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BackgroundTocilizumab (TCZ) has shown to be effective for large vessel vasculitis including giant cell arteritis (GCA) and Takayasu arteritis (TAK) (1-5). However, LVV-GCA and TAK show different demographic and clinical features that may influence on TCZ therapeutic response.ObjectivesTo compare the effectiveness of TCZ in patients with LVV-GCA and patients with TAK.MethodsObservational multicenter study of patients with LVV-GCA and TAK who received TCZ. Outcome variables were: a) proportion of patients who achieved complete clinical improvement along with normalization of laboratory markers (CRP ≤0.5mg/dL and/or ESR ≤ 20 mm/1st hour) at 12 months b) complete improvement in imaging techniques. A comparative study between patients with LVV-GCA and TAK was performed.ResultsWe evaluated 70 LVV-GCA and 57 TAK patients who received TCZ. Main clinical and demographic characteristic are described in Table 1. Patients with TAK were younger, had longer disease duration, had received more commonly previous biologic therapy and were receiving higher doses of prednisone at baseline. TCZ intravenous administration was more common in TAK patients (80.7% vs 48.6%; pTable 1.LVV-GCA (n=70)TAK (n=57)pGeneral featuresAge (years), mean ± SD67.2 ± 10.540.5 ± 16.3< 0.01Sex (female), n (%)51 (72.9)49 (86)0.07Disease evolution before TCZ onset (months), median [IQR]5 [2-15]12 [3-37]Baseline laboratory parametersESR (mm/1st hour), median [IQR]32 [12.5-54.7]31 [10-52]0.82CRP (mg/dL), median [IQR]1.4 [0.5-2.4]1.4 [0.5-3.5]0.41Baseline prednisone dose (mg/day), median [IQR]15 [10-20]30 [15-50]< 0.01Previous therapyConventional DMARDs, n(%)45 (64.3)44(77.2)0.51Biologic therapy, n (%)0(0)12 (21.1)TCZ therapyIntravenous, n (%)34 (48.6)46 (80.7)< 0.01Combined with MTX, n(%)24 (34.3)24 (42.1)0.37Follow-up time after TCZ onset, median [IQR]20 [10-36]18 [7-41]0.73Complete clinical improvement and ESR/CRP normalization at 12 months, n/N (%)35/47 (74.4)30/39 (76.9)0.79Complete improvement in imaging techniques, n/N(%)7/37 (18.9)8/38 (21.1)0.85CRP: C-reactive protein; DMARDs: Disease-modifying anti-rheumatic drugs ESR: erythrocyte sedimentation rate; GCA: giant cell arteritis; IQR: interquartile range; LVV: large vessel; MTX: methotrexate; n: Number of patients; N: total number of patients: TCZ: tocilizumab; TAK:takayasuFigure 1.ConclusionThe effectiveness of TCZ was similar in patients with LVV-GCA and TAK, despite a more refractory disease in TAK patients. A discordance between clinical and imaging activity improvement was observed in both LVV-GCA and TAK, as reported in previous studies (3).References[1]Calderón-Goercke M, et al. Semin Arthritis Rheum 2019; 49:126-35. https://doi.org/10.1016/j.semarthrit.2019.01.003[2]Prieto-Peña D et al. Ther Adv Musculoskelet Dis. 2021;13:175. PMID: 34211589.[3]Prieto Peña D et al. Clin Exp Rheumatol. 2021;39 Suppl 129:69-75. PMID: 33253103.[4]González-Gay MA, et al. Expert Opin Biol Ther. 2019;19:65-72. doi: 10.1080/14712598.2019.1556256.[5]Prieto-Peña D, et al. Semin Arthritis Rheum. 2019;48(4):720-727. doi: 10.1016/j.semarthrit.2018.05.007Disclosure of InterestsNone declared

BackgroundThe ultrasonographic (US) Halo Score provide a quantitative measure of the extent of vascular inflammation in patients with giant cell arteritis (GCA). High Halo Scores correlate with systemic markers of inflammation, rate of ocular ischaemia and may help to firmly diagnose GCA with high specificity. However, an increase in the intima media thickness (IMT) in patients with elevated cardiovascular risk (CVR) may lead to false-positive US findings.ObjectivesOur aim is to evaluate the impact of CVR on the diagnostic accuracy of the US Halo Score in patients with suspected GCA.MethodsThis is a retrospective observational study of patients suspected of having GCA and referred to our US fast track clinic. All patients underwent US exam within 24 hours per protocol. The IMT was measured in gray scale mode in cranial and extra-cranial (carotid, subclavian and axillary) arteries and the Halo Score was also determined to assess the extent of vascular inflammation. GCA diagnosis was confirmed after 6-month follow-up by the referring clinician. The European Society of Cardiology (ESC) Guidelines on CV Disease Prevention in clinical practice were used to define different categories of CVR. Patients were classified as very high, high, moderate or low CVR according to the Systemic Coronary Risk Evaluation (SCORE) obtained using the ESC CVD Risk Calculator app for mobile devices. Comparison between groups was performed and the diagnostic accuracy of the Halo Score in patients according to CVR was evaluated using ROC curves.ResultsOf the 157 patients referred to our US fast track clinic (67.5% female, mean age 73.7 years), 47(29.9%) had GCA confirmed after 6-month follow-up. There were no differences in CVR between patients with and without GCA (mean SCORE 20.6[21.6] vs 18.7[21];p=0.601). Among patients without GCA, extra-cranial artery IMT was significantly higher in patients with high/very high CVR than in those with low/moderate CVR (Table 1). The Halo Score was significantly higher in patients with high/very high CVR in non-GCA patients (9.38 (5.93) vs 6.16 (5.22);p=0.007). The area under the ROC curve of the Halo Score to identify GCA was 0.835 (CI95% 0.756-0.914), slightly greater in patients with low/moderate CVR (0.965 [CI95% 0.911-1]) versus patients with high/very high CVR (0.798[CI95% 0.702-0.895]) (Figure 1). A statistically weak positive correlation was found between the Halo Score and the SCORE (r 0.245;p=0.002).Table 1.Measurements of IMT in cranial and extracranial arteries and Halo Score values according to CVRArtery IMT mm, mean (SD)Patients with GCA n=47Patients without GCA n=110Patients with high/very high CVR n=37(78.7%)Patients with low/moderate CVR n=10(21.3%)pPatients with high/very high CVR n=79(71.8%)Patients with low/moderate CVR n=31(28.2%)pSuperficial temporal artery (both)0.66(0.25)0.45(0.11)0.0250.35(0.09)0.32(0.07)0.354Frontal branch (both)0.42(0.18)0.31(0.15)0.0560.26(0.05)0.26(0.06)0.577Parietal branch (both)0.43(0.17)0.35(0.12)0.1020.27(0.04)0.28(0.08)0.173Carotid artery (both)0.88(0.21)1.2(0.6)0.83(0.16)0.74(0.13)Subclavian artery (both)0.86(0.31)1.2(0.5)0.0010.74(0.18)0.6(0.13)Axillary artery (both)0.92(0.38)1.22(0.73)0.0210.72(0.16)0.59(0.15)Halo Score, mean (SD)18.5(8.8)17.2(10.6)0.699.38(5.93)6.16(5.22)0.007Figure 1.Diagnostic accuracy of the Halo Score for a clinical diagnosis of GCA after 6-month follow-up in (A) all GCA suspected patients, (B) patients with high/very high CVR and (C) patients with low/moderate CVRConclusionHigh CVR may influence the diagnostic accuracy of the US Halo Score leading to false-positive findings in these patients. Higher IMT values may be found in extracranial arteries of subjects with high/very high CVR without GCA. Thus, CVR should be taken into consideration in the US vascular assessment of patients with suspected GCA. These results need to be confirmed in larger cohorts to develop a modified US Halo Score applicable to patients with high CVR.Disclosure of InterestsNone declared

BackgroundLarge-vessel vasculitis are characterized by the wall inflammation of the involved vessels, which can be detected by imaging tools (1-3). Ultrasound (US) is one of the most commonly used tools for the diagnosis of giant cell arteritis (GCA), especially in patients with a cranial phenotype. Tocilizumab (TCZ) has shown efficacy in large-vessel vasculitis (LVV) including GCA (4,5). However, the improvement objectified by imaging techniques such as US after TCZ therapy is poorly documented.ObjectivesTo assess the effectiveness of TCZ improving the wall vessel inflammation by US.MethodsObservational, multicenter study of 26 GCA patients treated with TCZ. GCA was diagnosed according to: a) ACR criteria, and/or b) biopsy of temporal artery, and/or c) presence of signs of vessel wall inflammation by US, defined by the presence of halo sign. In all the cases a baseline US and in the follow-up was mandatory.Patients were divided into two subgroups: a) with, and b) without signs of improvement (partial or total) in the follow-up US.ResultsWe studied 26 patients (19 women/7 men; mean age, 76.3±9.7 years). Main clinical features of GCA with and without US improvement are shown in Table 1. We found no significant differences in any of the variables studied between the two groups.Table 1.Main features of 27 GCA patients treated with tocilizumab followed by Ultrasound (US).With US improvement (n=21)Without US improvement (n=5)pBaseline characteristics at TCZ onsetGeneral characteristicsAge(years), mean±SD77.3±8.972.2±12.90.270Sex, female/male (% female)17/4 (80,95)2/3 (40)0.101Time from GCA diagnosis to TCZ onset (months), median [IQR]6 [3-9]3 [1-6]0.452Systemic manifestations, n (%)Fever, n (%)1/21 (4.76)1/5 (20)0.354Constitutional syndrome, n (%)10/21 (47.62)2/5 (40)0.999PmR, n (%)11/21 (52.38)1/5 (20)0.330Ischaemic manifestations, n (%)Visual involvement, n (%)1/21 (4.76)1/5 (20)0.354Headache, n (%)15/21 (71.43)5/5 (100)0.298Jaw claudication, n (%)4/15 (26.67)¼ (25)0.999Laboratory dataESR, mm 1st hour, median [IQR]33 [22-49]55 [54-80]0.216CRP, mg/dL, median [IQR]1.5 [0.7-6.7]3.8 [1-4.2]0.948Prednisone dose, mg/day, median [IQR]13.7 [10-30]30 [12.5-30]0.505Time from TCZ onset and follow-up US (months)3.9±3.63.1±2.10.456After TCZ onset, 21 of 26 patients (80.7%) showed US signs of improvement (12 complete, 9 partial). In 4 out of 5 patients in whom there was no improvement in US findings, clinical improvement was observed at first month after starting TCZ.ConclusionTCZ seems to be effective controlling GCA including vascular involvement detected by US. This improvement can be seen by follow-up US, especially when performed at least 3 months after TCZ onset.References[1]Loricera J, et al. Rev Esp Med Nucl Imagen Mol. 2015; 34: 372-7. PMID: 26272121[2]Loricera J, et al. Clin Exp Rheumatol. 2015; 33: S19-31. PMID: 25437450[3]Prieto-Peña D, et al. Ther Adv Musculoskelet Dis. 2021; 13: 1759720X211020917. PMID: 34211589[4]Martínez-Rodríguez I, et al. Semin Arthritis Rheum. 2018; 47: 530-537. PMID: 28967430[5]Prieto-Peña D, et al. Semin Arthritis Rheum. 2019; 48: 720-727. PMID: 29903537AcknowledgementsTocilizumab in Giant Cell Arteritis Spanish Collaborative Group: Juan C. González Nieto (H. Gregorio Marañón), Juan R. de Dios (H.U. Araba), Esther Fernández (H. Clínico Universitario Virgen de la Arrixaca), Isabel de la Morena (H. Clínico Universitario de Valencia), Patricia Moya (H. Sant Pau), Roser Solans i Laqué (H. Valle de Hebrón), Eva Pérez Pampín (H.U. de Santiago), José L. Andréu (H.U. Puerta de Hierro), Marcelino Revenga (H. Ramón y Cajal), Juan P. Baldivieso Achá (H. U. de La Princesa), Eztizen Labrador (H. San Pedro), Andrea García-Valle (Complejo Asistencial Universitario de Palencia), Adela Gallego (Complejo Hospitalario Universitario de Badajoz), Carlota Iñíguez (H.U. Lucus Augusti), Cristina Hidalgo (Complejo Asistencial Universitario de Salamanca), Noemí Garrido-Puñal (H. Virgen del Rocío), Ruth López-González (Complejo Hospitalario de Zamora), José A. Román-Ivorra (H.U. y Politécnico La Fe), Sara Manrique (H. Regional de Málaga), Paz Collado (H.U. Severo Ochoa), Enrique Raya (H. San Cecilio), Valvanera Pinillos (H. San Pedro), Francisco Navarro (H. General Universitario de Elche), Alejandro Olivé-Marqués (H. Trías i Pujol), Francisco J. Toyos (H.U. Virgen Macarena), María L. Marena Rojas (H. La Mancha Centro), Antoni Juan Más (H.U. Son Llàtzer), Beatriz Arca (H.U. San Agustín), Carmen Ordás-Calvo (H. Cabueñes), María D. Boquet (H. Arnau de Vilanova), Noelia Álvarez-Rivas (H.U. Lucus Augusti), María L. Velloso-Feijoo (H.U. de Valme), Cristina Campos (H. General Universitario de Valencia), Íñigo Rúa-Figueroa (H. Doctor Negrín), Antonio García (H. Virgen de las Nieves), Carlos Vázquez (H. Miguel Servet), Pau Lluch (H. Mateu Orfila), Carmen Torres (Complejo Asistencial de Ávila), Cristina Luna (H.U. Nuestra Señora de la Candelaria), Elena Becerra (H.U. de Torrevieja), Nagore Fernández-Llanio (H. Arnáu de Vilanova), Arantxa Conesa (H.U. de Castellón), Eva Salgado (Complejo Hospitalario Universitario de Ourense).Disclosure of InterestsJulio Sanchez-Martin: None declared, Javier Loricera: None declared, Lara Sanchez-Bilbao: None declared, Eugenio de Miguel: None declared, Rafael Melero: None declared, E. Galíndez-Agirregoikoa: None declared, J. Narváez: None declared, Carles Galisteo: None declared, Juan Carlos Nieto González: None declared, Patricia Moya: None declared, Eztizen Labrador-Sánchez: None declared, Miguel A 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, Lilly, Pfizer, Roche, Bristol-Myers, Janssen, UCB Pharma and MSD, Grant/research support from: Abbvie, MSD and Roche

BackgroundUltrasound (US) is a valid imaging tool to detect signs of giant cell arteritis (GCA). Although the halo sign has always been considered the most useful finding for GCA diagnosis, modern high frequency transducers are able to precisely measure the intima-media thickness (IMT) of cranial and extracranial arteries. However, data on optimal cut-off values for IMT to differentiate patients and controls in clinical practice are limited.ObjectivesTo determine the optimal cut-off value for IMT of cranial and extracranial arteries in patients with suspected GCA.MethodsThis is a retrospective observational study of patients referred to our US fast-track clinic with suspected GCA. All patients underwent bilateral US examination of the cranial and extracranial (carotid, subclavian and axillary) arteries within 24 hours per protocol. The exam was performed using an EsaoteMyLab8 with a 12-18 MHz frequency transducer for cranial arteries and an 8-14 frequency transducer for extracranial arteries. The IMT was measured in gray scale mode and the presence of a non-compressible halo sign was checked in all arteries. The gold standard for GCA diagnosis was clinical confirmation by the referring rheumatologist after 6 months follow-up. Mean IMT values of each artery were compared between patients with and without GCA by independent samples T-test. Receiver operating characteristics analysis was performed and the Youden index was used to determine the optimal cut-off value for IMT of each artery.ResultsOf the 157 patients with suspected GCA (67.5% female, mean age 73.7 years) referred to the fast-track clinic, 47 (29.9%) had GCA clinical confirmation after 6 months. 41 (87.2%) patients with GCA had positive US findings (61.7% had cranial involvement, 44.7% extracranial involvement and 19.1% a mixed pattern of cranial and extracranial arteries). The following IMT cut-off values showed the highest diagnostic accuracy: 0.44mm for the common superficial temporal artery; 0.34 mm for the frontal branch; 0.36 mm for the parietal branch; 1.1 mm for the carotid artery: 1 mm for the subclavian and axillary arteries. The area under the ROC curve of the IMT for a clinical diagnosis of GCA was 0.984 (95% CI 0.959 - 1) for common superficial temporal artery, 0.989 (95% CI 0.976 -1) for frontal branch, 0.991 (95% CI 0.980 - 1) for parietal branch, 0.977 (95% CI 0.961 – 0.993) for carotid, 0.99 (95% CI 0.979 - 1) for subclavian and 0.996 (95% CI 0.991 -1) for axillary arteries (Table 1).Table 1.Optimal IMT cut-off values for cranial and extracranial arteriesArterySidePatients without GCAPatients with GCACut-off (mm)AUC (CI 95%)Sensitivity (%)Specificity (%)Common superficialtemporal artery mm, mean (SD)Right0.33 (0.06)0.68 (0.28)0.430.997 (0.988 -1)10097.1Left0.35 (0.11)0.57 (0.21)0.450.966 (0.905 -1)10092.3Both0.34 (0.08)0.63 (0.25)0.440.984 (0.959 -1)94.795.1Frontal branch mm, mean (SD)Right0.26 (0.05)0.4 (0.18)0.340.994 (0.983 -1)10097.1Left0.27 (0.05)0.4 (0.18)0.340.985 (0.962 -1)10096.1Both0.26 (0.05)0.4 (0.18)0.340.989 (0.976 -1)10096.6Parietal branch mm, mean (SD)Right0.27 (0.05)0.43 (0.18)0.360.994 (0.981 -1)10098.9Left0.27 (0.05)0.41 (0.16)0.360.987 (0.967 -1)10097.6Both0.27 (0.05)0.42 (0.17)0.360.991 (0.980 -1)10098.3Carotid mm, mean (SD)Right0.8 (0.17)0.88 (0.29)10.974 (0.949 – 0.999)10092.6Left0.82 (0.15)1 (0.42)1.20.982 (0.961 - 1)90.996.2Both0.81 (0.16)0.96 (0.36)1.10.977 (0.961 – 0.993)9094Subclavian mm, mean (SD)Right0.74 (0.18)0.99 (0.44)10.987 (0.97 - 1)10093.4Left0.67 (0.17)0.9 (0.35)1.10.991 (0.975 - 1)10098.3Both0.7 (0.18)0.94 (0.4)10.99 (0.979 - 1)10096Axillary mm, mean (SD)Right0.69 (0.16)0.99 (0.5)10.992 (0.982 - 1)10096Left0.67 (0.17)0.99(0.49)10.998 (0.995 -1)10098.3Both0.68 (0.17)0.99 (0.49)10.996 (0.991 -1)10097.1ConclusionDifferent IMT cut-off values for each artery are necessary to establish a correct US diagnosis of GCA. These proposed IMT cut-off values may help to improve the diagnostic accuracy of US in clinical practice.Disclosure of InterestsNone declared

Background:Coronavirus disease 2019 (COVID-19), has raised several questions in patients with immune-mediated inflammatory diseases (IMID). Whether the seroprevalence and factors associated with symptomatic COVID-19 are similar in IMID patients and in the general population is still unknown.Objectives:To assess the serological and clinical prevalence of COVID-19 in European IMID patients, along with the factors associated with its risk and the impacts the pandemic had on the IMID management.Methods:Prospective multicentre cross-sectional study among patients with five IMID (i.e. systemic lupus erythematous, Sjögren’s syndrome, rheumatoid arthritis, axial spondylarthritis or giant cell arteritis) from six tertiary-referral centers from France, Germany, Italy, Portugal, Spain and United Kingdom. Demographics, comorbidities, IMID, treatments, flares and COVID-19 details were collected. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) serological tests were systematically performed.Results:Between June 7 and December 8, 2020, 3028 patients were included (median age 58 years, 73.9% females). SARS-CoV-2 antibodies were detected in 166 (5.5%) patients. Symptomatic COVID-19 was seen in 122 patients (prevalence: 4.0%, 95% CI 3.4-4.8%); 23 (24.2%) of them were hospitalized and four (3.2%) died. In multivariate logistic regression analysis, symptomatic COVID-19 was more likely to be observed in patients with higher levels of C-reactive protein (OR: 1.18; 95% CI 1.05-1.33; p = 0.006), and increased with the number of IMID flares (OR: 1.27; 95% CI 1.02-1.58; p = 0.03). Conversely, it was less likely to occur in patients treated with biological therapy (OR: 0.51; 95% CI 0.32-0.82; p = 0.006). During the pandemic, at least one self-reported disease flare was seen in 654 (21.6%) patients. Also, 519 (20.6%) patients experienced changes in their treatment, with 125 of these (24.1%) being due to COVID-19.Conclusion:The SARS-CoV-2 prevalence in IMID patients over the study period seems to be similar to that of the general population1. The IMID inflammatory status seems to be independently associated with the development of COVID-19.References:[1]Pollán M, Pérez-Gómez B, Pastor-Barriuso R, Oteo J, Hernán MA, Pérez-Olmeda M, et al. Prevalence of SARS-CoV-2 in Spain (ENE-COVID): a nationwide, population-based seroepidemiological study. Lancet Lond Engl. 2020 Aug 22;396(10250):535–44.Disclosure of Interests:None declared.

Background:Giant cell arteritis (GCA) may be divided into cranial, and extracranial GCA. Tocilizumab (TCZ) has shown efficacy and safety in GCA and other large-vessel vasculitis (LVV) (1-5).Objectives:To compare the efficacy of TCZ in cranial and extracranial GCA.Methods:Multicenter observational study of 312 patients with GCA treated with TCZ. They were divided into 3 groups a) only cranial (cGCA), b) only extracranial (ecGCA), c) mixed affection (mixGCA). GCA was diagnosed by a) ACR criteria, and/or b) positive temporal artery biopsy, and/or c) LVV by imaging. Remission and sustained remission was defined according to EULAR definitions (1). In ecGCA and mixGCA we also studied the improvement (complete or partial) by imaging techniques.Results:We studied 312 patients (218 females; mean age, 73.4±9.6 years). TABLE shows the main features of the 3 groups. Remission at month 6 was higher in cGCA, as well as the sustained remission at month12 (FIGURE). At 18 and 24months, were similar in the 3 groups. Improvement by imaging techniques was partial/complete at 6,12,18 and 24 months, in 50%/0%,71%/0%, 61%/15% and 67%/17% respectively, in ecGCA, and in 75%/0%,53%/18%, 64%/12% and 50%/28% in mixGCA.Table 1.Main features of 312 patients at TCZ onset.Cranial GCA(n=152)Extracranial GCA(n=49)Mixed GCA(n=111)Cranial vs Extracranial GCApAge at TCZ onset, years, mean± SD76.0±8.265.4±12.273.5±8.10.000*Sex, female/male, n (% female)105/47 (69)33/16 (67)80/31 (72)0.960Time from diagnosis to TCZ onset (months, median [IQR]6 [2-21]7 [2-20]9 [3-25]0.765Biopsy-proven GCA, n (%)87/128 (68)0 (0)50/87 (57)0.000*Systemic manifestations at TCZ onset109 (72)32 (65)84 (76)0.501Fever, n (%)18 (12)1 (2)8 (7)0.048*Constitutional syndrome, n (%)52 (34)16 (33)47 (42)0.933PmR, n (%)88 (58)29 (59)71 (64)0.999Ischemic manifestations at TCZ onset117 (77)0 (0)70 (63)0.000*Visual involvement, n (%)31 (20)0 (0)16 (14)0.000*Headache, n (%)103 (85)0 (0)63 (57)0.000*Jaw claudication, n (%)39 (26)0 (0)21 (19)0.000*Acute phase reactantsESR, mm/1st hour, median [IQR]28 [9-53]24 [10-43]28 [15-48]0.462CRP, mg/dL, median [IQR]1.2 [0.3-3.4]0.7 [0.4-1.8]1.6 [0.4-3.8]0.153Prednisone dose at TCZ onset, mean ± SD26.2±17.615.4±14.220.1±14.90.000*TCZmono/TCZcombo, n (% TCZ mono)116/36 (76)26/23 (53)69/42 (62)0.003*Follow-up (months), mean ± SD27.3±21.132.7±23.327.9±22.00.143Figure 1.Remission and sustained remission of cGCA, ecGCA and mixGCA according to EULAR (1). In the first 3 months we only could assess cGCA because in ecGCA and mixGCA a control imaging was not performedConclusion:TCZ seems to be effective in all phenotypes but it is faster in cGCA in reaching remission. However, improvement by imaging techniques was partial and very rarely complete in ecGCA and mixGCA.References:[1]Hellmich B, et al. Ann Rheum Dis. 2020; 79: 19-30.[2]Stone JH, et al. N Engl J Med. 2017; 377: 317-28.[3]Calderón-Goercke M, et al. Semin Arthritis Rheum 2019; 49:126-35. https://doi.org/10.1016/j.semarthrit.2019.01.003.[4]Prieto Peña D et al. Clin Exp Rheumatol 2020 Nov 27. PMID: 33253103.[5]Loricera J, et al. Clin Exp Rheumatol 2016; 34:S44-53. PMID: 27050507Disclosure of Interests:Lara Sanchez-Bilbao: None declared, Javier Loricera: None declared, Vicente Aldasoro: None declared, Juan Pablo Valdivieso-Achá: None declared, Ignacio Villa-Blanco: None declared, Olga Maiz: None declared, Rafael Melero: None declared, Clara Moriano: None declared, Julio Sánchez: None declared, Eugenio de Miguel: None declared, Eva Perez-Pampín: None declared, Juan Ramón De Dios: None declared, Juan Carlos Nieto González: None declared, Eva Galíndez-Agirregoikoa: None declared, Patricia Moya: None declared, Francisca Sivera: None declared, José Luis Andréu Sánchez: None declared, Valvanera Pinillos: None declared, Andrea García-Valle: None declared, Paloma Vela-Casasempere: None declared, Noelia Alvarez-Rivas: None declared, Marcelino Revenga: None declared, Sara Manrique Arija: None declared, Carlos Fernández-López: None declared, Enrique Raya: None declared, Cristina Hidalgo: None declared, Ruth López-González: None declared, Cristina Campos Fernández: None declared, Antonio Juan-Mas: None declared, Beatriz Arca: None declared, Iñigo Rua-Figueroa: None declared, María Dolors Boquet: None declared, Antonio García: None declared, Adela Gallego: None declared, Eva Salgado-Pérez: None declared, Miguel A 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, Lilly, Pfizer, Roche, Bristol-Myers, Janssen, UCB Pharma and MSD, Grant/research support from: Abbvie, MSD and Roche

Background:There’s little data of patients with immune diseases (ID) treated with biologic and JAK-inhibitors and COVID19. Current consensus is to keep treatment, however more studies are needed to ascertain the risk in these patients.Objectives:To describe the associated risk factors and outcomes in hospitalized patients with ID treated with biologics and JAK-inhibitors of a tertiary center.Methods:Observational retrospective study of patients with COVID19 from March 1st 2020 to January 31st 2021. Out of all the patients receiving subcutaneous (SC) or intravenous (IV) biologics and oral (PO) JAK-inhibitors, we selected those requiring hospitalization due to pneumonia for analysis. We collected demographic data, comorbidities, seasonal flu vaccination, smoking history and the outcome (discharge/admission in an intensive care unit (ICU)/death). We used a composite index (Charlston’s index) for comorbidities.Results:Of 153 patients, 29 (18.9%) were hospitalized. 18 (62%) were women with a median age of 61 (IQ 52-69). 14 (48.2%) had rheumatoid arthritis, 5 (17%) had axial spondylarthritis and 4 (13.7%) had Chron’s disease. The main IV was Rituximab in 3 (50%), and abatacept, infliximab and vedolizumab had one each. Of the SC, tocilizumab and adalimumab had 5 (22.7%) each, etanecept and golimumab had 3 (13.6%) each and secukinumab, ustekinumab and abatacept had 2 (9%) each. The PO was tofacitinib. There were no outcome differences for each treatment.24 (82.7%) patients had at least 1 comorbidity with significative difference between patients (Table 1). There were 6 (20.6%) deaths, 3 (50%) in the ICU, 2 (33%) did not meet the ICU criteria, and 1 (16%) before ICU admission. None them had the same ID.Table 1.Baseline data.Variables, (%)Total29 (100)Deaths 6 (20.6)Discharged23 (79.3)PWomen (%)18 (62)4 (66.6)14 (61)0.79Age, Median (IQ range)61 (52-69)69.5 (62-78.5)59.9 (51-65.5)0.09Flu vaccine (%)15 (51.7)5 (83.3)10 (43.7)0.08ICU (%)8 (27.5)3 (50)5 (22)0.16TreatmentIV (%)6 (20.1)4 (66.7)2 (8.7)0.001SC (%)22 (75.8)2 (33.3)20 (87)PO (%)1 (3.4)0 (0)1 (4.3)0.6ComorbiditiesCharlston, Mean (standard deviation)2.72 (+/-1.6)5.16 (+/- 1.8)2.08 (+/-1.5)Hypertension/HTA (%)16 (55)5 (83.3)11 (48)0.11Dyslipidemia/DL (%)12 (41.3)4 (66.7)8 (34.8)0.15Obesity (%)10 (34.5)1 (16.7)9 (39.1)0.30Chronic obstructive pulmonary disease/COPD (%)3 (10.3)3(50)0 (0)Heart disease (%)8 (27.5)4 (66.6)4 (17.4)0.01Chronic kidney disease/CKD (%)7 (24.1)4 (66.6)3 (13)0.006Diabetes mellitus /DM (%)7 (24.1)3 (50)4 (17.4)0.001Previous or current smokers (%)9 (31)3 (50)6 (26)0.26Conclusion:The rate of COVID19 hospitalization in our patients was comparable to the general population’s (between 19-24% from 60 years plus) and the risk of in-patient death is also similar, around 24%1. Our study suggests that neither their ID nor their treatment influences their risk of a worse outcome.COPD, DM and previous heart disease were associated with worse outcome; however it seems that the main prognostic factor was the overall impact of comorbidities associated; as measured by the Charlston’s index, being significantly higher in the patients with a fatal outcome.A fatal outcome was more likely in IV biologics, however it could be explained by indication bias probably due to higher comorbidity and disability in these patients rather than an independent prognostic variable.References:[1]Mesas AE et al. Predictors of in-hospital COVID-19 mortality: A comprehensive systematic review and meta-analysis exploring differences by age, sex and health conditions. PLoS One. 2020 Nov 3;15(11)[2]Jovani, V et al.Incidence of severe COVID-19 in a Spanish cohort of 1037 patients with rheumatic diseases treated with biologics and JAK-inhibitors. ARD 2020–218152.Disclosure of Interests:None declared

Background:EULAR recommendations propose temporal and axillary arteries ultrasound (US) as first-line investigation when predominantly cranial giant cell arteritis (GCA) is suspected. Recently, two novel US scoring systems, the halo count and the Southend Halo Score, have been developed to quantify the extent of inflammation by US in GCA.Objectives:To assess whether adding the subclavian arteries examination into the ultrasound (US) Southend Halo Score, as proposed in the modified Halo Score, improves the diagnostic accuracy of GCA and its relationship with systemic inflammation.Methods:Retrospective observational study of patients referred to a GCA fast track pathway (FTP) over a 1-year period. Patients underwent US exam of temporal and large vessel (LV) (carotid, subclavian and axillary) arteries. The extent of inflammation was measured by the halo count, the Southend Halo Score and the modified Halo Score (Image 1). The gold standard for GCA diagnosis was clinical confirmation after 6 months follow-up.Results:64 patients were evaluated in the FTP, 17(26.5%) had GCA. Subclavian arteries involvement was present only in patients with GCA (29.4% versus 0%,pFigure 1.Proposed scores to quantify the extent of vascular inflammation by ultrasound in giant cell arteritis. A. Halo count, B. Halo Score, C: Modified Halo ScoreTable 1.Clinical, laboratory and ultrasound findings of patients included in the fast track pathway with or without GCA clinical confirmation.Totaln=64Patients with GCAn=17Patients without GCAn=47pAge, median (IQR)78 (69.3-83)78 (72.5-83)78 (66-83)0.5Female, n (%)42 (65.6%)10 (58.8%)32 (68.1%)0.491Temporal artery biopsy positive n=13, no. of patients5 (38.5%)5 (50%)0 (0%)0.23118F-FDG-PET/CT positive n=14, no. of patients7 (50%)5 (62.5%)2 (33.3%)0.592Fulfilling 1990 GCA criteria, no. of patients16 (25%)8 (47.1%)8 (17%)0.022PMR diagnosis before US examination, no. of patients21 (32.8%)4 (23,5%)17 (36,2%)0.386Headache, no. of patients31 (48.4%)12 (70.6%)19 (40.4%)0.033Jaw claudication, no. of patients12 (18.8%)9 (52.9%)3 (6.4%)Ocular ischaemia, no. of patients4 (6.3%)2 (11.8%)2 (4.3%)0.285Abnormal TA clinical examination, no. of patients5 (7.8%)3 (17.6%)2 (4.3%)0.112CRP (mg/dL), median (IQR)1.7(0-6.5)7 (2.1-14)1.1 (0-5.1)0.001ESR (mm/h), mean (SD)52.8 (34.6)68.3 (33.3)46.8 (33.3)0.044Haemoglobin (g/dL), mean (SD)12.5 (1.7)11.8 (1.6)12.7 (1.7)0.059Platelets 109/L, mean (SD)276.1 (105.8)323.4 (116.3)258.7 (97.3)0.52Positive US findings, no. of patients17 (26.6%)15 (88.2%)2 (4.3%)Temporal artery positive US findings, no. of patients13 (20.3%)12 (70.6%)1 (2.1%)Axillary positive US findings, no. of patients9 (14.1%)8 (47.1%)1 (2.1%)Subclavian positive US findings, no. of patients5 (7.8%)5 (29.4%)0 (0%)Temporal artery + axillary or subclavian positive US findings, no. of patients5 (7.9%)5 (29.4%)0 (0%)0.003Halo Count, median (IQR)0 (0-0.75)2 (1-4.5)0 (0-0)Halo Score, median (IQR)0 (0-4.5)18 (7-22.5))0 (0-0)Modified Halo Score, median (IQR)0 (0-2.75)8 (3-13.5)0 (0-0)Conclusion:The inclusion of subclavian arteries examination in the modified Halo Score does not improve the diagnostic accuracy of GCA. Nevertheless, it correlates better with markers of systemic inflammation in LV-GCADisclosure of Interests:None declared

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

Background:Juvenile idiopathic arthritis (JIA) requires frequent systemic treatments, the ideal goal being to achieve remission. Questions remain as to when treatment can be stopped and how to balance the risks and benefits of continuing medications against the possibility of a flare after discontinuation. There is currently little conclusive evidence on the management of JIA in remission.Objectives:To determine the time to remission and frequency of flares in patients with JIA after withdrawal of systemic treatment in a single-centre cohort.Methods:Patients with JIA visited in the last 15 years were included. A retrospective cross-sectional study was performed. Demographic data such as sex, age at diagnosis, current age, subcategories of JIA were collected. Systemic treatment was also collected (date of discontinuation, date of flare after discontinuation, time in remission, time of treatment until discontinuation, discontinuation of biologic therapy while maintaining methotrexate). For the analysis of qualitative variables, absolute and relative frequencies were used; for quantitative variables, median and IR; for comparative analysis, Chi-square and Mann-Whitney U test.Results:We included 146 patients with JIA. Demographic data are shown in Table 1. 134 (90.4%) patients required systemic treatment (synthetic and/or biological DMARDs), of which 61 (45.5%) discontinued treatment completely at some point. Of the total number of patients who discontinued all systemic treatment, 19 (31.1%) had a flare with a median time in remission of 4.1 years (IR 1.3-9.1). 17 of the 134 patients (12.7%) with systemic treatment discontinued biologic therapy while maintaining methotrexate, of which 12 (70.6%) patients had a flare with a median time in remission of 0.9 years (IR 0.6-1.2). The differences between the frequency of flares and median time in remission between both treatment discontinuation groups (all systemic treatment versus discontinuation of biologic while maintaining methotrexate) were statistically significant (pTable 1.Demographic characteristics of patients with JIAVariables N 146Sex: Women n (%) 89 (60.3)Age at onset (years, median (IR)) 4.6 (2.5-9.2)Current age (years, median (IR))16.7 (11.1-20.9)Types of JIA:Persistent oligoarticular n (%) 66 (45.2)Extended oligoarticular n (%) 11 (7.5)Polyarticular RF - n (%) 29 (19.9)Polyarticular RF + n (%) 3 (2.1)Enthesitis-related n (%) 12 (8.2)Psoriatic n (%) 9 (6.2)Systemic n (%) 16 (11.0)Conclusion:One third of the patients in whom all systemic treatment was withdrawn had a flare (70% remain in remission without medication) with a median time in remission of 4 years. On the other hand, two-thirds of patients in whom biologic therapy was withdrawn while maintaining methotrexate had a flare, with a shorter time in remission. The differences between both groups with withdrawal of systemic treatment are statistically significant (pReferences:[1]Halyabar O, Mehta J, Ringold S, Rumsey DG, Horton DB. Treatment Withdrawal Following Remission in Juvenile Idiopathic Arthritis: A Systematic Review of the Literature. Paediatr Drugs. 2019 Dec;21(6):469-492. doi: 10.1007/s40272-019-00362-6.[2]Simonini G, Ferrara G, Pontikaki I, Scoccimarro E, Giani T, Taddio A, Meroni PL, Cimaz R. Flares After Withdrawal of Biologic Therapies in Juvenile Idiopathic Arthritis: Clinical and Laboratory Correlates of Remission Duration. Arthritis Care Res (Hoboken). 2018 Jul;70(7):1046-1051. doi: 10.1002/acr.23435.[3]Chhabra A, Robinson C, Houghton K, Cabral DA, Morishita K, Tucker LB, Petty RE, Larché M, Batthish M, Guzman J. Long-term outcomes and disease course of children with juvenile idiopathic arthritis in the ReACCh-Out cohort: a two-centre experience. Rheumatology (Oxford). 2020 Dec 1;59(12):3727-3730. doi: 10.1093/rheumatology/keaa118.Disclosure of Interests:None declared

Background:Patients with inflammatory arthralgia (IA) are considered to be at increased risk for progression to RA. Ultrasound (US) has shown high sensitivity to detect synovitis compared with physical examination. Thus, US is recommended to identify subclinical synovitis in patients without clinical signs of inflammation.Objectives:To determine the frequency and pattern of US detected active inflammation in patients with IA and investigate factors contributing to predict this outcome.Methods:An US clinic is scheduled in an academic center running twice every week. A retrospective analysis of our US unit cohort during a period of 12 months was undertaken. Patients with IA and no previous diagnosis of inflammatory arthropathies were included for analysis. Inclusion criteria of IA definition included: severe symptoms presenting in the morning, duration of morning stiffness ≥60 min, symptoms predominantly located in MCP joints and absence of clinically detected synovitis by the referral rheumatologist. The following routinely collected variables were included in the analysis: demographics, clinical features and laboratory tests. Patients underwent bilateral US examination of hands and/or feet according to the European League Against Rheumatism (EULAR) guidelines. The presence of synovitis and tenosynovitis was assessed on a semi quantitative scale (0–3) for Grey Scale(GS)/Power Doppler(PD). Active inflammation was defined as PD synovitis and/or tenosynovitis >1 at any location. First, differences between groups were tested using chi-squared/Fisher and Student-t tests in the univariate analysis. Second, multivariate logistic regression models were employed to investigate the association between possible predictive factors of US active inflammation.Results:A total of 110 patients were included in the analysis. Mean age was 53.6±15.6 years, 80 (72.7%) were females, and mean symptoms duration was 11.7±9.9 months (Table1). A total of 76 (69.1%) patients presented with a polyarticular arthralgia pattern. US active inflammation were present in 38 (34.5%) patients (28.2% showed PD synovitis and 19.1% PD tenosynovitis). Hands were most commonly involved with PD synovitis at wrists in 18.2% and at MCP in 14.5% of patients. For PD tenosynovitis, the flexor MCP 2-5 (4.5%) and 6th extensor tenosynovitis (5.5 %) were the most frequent affected locations. Only 9 (8.2%) patients had erosions in hands and/or feet at baseline examination. In the univariate analysis, the higher ESR values, the shorter time from symptoms onset and the presence of ACPA were significantly associated with the presence of US active inflammation (pConclusion:US features of active inflammation are found in 1 over 3 patients with IA being PD synovitis the most common finding, specially at the wrists and MCP joints. Higher ESR and ACPA values are significantly associated with the presence of US active inflammation. Thus, we strongly recommend the use of PD US to detect subclinical inflammation in at-risk patients with IA with no sign of inflammation on clinical examination, especially those with high ESR and ACPA values.Table 1.Baseline characteristics of patients with IATotaln= 110US inflammatoryfindingsn= 38 (34.5%)Non-US inflammatoryfindingsn=72 (65.5%)pAge53.6 ± 15.657.2±16.251.6±13.40.071SexFemale80 (72.7%)26 (68.4%)54 (75%)0.461Smokingn= 87Non smoker45 (51.7%)12 (44.4%)33 (55%)0.412Smoker34 (39.1%)11 (40.7%)23 (38.3%)Former smoker8 (9.2%)4 (14.8%)4 (6.7%)ExtensionMonoarticular12 (10.9%)6 (15.8%)6 (8.3%)0.176Oligoarticular 22 (20%)10 (26.3%)12 (16.7%)Polyarticular76 (69.1%)22 (57.9%) 54 (75%)Time (months)from symptoms onset11.7 ± 9.99.1±8.113±10.50.035ESR (mm/h) n=4524.7 ± 18.233.1±21.820.3 ±14.4RF (IU/mL) n=5339.1 ± 230.528.5±5645.1±286.10.647ACPA (IU/mL) n=5698.1 ± 331.2209.4±488.426±125.20.01Disclosure of Interests:None declared

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

Background Chronic recurrent multifocal osteomyelitis (CRMO) is a rare autoinflammatory polygenic bone disease characterised by aseptic bone inflammation in paediatric population. Its management, clinical, radiological findings and treatment have not yet been standardised. Objectives Retrospective, descriptive multicentric study of patients diagnosed of CRMO in four tertiary level hospitals’ paediatric rheumatology section. There were 16 patients included. The clinical, radiological characteristics where analysed as well as response to treatment options. Results The median age at diagnosis was 10,5 years, female:male ratio 62,5:37,5%. The delay in the diagnosis had a median of 4.5 months, being less than one year in 11 patients. Bone pain was the first symptom in 100% of the patients accompanied by fever in 25% of them. A single patient presented perilesional arthritis. A slight-moderate increase on acute fase reactants was observed at the debut of the disease: median ESR 47.5 mm/h. The median number of locations at debut was 2.5 (range 1–14), with multifocal involvement in 75%. The most frequent location was tibia (56%), followed by pelvis (44%) and vertebrae (31,25%). Other locations less fenquent were: carpus (12.5%), femur (12.5%) mandible (6%) and sternum (6%). Biopsy was performed in 14/16 patients and bone scintigraphy with Tc99 in 12/16 patients, with pathological uptake observed in 91.6% of cases. MRI was the radiological test of suspected diagnosis in 15/16 patients. NSAIDs were the initial treatment. 5 patients received different antibiotic therapy regimens, without clinical or radiological improvement. 56.25% of patients required other treatments. Systemic corticosteroids were used in 12.5% of patients and bisphosphonates in 43.75% (100% of patients with axial involvement). After 6 months of treatment with biphosphonates, 57.14% had complete remission, 28.57% partial remission and 14.28% worsening. 12.5% of the patients had a torpid evolution, receiving sequential therapies with multiple synthetic or biological DMARDs (Anakinra, Canakinumab, Etanercept), and another 12.5% required surgery. Conclusions The diagnosis of CRMO is a challenge in the absence of pathognomonic features which leads to delay in diagnosis and the initiation of treatment. In our centres the biphosphonates were the treatment strategy used in patients with spinal involvement with 85.67% response at 6 moths. Disclosure of Interest None declared

Background Inflammatory arthropathies are a group of diseases that have common characteristics, like unknown etiology, autoimmune or autoinflammatory pathogenesis, genetic predisposition and chronicity. These affect people of all ages and can cause physical, psychic, and social disability. Physical activity is essential to decrease symptoms of pain, fatigue and weakness, improving joint mobility, increasing muscle mass, flexibility and psychosocial health. Objectives To describe the physical activity in patients with inflammatory arthropathies. Methods In this transversal, observational study we evaluated all patients with inflammatory arthropathies treated with intravenous biologic therapy in a Day Hospital Unit (DHU) of a tertiary hospital. We collected the following data: demographics, indexes of disease activity according to each disease, fatigue questionnaire (FACIT), health questionnaire SF-12, physical activity questionnaire (IPAQ), and fibromyalgia questionnaire (FIRST). This represents a cohort of patients that initiate an educational program on the importance of physical activity in the management of the disease. Results We included 222 patients (60.8% female), with a median age (SD; range) of 56.19 (12.89; 25–82) and a median disease duration (SD) of 16.54 (9.6). Of all included patients, 54.1% had rheumatoid arthritis, 39.2% had spondyloarthropathy and 6.8% has psoriatic arthritis; 26.8% were in remission, 39.2% presented moderate activity disease and 13.5% had high disease activity. Forty five (20.3%) patients had fibromyalgia according to FIRST questionnaire. According to the IPAQ questionnaire, 19.8% performed high physical activity, 24.8% moderate physical activity and 55.4% low physical activity. The FACIT score was significantly greater in patients with low physical activity (p In a multivariate regression logistic, only the gender and the age related with the physical activity, and not the disease; males (p=0.024; Exp(B) 2,389; IC 95% 1,124- 5,059) and younger persons (p=0,004; Exp (B) 0,959; IC 95%: 0,943–0,987) performed more physical activity. Conclusions Patients with inflammatory joint diseases perform low physical activity. The nurse plays a very important role in educating and informing the patients about their illness, and helping them in changing their lifestyle, encouraging increased physical activity with appropriate programs depending on the alterations that produce each disease, in order to improve their quality of life. 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

Background Assessment of nail involvement is currently made by clinical assessment using nail psoriasis severity index (NAPSI). Whilst clinical assessment can detect superficial nail changes, the matrix and the extensor tendon region are not accessible for clinical assessment. Musculoskeletal (MS) ultrasound (US) is playing an important role in the evaluation of psoriatic arthritis (PsA) patients. Recently, MSUS has been more used in the evaluation of nail involvement in psoriasis (Pso) and PsA patients. Objectives The primary objective of this observational, cross-sectional study was to assess the MSUS morphological and vascular abnormalities in nail in PsA and Pso patients compared with healthy controls. The secondary objective was to compare MSUS and clinical assessment of the nail in PsA and Pso patients. Methods We included patients with PsA (diagnosed according to CASPAR criteria) and patients with Pso without joint involvement (diagnosed by an experienced dermatologist according to clinical findings) and healthy controls. Clinical evaluation consisted in the evaluation of the NAPSI and PASI scores. The MSUS evaluation consisted in the evaluation of 10 hand nails. In B-mode (BM) we evaluated the followings: thickness of the nail bed from the distal phalanx bone surface to the ventral plate (PB) according to Worstman X et al.; thickness of the nail from dorsal to ventral plate (IP); dorsal and ventral plate morphology, echogenicity and integrity. Additionally, we performed a color Doppler (CD) evaluation for the presence of CD signal at the nail bed and matrix level. A score for BM and different scores for CD were calculated for each nail and sums of all nails for BM and CD scores were calculated for each patient. Results We evaluated 60 patients with PsA, 23 with Pso and 20 controls. 52.4% were female. The mean age (SD; range) was 50.2 (13.6; 23–83). The age was higher in patients (Pso and PsA) than in controls (p US measurements of IP and PB were significantly higher in patients than in controls in the majority of the nail (p Overall we found weak to moderate positive correlations between NAPSI and US scores for BM, both for matrix and bed. For most of the nails we found no correlation between NAPSI and CDUS scores; for the rest of the nails the correlation was weak, both positive and negative. References The MSUS measurements and scores showed to be higher in patients with PsA and Pso compared with controls, while CD scores showed no differences. Disclosure of Interest None declared

Background The development of biological therapies (BT) represented an important advancement in the treatment of patients with inflammatory arthritis. These treatments have proven to be effective in controlling disease activity, leading to clinical remission and avoiding disease progression, allowing a better prognosis of these patients. Objectives To describe the reasons of intra-venous BT interruption in a Rheumatology Day-Care Hospital. To evaluate whether this interruption of BT leads to an increased disease activity. Methods This is an observational, retrospective study performed in the Rheumatologic Day-Care Hospital of a UniversityHospital between June 2014 and December 2015. We included all patients treated with intra-venous BT [i.e. infliximab (IFX), tocilizumab (TCZ) and abatacept (ABA)] with a treatment interruption longer than 15 days. Demographics (i.e. gender, age), RA features (i.e. treatment, disease duration), clinical evaluation (i.e. tender joint count, swollen joint count, patient global pain intensity VAS) and laboratory tests (i.e. ESR, CRP) were recorded. Composite scores and indices (i.e. DAS28, CDAI, SDAI, ASAS, HAQ, BASDAI and BASFI) were calculated before and at BT re-starting. Results Out of 272 patients treated with BT, 55 (20.2%) had a temporary interruption of the treatment ≥15 days. Out of these patients, 56% were females, the mean (SD) age was 50 (32) years with a mean (SD) disease duration of 18.5 (18) years and a mean (SD) treatment duration of 8.1 (7.8) years. The diagnoses of these patients were as follows; 25 (45.4%) had rheumatoid arthritis (RA), 17 (30.9%) had spondyloarthritis (Sp), 8 (14.5%) had psoriatic arthritis (PsA), and 5 (9%) had other autoimmune disease. The BT received was as follows; 43 (78.1%) IFX, 8 (14.5%) TCZ and 4 (7.3%) ABA. Twenty two (40%) patients received also treatment with synthetic disease modifying drugs (sDMARDs) and 21 (38.1%) received also corticoid therapy with a mean dosage of 6.2 mg/day. The most frequent reasons for BT interruption were infections in 27 (49%) patients of which 11 required hospitalization (i.e. 8 pneumonias, 1 cholelithiasis, 1 lower urinary tract infection and 1 herpes zoster infection) and 16 didn't required hospitalization (i.e. 6 respiratory tract infections, 3 lower urinary tract infections, 1 herpes zoster infection, 1 cellulitis and 5 oral infections). The second reason for BT interruption was the performance of surgical procedures in 16 (29%) patients and the third reason was related to other situations such as the performance of diagnostic procedures for ruling out other diseases, work reasons or forgetfulness 12 (21.8%). In 3 patients the BT was interrupted because of a neoplasia (colon, lung and bladder adenocarcinoma) and 3 patients were pending to restart the treatment after ruling out cancer or infection. The mean (range) interruption duration was 72 (16–365) days. The disease activity before and after BT interruption is displayed in [table 1][1]. ![Figure][2] Conclusions The most frequent reasons for BT interruption were infections, in particular respiratory tract infections and surgical procedures. BT interruption occurred in a wide age range. After BT reintroduction, we detected an increase in pain and disease activity as well as a decrease in the functional status. Disclosure of Interest None declared [1]: #F1 [2]: pending:yes

Background Biologic therapies (BT) have been changed considerably the treatment of autoimmune and autoinflammatory rheumatic diseases. However, patients with joint prostheses may present adverse effects due to these therapies. Objectives To describe clinical features, treatment and complications of patients with joint prosthesis treated with BT. Methods We included all patients treated in our BT Unit from January 2010 to December 2014. For each patient we analysed demographic, diagnosis, treatments, number of prostheses and complications. Results Eight hundred patients were treated with TB in our TB Unit during the study period of which 77 (9.6%) patients needed joint prosthesis. The diagnosis of patients with joint prosthesis was as follows: 51 (66.2%) rheumatoid arthritis (RA), 16 (20.7%) ankylosing spondylitis (AS), 5 (6.5%) lupus erythematosus, 3 (3.9%) psoriatic arthritis and 2 (2.6%) juvenile idiopathic arthritis. We found 129 prostheses placed in the 77 patients, of which 56 (43.4%) were hip prostheses, 50 (38.7%) were knee prostheses, 5 (3.9%) were MCF prostheses, 4 (3.1%) were ankle prostheses, 3 (2.3%) were wrist prostheses, 2 (1.5%) were IF prostheses, 2 (1.5%) were elbow prostheses and 2 (1.5%) were shoulder prostheses. The mean age of prosthesis placing was 60 years (range 28-85) and the mean disease duration at the moment of prosthesis placing was 20 years. The most used BT were as follows: 28 (36.4%) infliximab, 20 (26%) rituximab, 16 (20.7%) tocilizumab, 9 (11.6%) abatacept and 4 (5.2%) etanercept. Thirty four (44.1%) patients had received more than one biological drug, 44 (57.1%) patients had concomitant synthetic disease-modifying anti-rheumatic drugs (sDMARDs) treatments and 41 (53.2%) patients had steroid treatment associated. We recorded 7 (5.4%) prosthetic infections in 7 (9.1%) patients of which 6 were diagnosed with RA and one with AS. From all infections, 2 occurred in prosthesis placed before the beginning of BT treatment and 5 occurred in prostheses placed during BT treatment. All patients with prosthesis infections were treated with concomitant sDMARDs and steroid treatment. Five patients needed prosthesis replacement for other reasons. Conclusions Most frequent prostheses were hip and knee prostheses. The majority of prosthetic infection occurred in patients with prosthetic placement during the BT treatment. All patients with prosthesis infections received also sDMARS and steroid treatments. Disclosure of Interest None declared

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 In the management of rheumatoid arthritis (RA) patients, the election of the drug and dosage needs to be personalized considering each particular subject conditions. Objectives Assessclinical characteristics, disease activity and concomitant treatment in patients with RA treated with diverse anti-TNF dosage strategies. Estimate annual associated costs (2011). Methods Cross-sectional study (2011) conducted at the Hospital Universitario Gregorio Maranόn Rheumatology Department. Inclusion criteria: patients diagnosed of RA (ACR 1987 revised criteria); attending routine follow-up visit; treated with anti-TNF: etanercept (ETN), adalimumab (ADA) or infliximab (IFX). Main variables were drug administered, dosage regimen, disease activity (DAS28) and concomitant treatment (DMARD). Anova test was using to compare groups. Doses where calculated based on percentage of dose, considering 100% the stardard dose in RA (ETN 50mg once weekly; ADA 40mg every other week; IFX 3 mg/kg/8 weeks). Escalated and reduced doses were defined as those higher and lower than standard doses respectively. Associated annual costs were estimated based on national pricing including tax. Results The study evaluated 195 patients (79% women, mean age 58.1). The distribution by anti-TNF used was n: 81 ETN, N: 56 ADA and n: 58 IFX. There were significant differences between years to first biologic from diagnosis (IFX:7.5, ETAN: 5.7 and ADA: 5.9, p:0.03) and % of patients with previous biologic drugs (IFX:,12.7, ETN: 36.7, ADA: 29.7, p:0.04) Dose regimens and estimated costs are shown in the Table. Regarding disease activity, 61.73% and 64.29% of patients were good responders (DAS 28 Conclusions Choosing a minimum effective dose is important to achieve the goal of effectiveness while reducing adverse events and costs. ETN seems to be associated with lower doses and less concomitant DMARDs while maintaining clinical control in RA patients. Disclosure of Interest None Declared