.41). Conclusion: Patients with elderly-onset RA have a higher IR than the general population. High IR in RA is present at diagnosis. IR in AR is not exclusively mediated by systemic inflammation, since patients with PMR do not have this increase. The predictors of presenting IR in late onset AR were BMI, scapular girdle involvement and abdominal perimeter. Only the abdominal perimeter was shown as an independent factor. Disclosure of Interests: None declared

Background: Adult-onset Still’s disease (AOSD) is an uncommon disease with an unpredictable clinical course and variable prognosis. Sometimes, it requires biologic treatment in early phases. A prognosis score has been described, which has never been applied in a Spanish case series. Objectives: To apply the prognosis score described by Pouchot et al (Systemic Score System (SSS)) on a 64 cases series diagnosed with AOSD in Spanish population and to determine if SSS high values registered at the onset of the pathology are related to AOSD clinical patterns (monocyclic, polycyclic and chronic course), requirement of biologic treatment along the disease’s course and development of AOSD clinical complications. To establish the relationship between its value and the AOSD-related mortality. Methods: Retrospective study realized in two University Hospitals. Clinical, laboratory, AOSD-related complications data, administered biologic treatments and number of deaths (AOSD related or not) were recorded. Each patient was characterized for the presence of AOSD-related complications such as macrophage activation syndrome (MAS), myocarditis, lung involvement (pulmonary hypertension, interstitial infiltrate), renal involvement (tubulointerstitial nephritis, acute renal failure), secondary amyloidosis and AOSD-related death. SSS was applied at the onset of the disease development, assigning a point to each of the next 12 variables: fever, exanthema, pleuritis, pneumonitis, pericarditis, alteration of liver tests or hepatomegaly, splenomegaly, lymphadenopathy, odynophagia, leukocytosis >15,000/mm3, myalgia and abdominal pain. A ≥7 score has been validated on other populations as the one which identifies the patients with high risk of complications. The relationship between SSS value and the next parameters was determined: clinical course, complications, biologic treatments administered and AOSD-related mortality. Results: Data from 64 patients was analyzed (40,6% men, mean age 37 years). SSS values of Conclusion: The prognosis score described by Pouchot et al could be useful to identify those patients with high risk of developing clinical complications and those who will need biologic treatment along the course of their disease. It is necessary a higher number of patients to determine if the score could be useful to estimate the death risk related to AOSD complications. References: [1] Ruscitti, et al. Adult-onset Still’s disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers. BMC Medicine (2016)14:194 Disclosure of Interests: Ivette Casafont-Sole: None declared, Susana Holgado: None declared, J. Narvaez Consultant for: Bristol-Myers Squibb, Maribel Mora: None declared, Josep Roca: None declared, Anahy Brandy-Garcia: None declared, Lourdes Mateo: None declared, Melania Martinez-Morillo: None declared, Laia Gifre: None declared, Maria Aparicio Espinar: None declared, Agueda Prior-Espanol: None declared, Anne Riveros: None declared, Clara Sanguesa: None declared, Jordi Camins-Fabregas: None declared, Annika Nack: None declared, Joan Miquel Nolla: None declared, Alejandro Olive: None declared

Background Polyarthritis at the elderly people usually has a similar onset with an acute inflammatory character and scapular girdle involvement. Differentiating elderly onset rheumatoid arthritis (EORA) and polymyalgia rheumatica (PMR) can be a diagnostic challenge. Objectives To analyse the clinical and analytical differences between EORA and PMR. Methods Longitudinal observational study of patients older than 60 years newly diagnosed with EORA (ACR/EULAR 2010) and PMR (ACR/EULAR 2012). Inclusion: consecutive and voluntary. Follow-up time: 12 months. A single rheumatologist made all follow-up visits. The clinical-epidemiological and analytical characteristics were collected. The statistical study was performed with Stata 15.1. Results 45 EORA were recruited (53% women; mean age 74.8 ± 7.5) and 20 PRM (85% women; mean age 76.6±5.0). 75% of EORA had scapular girdle involvement, but only 44% of the pelvic girdle. All had peripheral arthritis, and the small joints of the hands were involved in 93.3%, with edema in 46.7%. Forty percent of EORA patients were seropositive (RF> 20 IU/mL and/or ACPA> 20 U/mL): 33% RF positive (132.8 ± 126 IU/mL), 28.9% for ACPA (2 cases [15.4%] from 100 to 250 U/mL and 11 cases [84.6%] above 250 U/mL) and in 10 patients [22.2%] double positive). All patients with PMR patients had shoulder girdle involvement and 90% of the pelvic girdle. None of them had peripheral arthritis. RF was positive in one patient (73 IU/mL) and ACPA in 2 patients (titers between 20-40 U/mL). No patient was double positive. Table 1 and 2. After 12 months of follow-up, there was no difference between the dose of glucocorticoids received in patients with EORA and PMR (p = 0.684). There were also no differences in glucocorticoids adverse effects according to the diagnosis (p = 0.734). Regarding the use of immunosuppressors, this was higher in patients with EORA (91% EORA and 20% PMR), according to the usual clinical practice guidelines. The percentage of remission in PMR at 12 months was 95%. However, using DAS 28-VSG, only 40.9% of patients with EORA were in remission at 12 months (p=0.003). Conclusion The female predominance was higher in PMR than in EORA. The scapular girdle involvement, but especially the pelvic girdle, was more frequent in PMR. In contrast, involvement of peripheral joints and edema were more frequent in EORA. RF and ACPA were more frequent in EORA. There were no other analytical differences that would help their differential diagnosis. The mean and accumulated doses of glucocorticoids during the first 12 months were similar, as well as the percentage of side effects. Immunosuppressors are more frequently used in EORA than in PMR. Remission is achieved more commonly in PMR than in EORA. References [1] Olivieri I, Pipitone N, D’ Angelo S, Padula A, Salvarani C. Late-onset rheumatoid arthritis and late-onset spondyloarthritis. Clin Exp Rheumatol 2009;27(4 Suppl 55):S139-145. [2] Pease CT, Haugeberg G, Montague B, Hensor EMA, Bhakta BB, Thomson W, et al. Polymyalgia rheumatica can be distinguished from late onset rheumatoid arthritis at baseline: results of a 5-yr prospective study. Rheumatology 2009;48(2):123-7. Disclosure of Interests None declared

Background Denosumab (Dmab), an anti-receptor activator of nuclear factor kappa-B ligand (RANKL) monoclonal antibody, has been shown to increase bone mineral density (BMD) at lumbar spine and proximal femur up to 21.6% and 9.1% respectively at 10 years of treatment. Additionally, Dmab has shown a marked decrease of vertebral, nonvertebral and femoral fractures during treatment. Nowadays, the existence of inadequate response to Dmab treatment remains unknown. Objectives: to describe the clinical, analytical and densitometric characteristics of patients with an inadequate response (IR) to Dmab treatment. IR was defined as the presence of a new fragility fracture during Dmab treatment or a significant decrease in BMD (≥5% at lumbar spine or ≥4% at proximal femur) within at least 12 months of therapy. Methods: retrospective study including patients with osteoporosis with an IR to Dmab. Therapeutic compliance was checked by clinical anamnesis and the electronic prescription. Risk factors for osteoporosis, history of fragility fractures, previous anti-osteoporotic treatment, densitometric and analytical data were collected before and at the moment when IR was diagnosed. Results Fourteen patients were included (12 women and 2 men) with mean age of 75 ± 9 years. The causes of osteoporosis were: postmenopausal (n=8, 57.14%), induced by glucocorticoids (n=3, 21.43%), alcoholic (n = 1, 7.14%) and multifactorial (n=2; 14.28%). Nine patients (64.28%) had been previously treated with oral or intravenous bisphosphonates for a mean of 5.8 ± 2.76 years. Nine patients (64.28%) had previous vertebral fractures (median 2, range 1-8), 2 of them had also presented a femoral fracture. During Dmab treatment, 7 patients (50%) presented a decrease in BMD (mean loss: proximal femur -3.5%, p=0.09; lumbar spine - 5.8%, p=0.046;) and 7 had incidental fractures: 5 vertebral (median 1, range 1-4), 1 humerus and 1 femur. The duration of treatment with Dmab was 3.82 ± 1.85 years in patients who sustained fragility fractures and 2.39 ± 1.4 years in patients with a BMD decrease. A multiple myeloma was diagnosed in a patient with vertebral fractures during Dmab treatment. After The identification of Dmab RI most patients mantained same treatment. Of the patients with incidental fragility fractures, 2 started combined treatment with teriparatide and Dmab, 1 changed to teriparatide and 2 maintained the same treatment. Of the 7 patients with BMD only 1 changed to zoledronic acid. Conclusion Most patients with IR to Dmab treatment had previous fragility fractures and had been previously treated with bisphosphonates for a mean duration of 5 years. The patients with a significant decrease in BMD had lesser duration of Dmab treatment than those who sustained fractures during Dmab treatment. Only one patient had a clinical cause for the IR development. Disclosure of Interests None declared

Background: The antisynthetase syndromes (ASSD) are characterized by the presence of anti-aminoacyl transfer RNA synthetase (ARS) autoantibodies and clinical features including myositis, arthritis, interstitial lung disease (ILD), Raynaud’s phenomenon (RP), mechanic hands (MH), and fever. Two aSSD diagnosis criteria have been developed; those proposed by Connors, and the stricter criteria proposed by Solomon (1, 2). Additionally, other symptoms of connective tissue diseases (CTD) can be present. Objectives: To describe a series of patients with positive aRS and analyze: 1) the associated non-ARS antibodies, 2) the clinical manifestations that are not included in the different aSSD diagnosis criteria; and 3) the initial diagnosis attributed by the Rheumatologist in the clinical records. Methods: We performed an observational retrospective study in two hospitals. All patients with clinical suspicion of aSSD or myopathy and positive aRS in the myositis immunoblot (Euroimmun assay) were included. Results: We analyzed 37 patients; 70.3% woman, with a mean age at the moment of the first symptom of 50.5 (SD±14.0) years, and 51.4 (SD±14.0) years at the moment of the aRS detection. The frequency of aRS was: anti-Jo1 (n=17), anti-PL12 (n=8), anti-PL7 (n=4), anti-EJ (n=4), and anti-OJ (n=4). The rate of the aSSD clinical manifestations was: arthritis (56.7%), ILD (54.1%), muscle weakness (48.6%), RP (37.8%), MH (29.7%) and fever (21.6%). Thirty-four patients (91.9%) met Connors’ criteria and 17 of them (45.9%) also met Solomon’s criteria. Non-antisynthetase characteristics: - associated non-ARS antibodies: anti-Ro52 (n=17, 46%); anti-PM/Scl75 (n=6, 16.2%); anti-PM/Scl100 (n=5, 13.5%); anticentromere (n=3, 8.1%); rheumatoid factor (n=2, 5.4%); anti-CCP (n=2, 5.4%); and anti-DNAds (n=2, 5.4%). Other antibodies detected only once: anti-SRP, lupic-anticoagulant, anti-Ku, anti-Mi2, anticardiolipin, Nor90, Th/To. - Most frequent clinical manifestations not included in the aSSD diagnosis criteria: dysphagia (n=10, 27%); dermatomyositis rash (n=9, 24.3%); and SICCA sympthoms (n=7, 18.9%). - First diagnosis attributed by the clinicians in the medical records: aSSD (n=19, 51.35%), dermatomyositis/polymyositis (DM/PM) (n= 3), overlap syndrome (n= 3), systemic lupus erythemoatous (SLE) (n= 3), primary Sjogren’s syndrome (SS) (n=2); rheumatoid arthritis (RA) (n=2); undiferenciated-CTD (UCTD) (n=2); and Systemic Sclerosis (SSc) (n=1; 2.7%). Overlaps included 2 DM/PM-SSc and 1 DM/PM-SS, and both UCTD was described with SSc pattern. Thus, of the 18 cases not diagnosed as aSSD, 6 (33.3%) presented DM/PM diagnosis pattern and 6 (33.3%) SSc pattern. - additionally, when we applied the aSSD diagnosis criteria, 7 of the 18 cases (38.8%) not diagnosed as aSSD by the Rheumatologist fulfilled Solomon’s criteria. Conclusion: In our series, the most frequent non-ASSD profiles associated to aRS positivity was DM/PM, SSc and Sjogren’s syndrome. This, considering the most frequently associated non-ARS, the most frequent non-ASSD clinical manifestations and that the most frequent non-ASSD diagnosis diagnoses or profiles described by the Rheumatologist. Our results suggest that aRS can be present in patients with other CTD without presenting aSSD, and additionally that aSSD can be present in overlaps with other CTD. References [1] Solomon J, et al. Jornal brasileiro de pneumologia. 2011;37(1):100-9. [2] Connors GR, et al. Chest. 2010;138(6):1464-74. Disclosure of interests: Martin Greco: None declared, Maria Jesus Garcia de Yebenes: None declared, inmaculada alarcon: None declared, anahy Brandy-Garcia: None declared, Inigo Rua-Figueroa: None declared, Estibaliz Loza Grant/research support from: Roche, MSD, Pfizer, abbvie, BMS, UCB, actelion, Celgene, Grunenthal and Sanofi, Teresa Oton: None declared, R. Lopez-Sanchez: None declared, Laur Caceres Martin: None declared, Loreto Carmona Grant/research support from: abbvie, actelion, astellas, BMS, Eisay, Gebro Pharma, Grunenthal, Leo Pharma, Lilly, MSD, Novartis, Pfizer, Roche, Sanofi-Aventis and UCB Pharma, Paid instructor for: Novartis

Background: Behcet’s disease (BD) is a variable vessel vasculitis and typically presents with mucocutaneous involvement. However, any organ can be affected, being the neurological affectation (neurobehcet, NB) one of the most serious manifestations. Objectives: Our aim was to assess the efficacy and safety of biological therapy as treatment of NB. Methods: We set up a multicenter observational study of 29 patients with NB on treatment with biological therapy (BT). NB diagnosis was made by neuroimaging, CSF analysis and/or suggestive clinical signs of central and/or peripheral nervous system involvement, excluding infectious causes or more prevalent pathology. Results are expressed as mean±SD or as median and interquartile range (IQR) as appropriate. Results: 29 patients (15♂/14♀) with an average age of 39.6 ± 10.5 years. HLA-B51 was positive in 48.3% of the patients. Table shows the non-neurological manifestations. Regarding the neurological manifestations, 23 patients (79.3%) had parenchymal involvement (hemiparesis (n=6), brainstem involvement (n=1), encephalopathy (n = 4), optic neuropathy (n=3), dysphasia (n=1), polyneuropathy (n=6), cognitive impairment (n=4), and non-steroidal psychosis (n=1), while the remaining 6 patients (20.7%) presented aseptic meningitis as a non-parenchymal affectation (Table). Prior to BT, patients had received the following treatment: oral prednisone (n=27), methylprednisolone bolus (n=9), CsA (n=8), AZA (n=16), MTX (n=14) and mycophenolate (n=2). After a median of 31 [10-60] months since the beginning of the neurological symptoms, the following BT was initiated: infliximab (IFX)(n=17), adalimumab (ADA)(n=7), tocilizumab (TCZ) (n=2), golimumab (GOL) (n=2) and Etanercept (ETN) (n=1). A first switch to ADA was necessary in 8 patients with IFX due to primary failure. In addition, 2 of them needed a second switch to TCZ, getting a partial response. The BT was discontinued in 5 patients, 2 of them for obtaining clinical remission and the remaining 3 for inefficacy. After a median follow-up of 5.4±4.6 years, complete response was obtained in 15 patients, partial response in 11 and no response in the remaining 3. We observed an anaphylactic reaction and psoriasis induced by IFX, without other serious adverse events (Table). Conclusion: BT, especially anti-TNF, seems effective and safe for treatment in NB. Disclosure of Interests: Jose Luis Martin-Varillas: None declared, Inigo Gonzalez-Mazon: None declared, Belen Atienza-Mateo: None declared, Monica Calderon-Goercke: None declared, D. Prieto-Pena: None declared, Lara Sanchez Bilbao: None declared, Vanesa Calvo-Rio: None declared, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Esther Vicente: None declared, Olga Maiz: None declared, Clara Moriano: None declared, Elvira Diez Alvarez: None declared, Jose Luis Andreu Sanchez: None declared, Concepcion Delgado Beltran: None declared, Marta Loredo Martinez: None declared, J. Narvaez Consultant for: Bristol-Myers Squibb, Angel Ramos Calvo: None declared, Francisca Sivera: None declared, Enrique Raya: None declared, Norberto Ortego: None declared, Jose Luis Callejas-Rubio: None declared, Anahy Brandy-Garcia: None declared, Alejandro Olive: None declared, Sabela Fernandez: None declared, Ricardo Gomez de la Torre: None declared, Ignacio Torre-Salaberri: None declared, Julio Sanchez: None declared, ANA URRUTICOECHEA-ARANA: None declared, Eva Salgado-Perez: None declared, Rafael Melero: None declared, Olga Martinez Gonzalez: None declared, Susana Romero-Yuste: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., 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

Background: The antisynthetase syndromes (ASSD) are characterized by the presence of anti-aminoacyl transfer RNA synthetase (ARS) autoantibodies; which difficult the binding of amino acids to the transfer RNA during the protein synthesis. ARS can be detected by indirect immunofluorescence (IIF), and can be identified by immunoblot assay and ELISA (Enzyme-Linked ImmunoSorbent Assay) and immunoblotting. The main clinical features of the ASSD are myositis, arthritis, interstitial lung disease, Raynaud’s phenomenon, mechanic hands, and fever. Two ASSD diagnosis criteria have been developed; those proposed by Connors, and the stricter criteria proposed by Solomon (1, 2). Objectives: To evaluate the performance of the ARS and their IIF patterns in the ASSD diagnosis. Methods: We performed an observational retrospective study in one center during the period 06/2008-06/2018. We searched all the myositis immunoblots (Euroimmun assay) requested by the Rheumatologists under suspicion of ASSD or myositis. We assessed: 1) the rate of cases with positive ARS; 2) the rate of cases with Connor’s or Solomon’s diagnosis criteria fulfillment; and 3) their relation with the IIF patterns (Hep-2 cells; ≥1/80) evaluated by an expert in autoimmune tests. Results: A total of 140 myositis immunoblots were searched. Twenty-seven cases (19.3%) presented positive ARS: anti-Jo1 (n=13), anti-PL-12 (n=7), anti-PL-7 (n=1), anti-EJ (n=2), and anti-OJ (n=4). Twenty-five of these (17.9%) fulfilled Connors’ criteria, and 15 (10.7%) additionally met Solomon’s criteria. Thus, the fulfillment of Connor’s and Solomon’s criteria in patients with a positive ARS was of 92.6% and 55.5%, respectively. All cases (100%) with positive ARS presented positive immunofluorescence: 19 (70.4%) showed a cytoplasmic pattern (10 of them with an associated nuclear pattern) and 8 cases (29.6%) presented only a nuclear pattern. On the other hand, 99 of the 113 cases (87.6%) with negative ARS presented positive IIF: 29 (25.7%) showed a cytoplasmic pattern (21 of them with an associated nuclear pattern) and 42 cases (37.2%) presented only a nuclear pattern. Correlating the ARS positivity, IIF pattern and the diagnosis criteria fulfillment: - 13 of 15 cases (86.6%) with positive ARS and Solomon’s criteria fulfillment presented a cytoplasmic pattern; and 2 of 15 cases (13.3%) presented only a nuclear pattern. - 13 of 19 cases (68.4%) with positive ARS and cytoplasmic pattern fulfilled Solomon’s criteria; and 6 only fulfilled those from Connors’. Conclusion: One-fifth of the immunoblots requested by Rheumatologists presented positive ARS; almost all these cases fulfilled Connor’s criteria, and more than a half fulfilled the stricter Solomon’s criteria. All patients with positive ARS, and a high rate of those without ARS, presented positive IIF. The presence of a cytoplasmic pattern was considerably higher in patients with ARS positivity and in those that met Solomon’s criteria. Thus, our results suggests that in patients evaluated by a Rheumatologist, with clinical suspicion of ASSD or myositis and with ARS positivity, the probability of fulfilling Solomon’s criteria is higher when the IIF presents a cytoplasmic pattern than when only a nuclear pattern is observed. Nevertheless, presenting only a nuclear pattern does not exclude the detection of ARS in the myositis immunoblot and the fulfillment of Solomon’s criteria. References [1] Solomon J, et al. Jornal brasileiro de pneumologia. 2011;37(1):100-9. [2] Connors GR, et al. Chest. 2010;138(6):1464-74. Disclosure of Interests: Martin Greco: None declared, Maria Jesus Garcia de Yebenes: None declared, Inmaculada Alarcon: None declared, Anahy Brandy-Garcia: None declared, Inigo Rua-Figueroa: None declared, Estibaliz Loza Grant/research support from: Roche, MSD, Pfizer, Abbvie, BMS, UCB, Actelion, Celgene, Grunenthal and Sanofi, Teresa Oton: None declared, Francisco Rubino: None declared, Juan Manuel Diaz: None declared, Felix Francisco: None declared, Soledad Ojeda: None declared, Loreto Carmona Grant/research support from: Abbvie, Actelion, Astellas, BMS, Eisay, Gebro Pharma, Grunenthal, Leo Pharma, Lilly, MSD, Novartis, Pfizer, Roche, Sanofi-Aventis and UCB Pharma, Paid instructor for: Novartis

Background Clinical manifestations of systemic lupus erythematosus (SLE) and infections sometimes are difficult to distinguish. In clinical practice low complement and anti(ds)DNA levels are used to assess lupus activity but its determination usually requires some days. Leukocyte count, CRP and ESR cannot discriminate SLE from infectious processes. Calprotectin could be a good biomarker to assess lupus activity since it is more specific than CRP and ESR and faster to analyse than anti(ds)DNA. Objectives Our aim is to determine serum calprotectin levels in patients with SLE, and its correlation with analytical and clinical manifestations, especially with disease activity. Methods A total of 148 patients were included. All patients included fulfilled the SLE criteria (SLICC 2012). A quantitative ELISA analysis was performed to assess levels of serum calprotectin (CALPRO AS, Norway). Other biomarkers of lupus disease activity were also assessed (levels of anti(ds)DNA, hypocomplementemia, ESR and CRP). Clinical variables and activity/damage index (SLEDAI/SLICC) were also evaluated. The study was approved by the Clinical Research Ethics Committee of the hospital and all patients signed an informed consent. The results were compared with a healthy control group of similar age and sex (n=20). Results 134 patients (92%) were women with a mean age of 46±12 years and an average SLE evolution of 12±7 years. Mean SLEDAI was 2±2 (105 inactive [ 13]). Mean SLICC was 0.31±0.70. No significant differences were observed in serum calprotectin levels between patients with SLE and healthy controls (2.93±2.35 vs 2.17±1.49 µg/mL, p=0.160). Calprotectin was positively correlated with CRP (r=0.447, p= 100UI/mL) had higher calprotectin compared to patients with lower anti(ds)DNA (3.20±2.63 vs 2.42±1.57 µg/mL; p=0.027), however this pattern was not observed with hypocomplementemia. Contrary to what we expected, we did not observe significant differences on calprotectin levels depending on SLEDAI index classification (cutoff at 4 and 12). Moreover, no differences were observed on calprotectin levels between those patients with/without clinical manifestations such as serositis, arthritis or glomeruloneprhitis. Patients with antiphospholipid antibodies had higher calprotectin levels (3.75±2.04 vs 2.77±2.38 µg/mL;p=0.045). Conclusion Serum calprotectin levels were positively correlated with CRP levels and leukocyte count. Patients with higher anti(ds)DNA levels had higher calprotectin levels, however we did not observe significant differences depending on SLEDAI index or the presence of arthritis, serositis neither glomerulonephritis. Even that calprotectin determination is faster than anti(ds)DNA levels and could be helpful in assessing inflammatory activity. There is an interesting relation between antiphospholipid antibodies and calprotectin. This study should be continued in a larger sample of active SLE patients to assess its utility in clinical practice as a discriminating biomarker for flares and even infection Disclosure of Interests None declared

Background: Dermatomyositis (DM) is an idiopathic inflammatory myopathy. The recent years has increased its knowledge thanks to best characterization of myositis-specific antibodies that correlate with different clinical phenotypes.with different clinical phenotypes. Objectives: To describe the clinical and analytical features of a series of dermatomyositis: clinical debut, clinical manifestations as well as the treatments received and the evolution of the disease. Methods: Patients diagnosed of dermatomyositis in a tertiary hospital between the years 1978-2018 according to the criteria of Bohan and Peter (1975) and according to Dalakas’ classification criteria (2015). Clinical, analytical and immunological profile data were collected, as well as treatments received and the evolution of the disease. Results: A total of 59 inflammatory myopathies diagnosed between the years 1985 and 2018 were included. 46 were dermatomyositis (78%), 9 polymyositis (15%) and 4 necrotizing myositis (7%). 69% were women and 22% were smokers. Clinic started at 54 ± 17 years. The initial manifestation was pulmonary in 26.7% followed by cutaneous manifestations (24.4%) and the muscular (22.2%), while a 17.8% started skin and muscular manifestations at the same time. 45.5% behaved like a myopathic DM while a 28% as amyopathic DM and antisynthetase syndrome respectively. 38% presented interstitial involvement being the most common non-specific interstitial pneumonitis (76%)followed by usual interstitial pneumonitis (17%). In these patients, DLCO was decreased (mean of 58.9% and 13.2) as well as the FVC (average of 58%, 2.5L). 73.3% presented cutaneous involvement being the most common manifestations the Gottron papules (37.8%) and the heliotrope rash (35.6%), and up to 27% had cuticular affectacion. 64% had muscle involvement, afecting proximal and symmetrical. Neck flexors were affected in a 38% of patients while 20% had dysphagia.Only 3 patients presented dysphonia (7%) and 2 myocarditis Analytical data Conclusion: 80% received corticotherapy at a dose of mg/kg/day and 20% required high doses of metilprednisolone due to muscular involvement or pulmonary. 18% immunoglobulins and 11% cyclophosphamide. As maintenance 80% received disease modifiying antirheumatic drug in addition to corticosteroid therapy in descending doses (azathioprine 22%, dolquine 15%, tacrolimus 13.3%, rituximab 11%) due to muscle (29.9%), cutaneous (24.4%) and pulmonary involvement (22.2%). As complications, 2 cases of the syndrome were registered hemophagocytic Virtually all patients they presented pulmonary hypertension (mean 34 ± 12 mmHg). 5 patients (11.1%) were diagnosed with neoplasia, two of them after the diagnosis of DM. The mortality was 24%. 3 patients died due to a rapidly progressive pneumonitis, another 2 due to alveolar hemorrhage and three of them due to complication of the neoplastic disease. Dermatomyositis occurs in a variable way, with predominance of pulmonary, skin and muscle manifestations. They require corticotherapy and immunosuppressive treatment for maintenance, even so, mortality is high. Disclosure of interests: None declared

Background Extra-articular manifestations (EAM) in rheumatoid arthritis (RA) are more prevalent in patients with seropositive, nodular and long term disease. Around 40% of patients with RA can develop EAM, although the incidence has decreased with the “treat to target” strategy. Objectives To describe EAM in a cohort of patients with rheumatoid arthritis. Methods Retrospective descriptive study of patients diagnosed with RA between 2000 and 2018 who are currently under active biological treatment. Cardiovascular risk factors, baseline characteristics of RA and EAM during their evolution are collected. Statistical analysis is performed with SPSS 24. Results We recruited 108 patients, 81.5% female, with mean age at onset of RA of 50 ±15 years (range 17-82). RF was positive in 84.3% and ACPA in 81.3%. Erosions were observed on hands or feet at diagnosis in 35.2%. Mean follow up period: 12±8 years. The most common initial treatment was methotrexate (82.4%) and 44.4% received other DMARDs: leflunomide (32.3%), hydroxychloroquine (16.2%) and others (9.1%). DAS28VSG at diagnosis was 4.15±1.18 (1.89-6.02) and at the introduction of the first biological drug it was 5.02 ±1.16 (mean years since diagnosis 6±5). The first biological drug was etarnecept (40.7%), followed by adalimumab (17.6%), abatacept (15.7%), rituximab (7.4%), tocilizumab (7.4%) and infliximab (4.6%). 57.4% discontinued the first biologic: secondary failure (58.1%) or adverse effects (20.6%). 65.7% (71 patients) developed some EAM during disease evolution and 14.8% appeared during the first year. During this first year, secondary Sjogren (37.5%) was the most frequent, followed by rheumatoid nodules (18.7%), EPID (18.7%) and osteoporosis (18.7%). Two or more EAMs were present in 33.3% of patients. Fifty-four patients (56.3%) did not present any EAM at the beginning of the biological treatment and 29.6% (21 patients) developed EAM afterwards, with an average of 1±3 years. Pulmonary involvement was the first EAM to appear after the onset of biological treatment, followed by anemia (both in the first year). Summarizing the data of all patients over time, the most frequent EAMs were: osteoporosis (28.7%, mean appearance: 5 years from diagnosis); secondary Sjogren syndrome (27.8%, mean 6 years); normochromic normochromic anemia (21.3%, mean 8 years) and pulmonary involvement (17.6%, mean 4 years), presented as EPID (63.3%), NINE (25%), NIU (8.3%) and pulmonary nodules (8.3%). Other EAM observed were: cutaneous involvement in the form of rheumatoid nodules (15.7%, mean 5 years); ophthalmological involvement (6.5%, mean 18 years) (PUK 2.8%, uveitis 1.9%, scleritis 0.9%, episcleritis 0.9%); amyloidosis (3.7%, mean 16 years) and vasculitis (2.4%, mean 14 years). Conclusion EAM have a high prevalence in RA patients receiving biologic treatment (76.6%). A third part of patients had 2 or more EAMs. 14.8% develop the first EAM during the first year of evolution of RA and 30% in the first 5 years. The most frequent are osteoporosis and Sjogren’s syndrome, according to what is described in the literature. Pulmonary involvement is the most rapid during the evolution of RA with an average of 4 years. References [1] Marcucci E, Bartoloni E, Alunno A, Leone MC, Cafaro G, Luccioli F, et al.Extra-articular rheumatoid arthritis. Reumatismo. 2018Dec20;70(4):212-224 Disclosure of Interests None declared

Background:Secukinumab is a human monoclonal antibody directed against IL-17A, approved for the treatment of psoriatic arthritis (PsA) and ankylosing spondylitis (AS). The safety profile of secukinumab was favourable in clinical studies, but there is still scarce evidence in clinical practice. Similarly, we currently have less data regarding the real survival of secukinumab compared to other biological therapies such as anti-TNF.Objectives:To analyze the retention rate and safety of secukinumab as well as the causes and factors associated with its survival in patients with ankylosing spondylitis and psoriatic arthritis in real clinical practice.Methods:we conducted a retrospective longitudinal observational multicenter study of all patients with PsA and Spondyloarthritis (SpA) who had received at least one dose of secukinumab. Adverse events and drug retention were considered the main variables. In addition, we collected variables predicting drug retention. We estimated the total adverse event rate, by severity and type of event, and drug retention (mean duration and retention at 6 months, 1 year and 2 years), all with 95% confidence intervals (95% CI). Survival was analyzed using Kaplan-Meier curves and predictive factors using Cox regression, with the Hazard Ratio (HR) as a measure of the association.Results:154 patients were included, 59 with PsA (38%) and 95 with SpA (62%), with a mean age of disease onset of 49 years (SD ± 11), being 55% men. The mean disease duration was 6.5 years (ICR 2-8). The median number of previous biologics was 2 (SD ± 1). Secukinumab was the first line of treatment in 13 patients (8%), the second line in 46 (30%), the third line in 54 (35%) and subsequent lines in 41 (27%). The median survival of secukinumab was 23 months (ICR 5-32), with a 1-year retention rate of 66% and a 2-year retention rate of 43%. The most frequent cause of discontinuation was inefficacy (59%) and the second one was adverse events (AE) (36%). Most patients who discontinued due to AEs (71%) did so during the first 6 months of treatment. Only 2 major cardiovascular events were collected, and 2 cases of Crohn’s disease occurred during the exposure. The factors identified as predictors of survival for secukinumab were: duration of disease (HR 0.96, 95% CI 0.93-0.99 p=0.012), number of previous biologics (HR 1.18, 95% CI 1.04-1.34 p=0.011), male gender (HR 0.63, 95% CI 0.43-0.90 p=0.013), obesity (HR 0.31, 95% CI 0.18-0.54 p=0.000) and depression (HR 2.54, 95% CI 1.64-3.94 p=0.000).Conclusion:In this study of real clinical practice, secukinumab showed a 66% retention rate at one year in a population mostly refractory to biological therapy. The main cause of discontinuation was lack of efficacy. The AAs that led to drug discontinuation occurred mainly in the first 6 months of treatmentAcknowledgments:Raquel Linge, Agnes Díaz and Juan CalatayudDisclosure of Interests:Ignacio Villa-Blanco Consultant of: UCB, Speakers bureau: Novartis, MSD, Lilly, Sara Alonso Castro: None declared, Sabela Fernández: None declared, José Luis Martín-Varillas Grant/research support from: AbbVie, Pfizer, Janssen and Celgene, Speakers bureau: Pfizer and Lilly, Lilian Consuelo Charca Benavente: None declared, Marina Pino Martínez: None declared, Leyre Riancho-Zarrabeitia Grant/research support from: Yes, Speakers bureau: Yes, Isla Morante Bolado: None declared, Montserrat Santos Gómez: None declared, Anahy Brandy-Garcia: None declared, Elena Aurrecoechea: None declared, Loreto Carmona Grant/research support from: Novartis Farmaceutica, SA, Pfizer, S.L.U., Merck Sharp & Dohme España, S.A., Roche Farma, S.A, Sanofi Aventis, AbbVie Spain, S.L.U., and Laboratorios Gebro Pharma, SA (All trhough institution), Rubén Queiró Silva: None declared