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BackgroundTocilizumab (TCZ) is the only biologic drug approved in giant cell arteritis (GCA), based in two clinical trials (CT) (1,2). CT included selected patients who may differ from those of clinical practice (CP). A high proportion of GCA patients treated with TCZ in CT had a newly diagnosed GCA, whereas in CP, most of them are refractory/recurrent GCA (3,4). Although in CT the efficacy of TCZ seems to be similar in patients with newly diagnosed GCA and in patients with refractory/recurrent GCA, in CP it is not documented.ObjectivesTo compare in CP, the effectiveness and safety of TCZ in newly diagnosed vs refractory/recurrent GCA.MethodsMulticentre observational study on 471 GCA patients treated with TCZ. GCA was diagnosed by: a) ACR criteria, and/or b) temporal artery biopsy, and/or c) imaging techniques. A comparative study between patients with newly diagnosed GCA (6 weeks) (according to GiACTA study definitions) (2). Sustained remission was based on EULAR definitions (5).ResultsThe 471 GCA patients were divided into 2 subgroups: a) newly diagnosed GCA (n=91) and b) refractory/recurrent GCA (n=380) (Table 1).Table 1.Main features of patients with newly diagnosed GCA and refractory/recurrent GCA treated with tocilizumab.Newly diagnosed GCA (n=91)Refractory/recurrent GCA (n=380)pBaseline characteristics at TCZ onset Age(years), mean±SD74.3±8.573.3±9.10.35 Sex, female/male (% female)60/31 (66)282/98 (74)0.11 Time from GCA diagnosis to TCZ onset (months), median [IQR]1 [0.5-1]10 [4-24]0.0001 ESR, mm 1st hour, median [IQR]46 [17.5-80.5]27 [10-50]0.02 CRP, mg/dL, median [IQR]2.1 [0.7-8.5]1.3 [0.4-2.8]0.13 Haemoglobin, g/dL, mean±SD12.3±1.512.7±1.50.03 Prednisone dose, mg/day, median [IQR]40 [21.2-50]15 [10-30]Effectiveness and Safety after TCZ onsetFollow-up, (months), median [IQR]15 [6-27.5]22 [11-37]0.004Relevant adverse events, n (%)23 (25)102 (27)0.54Relevant adverse events per 100 patients-year2015NSSerious infections, n (%)13 (14)53 (14)0.49Serious infections per 100 patients-year11.28NSMACES, n (%)0 (0)1 (0.3)-MACES per 100 patients-year00.2-Malignancies n (%)2 (2)3 (0.8)0.99Malignancies per 100 patients-year1.60.5NSAbbreviations: CRP: C-reactive protein;ESR: erythrocyte sedimentation rate;GCA: giant cell arteritis; IQR: interquartile range; IV: intravenous; MACEs: major adverse cardiovascular events; NS: non significant; SC: subcutaneous; SD: standard deviationNo significant differences were observed between both groups in sustained remission, although a greater tendency towards sustained remission is observed in newly diagnosed than in refractory/recurrent GCA patients (Figure 1). The decrease in glucocorticoids dose was faster in the first three months in the newly diagnosed GCA group, but thereafter, was similar in both groups, as well as the appearance of relevant adverse events and serious infections.Figure 1.A) Sustained remission, and B) median prednisone dose required in patients with newly diagnosed GCA and in patients with refractory/recurrent GCA treated with tocilizumab.ConclusionThe effectiveness and safety of TCZ seems to be similar in patients with newly diagnosed GCA and in patients with refractory/recurrent GCA.References[1]Villiger PM, et al. Lancet. 2016; 387:1921-1927. PMID: 26952547[2]Stone JH, et al. N Engl J Med. 2017; 377:317-328. PMID: 28745999[3]Calderón-Goercke M, et al. Semin Arthritis Rheum. 2019; 49: 126-135. PMID: 30655091[4]Calderón-Goercke M, et al. Clin Exp Rheumatol. 2020; 124: S112-119. PMID: 32441643[5]Hellmich B, et al. Ann Rheum Dis. 2020; 79: 19-30. PMID: 31270110AcknowledgementsTocilizumab 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, Clara Moriano: None declared, Santos Castañeda: None declared, J. Narváez: None declared, Vicente Aldasoro: None declared, Olga Maiz: None declared, Rafael Melero: None declared, Ignacio Villa-Blanco: None declared, Paloma Vela-Casasempere: None declared, Susana Romero-Yuste: None declared, Jose Luis Callejas-Rubio: None declared, Eugenio de Miguel: None declared, E. Galíndez-Agirregoikoa: None declared, Francisca Sivera: None declared, Carlos Fernández-López: None declared, Carles Galisteo: None declared, Iván Ferraz-Amaro: None declared, Lara Sanchez-Bilbao: None declared, Monica Calderón-Goercke: None declared, Jose Luis Hernández Hernández: 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

BackgroundLarge vessel involvement in Giant Cell Arteritis (GCA), especially the aorta and/or its main branches, is frequent. Tocilizumab (TCZ) has shown efficacy and safety in GCA and other large-vessel vasculitis (1-4).ObjectivesTo assess the efficacy and safety of TCZ in GCA patients with involvement of the aorta and/or its main branches.MethodsMulticenter observational study of 196 patients with GCA and involvement of the aorta and/or its major branches treated with TCZ. GCA was diagnosed by: a) ACR criteria, and/or b) temporal artery biopsy, and/or c) imaging techniques. The presence of aortitis was performed by imaging techniques, mainly PET, and A-MRI.Maintained remission was considered according to EULAR definitions (5).ResultsThe main features of the 196 patients are showed in Table 1. Polymyalgia rheumatica, constitutional syndrome and headache were the most frequent clinical manifestations at TCZ onset. At 6 months after starting TCZ, 20% of the patients reached a sustained remission, that was progressively increasing. (Figure 1). A corticosteroid-sparing effect was observed from month 1 of TCZ onset (Figure 1). Relevant adverse events were observed in 12 per 100 patients-year, documenting serious infections in 4.8 per 100 patients-year (Table 1).Table 1.Main features of 196 GCA patients with involvement of the aorta and/or its main branches treated with TCZ.GCA (n=196)Features at TCZ onsetAge(years), mean±SD71.3±9.5Sex, female/male (% female)148/48 (75)Time from GCA diagnosis to TCZ onset (months), median [IQR]7 [2-18.25]Systemic manifestations, n (%)Fever, n (%)24 (12)Constitutional syndrome, n (%)87 (44)PmR, n (%)131 (67)Ischaemic manifestations, n (%)Visual involvement, n (%)16 (8)Headache, n (%)74 (38)Jaw claudication, n (%)27 (14)Laboratory dataESR, mm 1st hour, median [IQR]32 [14-54]CRP, mg/dL, median [IQR]1.5 [0.6-3.2]Prednisone dose, mg/day, median [IQR]15 [10-30]Safety after TCZ onsetRelevant adverse events, per 100 patients-year12Serious infections, per 100 patients-year4.8Figure 1.A) Sustained remission, and B) median prednisone dose required in GCA patients with aortitis treated with tocilizumabConclusionTCZ seems to be effective and relatively safe in GCA patients with involvement of the aorta and/or its main branches.References[1]Calderón-Goercke M, et al. Semin Arthritis Rheum. 2019; 49: 126-135. PMID: 30655091[2]Loricera J, et al. Clin Exp Rheumatol. 2016; 34: S44-53. PMID: 27050507[3]Loricera J, et al. Clin Exp Rheumatol. 2015; 33: S19-31. PMID: 25437450[4]Prieto-Peña D, et al. Ther Adv Musculoskelet Dis. 2021; 13: 1759720X211020917. PMID: 34211589[5]Hellmich B, et al. Ann Rheum Dis. 2020; 79: 19-30. PMID: 31270110Disclosure of InterestsLara Sanchez-Bilbao: None declared, Javier Loricera Speakers bureau: from Roche, Novartis, UCB Pharma, Celgene, and Grünenthal., Rafael Melero: None declared, Santos Castañeda Speakers bureau: UAM-Roche, EPID- Future chair, Department of Medicine, Universidad Autónoma de Madrid, Madrid, Spain., Clara Moriano: None declared, Iván Ferraz-Amaro: None declared, J. Narváez: None declared, Vicente Aldasoro: None declared, Olga Maiz: None declared, Ignacio Villa-Blanco: None declared, Paloma Vela-Casasempere: None declared, Susana Romero-Yuste: None declared, Jose Luis Callejas-Rubio: None declared, Eugenio de Miguel: None declared, E. Galíndez-Agirregoikoa: None declared, Francisca Sivera: None declared, Carlos Fernández-López: None declared, Carles Galisteo: None declared, Julio Sanchez-Martin: None declared, Monica Calderón-Goercke: None declared, J. Luis Hernández: 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, Pfizer, Roche, Bristol-Myers, Lilly, Janssen, and MSD., Grant/research support from: Abbvie, MSD, and Roche

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

BackgroundEpidemiological information on Giant Cell Arteritis (GCA) comes mainly from the Scandinavian countries of northern Europe, which show a higher incidence than the countries of southern Europe. GCA clinical manifestations can be divided into cranial, extracranial, and general syndrome.ObjectivesIn a large series of GCA from Spain, we studied a) the incidence of GCA, b) clinical manifestations, and c) comorbidities at the time of disease diagnosis.MethodsARTESER is a retrospective epidemiological observational study of GCA promoted by the Spanish Society of Rheumatology in which 26 hospitals participate. The inclusion criteria were: all new patients diagnosed with GCA by a) ACR criteria, b) positive diagnostic test (temporal artery biopsy, temporal artery ultrasound or other relevant imaging techniques) and/or c) investigator’s clinical judgment. The patient recruitment period ranged from June 1, 2013 to March 29, 2019. The overall incidence of GCA per 100,000 people ≥50 years for the whole period and the mean annual incidence were evaluated. The clinical variables were collected by reviewing the patient’s medical history.Results1675 patients were included. The average annual incidence rate was 7.42 (95% CI: 6.57-8.27). All the cases were older than 50 years, and the age group with the highest annual incidence was that of 80 to 84 years, where it reached a value of 22.63 (95% CI: 22.04 -23.22). The mean annual incidence is higher in women than in men 10.07 (95% CI: 8.74-11.55) vs 4.81 (95% CI 3.84-5.93) (Table 1).Table 1.General characteristics, comorbidities and clinical manifestationsEpidemiologic, demographic and diagnosisMenWomenTotalGender, n (%)497 (29.7)1178 (70.3)1675Incidence annual rate (95% CI)4.81 (3.84-5.93)10.07 (8.74-11.55)7.42 (6.57-8.27)Age at diagnosis, years, mean (SD)76.9 (8.3)76.9 (8.0)76.9 (8.1)Diagnosis only by ACR Criteria89 (17.91)266 (22.58)355 (21.19)Diagnosis only with objective tests73 (14.69)140 (11.88)213 (12.72)Diagnosis ACR criteria + diagnosis objective tests311 (62.58)734 (62.31)1045 (62.39)Diagnosis by clinical judgment24 (4.8)38 (3.2)62 (3.7)Comorbidities at diagnosisArterial hypertension, n (%)330 (66.8)749 (63.7)1079 (64.6)Dyslipidemia, n (%)238 (48.3)563 (47.9)801 (48.0)Cranial clinical manifestationsNew-onset headache, n (%)382 (76.9)955 (81.1)1337 (79.9)Visual Clinic, n (%)194 (39.0)411 (34.9)605 (36.1)Extracranial manifestations and general syndromePolymyalgia rheumatica, n (%)178 (35.8)521 (44.3)699 (41.8)Asthenia, n (%)239 (48.1)634 (53.9)873 (52.2)Analysis at diagnosisErythrocyte sedimentation rate mm/h, mean (SD)72.3 (34.7)77.4 (33.0)75.9 (33.6)The principal clinical characteristics of the population is shown in Table 1, the mean age at diagnosis was 76.9±8.1 years, 1178 (70.3%) were women. 1045 patients (62.39%) had ACR criteria and some positive objective test, 355 patients (21.9%) presented only ACR criteria and 213 (12.72%) only had a positive diagnostic test; 62 (3.7%) of the patients underwent diagnosis based on clinical judgment. The more frequent comorbidity was arterial hypertension (n=1079; 64.6%), followed by dyslipidemia (n=801, 48%). The predominant cranial manifestation was headache (n= 1337; 79.9%) and 605 patients experienced visual symptoms (36.1%). Polymyalgia rheumatica (n=699; 41.8%) and asthenia (n=837; 52.2%) were the most frequent extracranial and general syndrome manifestation, respectively. Regarding laboratory parameters, the most characteristic data was the increase of ESR (75.9±33.6 mm/1st h).ConclusionThe mean annual incidence of GCA in Spain, 7.42 (95% CI: 6.57-8.27), is lower than that of the Scandinavian countries. It is higher in people older than 80 years. More than 60% of the patients met the ACR criteria and had a positive diagnostic test. Cranial manifestations constituted the most clinical features. The most frequent clinical manifestations are cranial. Up to a third of patients had visual manifestations.AcknowledgementsThis study has been funded by ROCHE Farma. The funder has not participated in the design, analysis, or interpretation of the resultsDisclosure of InterestsNone declared

BackgroundPositron emission tomography (PET) is one of the tools available for the diagnosis of extracranial large-vessel vasculitis (1-5). Tocilizumab (TCZ) has shown efficacy in large-vessel vasculitis (LVV) including GCA. However, the improvement objectified by imaging techniques after TCZ therapy in extracranial GCA patients is controversial.ObjectivesTo assess the effectiveness of TCZ improving the wall vessel inflammation by PET in GCA patients with large-vessel involvement.MethodsObservational, multicenter study of 101 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 PET, defined by the presence of vascular wall uptake of Fluorodeoxyglucose (FDG). Patients were divided into two subgroups: a) with, and b) without signs of improvement (partial or total) in the follow-up PET.ResultsWe studied 101 patients (74 women/27 men; mean age 69.7±9.3 years). Main clinical features of GCA with and without PET improvement are shown in Table 1. The group of patients which experienced PET improvement was older and was receiving higher doses of corticosteroids at TCZ onset.Table 1.Main features of 101 GCA patients treated with tocilizumab and with presence of signs of vessel wall inflammation by PET.With PET improvement (n=88)Without PET improvement (n=13)pBaseline characteristics at TCZ onsetGeneral characteristicsAge(years), mean±SD70.6±9.163.8±9.20.014Sex, female/male (% female)67/21(76)7/6 (54)0.103Time from GCA diagnosis to TCZ onset (months), median [IQR]11 [4-24.2]4 [2-6]0.102Systemic manifestations, n (%)Fever, n (%)5 (6)2 (15)0.225Constitutional syndrome, n (%)36 (41)4 (31)0.466PmR, n (%)53 (60)9 (10)0.761Ischaemic manifestations, n (%)Visual involvement, n (%)2 (2)1 (1)0.342Headache, n (%)30 (34)3 (23)0.538Jaw claudication, n (%)8 (9)0 (0)0.592Laboratory dataESR, mm 1st hour, median [IQR]38.0 ± 26.213.54 ± 9.90.001CRP, mg/dL, median [IQR]1.5 [0.7-2.4]1 [0.5-1.7]0.179Prednisone dose, mg/day, median [IQR]40.3 ± 19.421.9 ± 12.70.001Time from TCZ onset and follow-up PET (months)13.1±8.010.1±5.30.446ConclusionTCZ seems to be effective controlling GCA including vascular involvement detected by PET. However, the improvement observed by PET is most often partial, and rarely complete.Figure 1.Improvement by PET according to the time of the test.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, Santos Castañeda: None declared, Clara Moriano: None declared, J. Narváez: None declared, Vicente Aldasoro: None declared, Olga Maiz: None declared, Rafael Melero: None declared, Ignacio Villa-Blanco: None declared, Paloma Vela-Casasempere: None declared, Susana Romero-Yuste: None declared, Jose Luis Callejas-Rubio: None declared, Eugenio de Miguel: None declared, E. Galíndez-Agirregoikoa: None declared, Francisca Sivera: None declared, Carlos Fernández-López: None declared, Carles Galisteo: None declared, Iván Ferraz-Amaro: None declared, Lara Sanchez-Bilbao: None declared, Monica Calderón-Goercke: None declared, Jose Luis Hernández Hernández: 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

BackgroundTocilizumab (TCZ) has shown efficacy in large-vessel vasculitis, including Giant Cell Arteritis (GCA) (1-3). Clinical trials with TCZ in GCA was performed with intravenous (iv) TCZ in a phase 2 trial (3), and with subcutaneous (sc) TCZ in the phase 3 GiACTA (4). However, in GCA there are no studies comparing IV vs SC TCZ.ObjectivesTo compare the efficacy of TCZ in GCA patients according to the route of administration IV-TCZ vs SC-TCZ.MethodsMulticentre study of 471 patients diagnosed with GCA and treated with TCZ. They were divided into 2 groups according to the route of administration: a) IV, and b) SC. GCA was diagnosed by: a) ACR criteria, and/or b) temporal artery biopsy, and/or c) imaging techniques. Sustained remission was established according to EULAR definitions (5).ResultsWe studied 471 patients (mean age, 74±9 years) treated with TCZ, 238 with IV-TCZ and 233 with SC-TCZ (Table 1). The time between diagnosis of GCA and TCZ onset was shorter in the SC TCZ group. Regarding acute phase reactants at the beginning of TCZ, no differences were found between both groups. There were no significant differences in sustained remission or in glucocorticoid-sparing effect of TCZ (Figure 1). Patients on IV TCZ treatment suffered more relevant adverse effects during follow-up.Table 1.Main characteristics of GCA patients treated with intravenous and subcutaneous tocilizumabIV TCZ (n= 238)SC TCZ (n=233)PBaseline characteristics at TCZ onsetAge(years), mean±SD73.3±8.773.7±9.30.63Sex, female/male (% female)175/63 (73)167/66 (72)0.65Time from GCA diagnosis to TCZ onset (months), median [IQR]8 [3-23.5]5 [2-15]0.016ESR, mm 1st hour, median [IQR]30.5 [12.5-53]28 [10-56.5]0.66CRP, mg/dL, median [IQR]1.4 [0.5-2.8]1.4 [0.4-4]0.92Prednisone dose, mg/day, median [IQR]20 [10-40]20 [10-36.2]0.69Safety after TCZ onsetFollow-up, (months), median [IQR]27 [16-44]14 [6-26.7]Relevant adverse events, n (%)80 (34)46 (19)Relevant adverse events per 100 patients-year12.715.2NSSerious infections, n (%)44 (18)21 (9)0.44Serious infections per 100 patients-year6.77.2NSMACEs, n (%)/1 (0.4)0 (0)-MACEs per 100 patients-year0.10NSMalignancies, n (%)4 (1.7)1 (0.4)0.20Malignancies per 100 patients-year0.60.3NSAbbreviations: CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; GCA: giant cell arteritis; IQR: interquartile range; IV: intravenous; MACEs: major adverse cardiovascular events; NS: non significant; SC: subcutaneous; SD: standard deviationConclusionIn GCA, TCZ seems equally effective and safe regardless of the route of administration IV or SC.References[1]Calderón-Goercke M, et al. Semin Arthritis Rheum. 2019; 49: 126-135. PMID: 30655091[2]Prieto-Peña D, et al. Ther Adv Musculoskelet Dis. 2021; 13: 1759720X211020917. PMID: 34211589[3]Villiger PM, et al. Lancet. 2016; 387:1921-1927. PMID: 26952547[4]Stone JH, et al. N Engl J Med. 2017; 377:317-328. PMID: 28745999Hellmich B, et al. Ann Rheum Dis. 2020; 79: 19-30. PMID: 31270110Disclosure of InterestsNone declared

BackgroundVisual involvement is the most feared complication of Giant Cell Arteritis (GCA) (1-5). Permanent visual loss (PVL) may be preceded by transient visual loss. Once blindness is established, the prognosis is poor. Most of the series of predictive factors of visual involvement in GCA are old and with a small number of patients.ObjectivesTo assess the predictive factors of visual involvement and PVL in GCA.MethodsMulticenter observational study of 471 patients with GCA. The diagnosis of GCA was performed between 2016 and 2021 according to: a) ACR criteria, and/or b) temporal artery biopsy, and/or c) imaging techniques.From the 471 patients, we selected patients who developed a) visual involvement at any time during GCA and b) PVL. PVL was defined as partial or complete visual loss of >24 hours. Predictive factors were identified by multivariate analysis.ResultsVisual involvement was observed in 122 cases and PVL in 60 (Table 1). The ischemic and systemic manifestations set of variables associated with visual involvement were headache, and jaw claudication, whereas large-vessel involvement was a protective variable (Figure 1). The area under the curve (AUC) for the model was 0.72 (95%CI 0.67-0.77; pFigure 1.Forest plot of multivariate analysis.Table 1.Main features of the patientsOverall (n= 471)GCA without visual involvement (n=349)GCA with visual involvement (n= 122)GCA with PVL (n=60)P visual vs non visual involvementP PVL vs non visual involvementAge at diagnosis of GCA (mean±SD)72±971±975±875±90.0010.001Female/Male (% of female)342/129 (73)265/84 (76)77/45 (63)41/19 (68)0.0060.21Positive TAB, n (%)201 (43)146 (42)55 (45)33 (55)0.530.34Cardiovascular risk factorsHigh blood pressure, n (%)272 (58)189 (54)83 (68)40 (67)0.0130.058Dyslipidemia, n (%)241 (51)175 (50)66 (54)32 (53)0.610.63Diabetes, n (%)81 (17)50 (14)31 (25)16 (27)0.0070.016Previous or current smoking history, n (%)47 (10)31 (9)16 (13)8 (13)0.210.27CHADS2 score, median [IQR]1 [1-2]1 [0-2]2 [1-2]2 [1-2]0.0010.004Ischemic manifestationsHeadache, n (%)259 (55)167 (48)92 (75)42 (70)0.0000.002Jaw claudication, n (%)112 (24)63 (18)49 (40)26 (43)0.0000.000Systemic manifestationsFever, n (%)57 (12)47 (13)10 (8)4 (7)0.120.20Constitutional syndrome, n (%)175 (37)132 (38)43 (35)20 (33)0.550.47PmR, n (%)284 (60)218 (62)66 (54)29 (48)0.0940.022Large-vessel involvement, n (%)254 (54)211 (60)43 (35)20 (33)0.0000.000ESR, mm/1st hour, median [IQR]32 [12-57]30 [11-54]34 [15-67]42 [12-67]0.220.28CRP (mg/dL), median [IQR]1.5 [0.5-3.4]1.4 [0.5-3.0]1.5 [0.4-4.7]1.5 [0.4-3.6]0.0420.30In the same line, the set of variables associated with PVL were headache, and jaw claudication. By contrast, polymyalgia rheumatica (PmR), and large-vessel involvement were protective factors (Figure 1). The AUC for this model was 0.77 (95%CI 0.71-0.83; pConclusionHeadache, and jaw claudication seem to be associated with visual involvement in GCA, while large vessel involvement seems to be a protective factor. PmR also appears to be a protective factor for the development of PVL.References[1]Calderón-Goercke M, et al. Semin Arthritis Rheum. 2019; 49: 126-135. PMID: 30655091[2]Baalbaki H, et al. Clin Rheumatol. 2021; 40: 3207-3217. PMID: 33580374[3]González-Gay MA, et al. Arthritis Rheum. 1998; 41: 1497-1504. PMID: 9704651[4]Prieto-Peña D, et al. Semin Arthritis Rheum. 2019; 48: 720-727. PMID: 29903537[5]Martínez-Rodríguez I, et al. Semin Arthritis Rheum. 2018; 47: 530-537. PMID: 28967430AcknowledgementsTocilizumab in Giant Cell Arteritis Spanish Collaborative Group.Disclosure of InterestsLara Sanchez-Bilbao: None declared, Javier Loricera Speakers bureau: Roche, Novartis, UCB Pharma, Celgene, and Grünenthal, Clara Moriano: None declared, Santos Castañeda Speakers bureau: UAM-Roche, EPID- Future chair, Department of Medicine, Universidad Autónoma de Madrid, Madrid, Spain., Iván Ferraz-Amaro: None declared, J. Narváez: None declared, Vicente Aldasoro: None declared, Olga Maiz: None declared, Rafael Melero: None declared, Ignacio Villa-Blanco: None declared, Paloma Vela-Casasempere: None declared, Susana Romero-Yuste: None declared, Jose Luis Callejas-Rubio: None declared, Eugenio de Miguel: None declared, E. Galíndez-Agirregoikoa: None declared, Francisca Sivera: None declared, Carlos Fernández-López: None declared, Carles Galisteo: None declared, Julio Sanchez-Martin: None declared, Monica Calderón-Goercke: None declared, J. Luis Hernández: 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, Pfizer, Roche, Bristol-Myers, Lilly, Janssen, and MSD., Grant/research support from: Abbvie, MSD, and Roche

BackgroundTocilizumab (TCZ) has shown to be useful in the treatment of large-vessel vasculitis, including giant cell arteritis (GCA) (1-4). There is general agreement on the initial and the standard maintenance dose of TCZ. However, information on duration and optimization of TCZ in GCA is really scarce.ObjectivesOur aim was to assess the effectiveness and safety of TCZ therapy optimization in an unselected wide series of GCA in real-world clinical practice.MethodsMulticenter study on 471 patients with GCA who received TCZ therapy. Once complete remission was reached (n=231) TCZ was optimized in 125 patients. We compared patients in whom TCZ was optimized (TCZOPT group) or not (TCZNON-OPT group). Complete remission was defined as normalization of clinical and analytical (CRP and ESR) data. Optimization was done by decreasing the dose and/or prolonging the TCZ dosing interval progressively. We performed a comparison in effectiveness and safety parameters between optimized and non-optimized patients.ResultsWe evaluated 231 GCA patients treated with TCZ with complete remission. No demographic or laboratory data differences was observed at TCZ onset between both groups (Table 1). The mean prednisone dose was higher in the TCZNON-OPT group at TCZ onset. The first TCZ optimization was performed after a median [25-75th] follow-up of 12 [6-17] months.Table 1.Main general features at TCZ onset of 231 GCA patients with prolonged remission.OPTIMIZED-TCZ GROUP (n=125)NON-OPTIMIZED TCZ GROUP (n=106)pGENERAL FEATURES Age, years, mean± SD72.7±8.674±8.70.197 Sex, female/male n (% female)91/34 (72.8)74/32 (69.8)0.616 Time from GCA diagnosis to TCZ onset (months), median [IQR]8 [2-21.5]5 [2-21]0.384SYSTEMIC MANIFESTATIONS Fever, n (%)14 (11.2)15 (14.2)0.500 Constitutional syndrome, n (%)54 (43.2)39 (36.8)0.322 PMR, n (%)75 (60)69 (65.1)0.426ISCHEMIC MANIFESTATIONS Visual involvement, n (%)14 (11.2)16 (15.1)0.380 Headache, n (%)66 (52.8)62 (58.5)0.386 Jaw claudication, n (%)24 (19.2)25 (23.6)0.417AORTITIS (large-vessel involvement), n (%)65 (52)42 (39.6)0.060ANALYTICAL FINDINGS ESR, mm/1st hour, mean (SD)39.1±29.337.5±33.50.334 CRP, mg/dL mean (SD)2.6± 3.42.7± 40.305 Hemoglobin, g/dL, mean (SD)13.5±9.612.9±1.50.153GLUCOCORTICOIDS Prednisone dose, mg/d mean (SD)20.3±16.427±17.80.001Abbreviations: CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; GCA: giant cell arteritis; IQR: interquartile range; IV: intravenous; PMR: polymyalgia rheumatica; SC: subcutaneous; SD: standard deviation; TCZ: tocilizumab.The median prednisone dose at first TCZ optimization was 2.5 [0-5] mg/day. At the end of follow-up prolonged remission was observed in 78.2% of TCZOPT group compared with 66.7% in the TCZNON-OPT group (p= 0.001) (Figure 1). Seven (5.6%) of the 125 optimized cases relapsed. Serious adverse events were similar in both groups, while serious infections were more frequent in the TCZNON-OPT group (p=0.009).ConclusionOnce complete remission is reached in GCA patients under TCZ treatment, optimization of biologic may be performed. Based on our experience it could be performed by reducing the dose or by prolonging dosing interval of TCZ. It seems to be an effective and safe practice.References[1]Calderón-Goercke M, et al. Semin Arthritis Rheum. 2019; 49: 126-135. PMID: 30655091[2]Loricera J, et al. Clin Exp Rheumatol. 2016; 34: S44-53. PMID: 27050507[3]Prieto-Peña D, et al. Ther Adv Musculoskelet Dis. 2021; 13: 1759720X211020917. PMID: 34211589[4]Loricera J, et al. Int Immunopharmacol. 2015; 27: 213-9. PMID: 25828585Disclosure of InterestsCarmen Álvarez-Reguera: None declared, Monica Calderón-Goercke: None declared, J. Loricera: None declared, Clara Moriano: None declared, Santos Castañeda: None declared, J. Narváez: None declared, Vicente Aldasoro: None declared, Olga Maiz: None declared, Rafael Melero: None declared, Ignacio Villa-Blanco: None declared, Paloma Vela-Casasempere: None declared, Susana Romero-Yuste: None declared, Jose Luis Callejas-Rubio: None declared, Eugenio de Miguel: None declared, E. Galíndez-Agirregoikoa: None declared, Francisca Sivera: None declared, Carlos Fernández-López: None declared, Carles Galisteo: None declared, Iván Ferraz-Amaro: None declared, Julio Sanchez-Martin: None declared, Lara Sanchez-Bilbao: None declared, Jose Luis Hernández Hernández: None declared, Miguel Á. González-Gay Consultant of: Abbvie, Pfizer, Roche, Sanofi and MSD., Grant/research support from: Abbvie, MSD, Jansen and Roche., Ricardo Blanco Consultant of: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Lilly and MSD, Grant/research support from: Abbvie, MSD and Roche.

Background:Biosimilar drugs has supposed a huge savings in our health systems and there are data that conclude that they work as original molecules when we making these switchings. There are limited studies that analize these patients across the time (1-3).Objectives:Long term follow up of patients after switching from original etanercept to biosimilar drug.Methods:We reviewed 187 patients who started Etanercept original in Rheumatology department at Navarra Hospital Complex and their switch to Etanercept biosimilar with a follow-up of 18 months.Results:The switch was performed in 176 patients with rheumatoid arthritis (RA), spondyloarthritis (SpA) and psoriatic arthritis (PsA).At 18 months of follow-up 26,1% discontinued treatment; 13% at 6 months, 9,1% at 12 months and 4% patients at 18 months. The median time in etanercept original drug before switching was 52 months.There were 46 withdrawal patients with etanercept biosimilar: 34 inefficacy, 3 skin reaction, 2 malignancies (lymphoma and GIST), 1 injection site reaction, 1 headache, 1 diarrhea, 1 recurrent urinary infection, 1 heart failure and 2 deaths. 25 patients returned to Etanercept original but 3 did not achieve good response; all of them in RA group.Conclusion:In our series 50 % of withdrawal happened at 6 months of follow-up. 73,9% had a good response with etanercept biosimilar at 18 months. The main reason to stop biosimilar drug was inefficacy.Longer-term follow-up and greater number of patients are necessary to ratify these data.References:[1]Selmi C, et al. BENEFIT: real-world effectiveness of SB4 after transition from reference etanercept in rheumatoid arthritis and axial spondyloarthritis. Clin Exp Rheumatol. 2020 Jun 30. Epub ahead of print.[2]Glintborg B, et al. To switch or not to switch: results of a nationwide guideline of mandatory switching from originator to biosimilar etanercept. One-year treatment outcomes in 2061 patients with inflammatory arthritis from the DANBIO registry. Ann Rheum Dis. 2019 Feb;78(2):192-200.[3]Tweehuysen L, et al. Open-Label, Non-Mandatory Transitioning From Originator Etanercept to Biosimilar SB4: Six-Month Results From a Controlled Cohort Study. Arthritis Rheumatol. 2018 Sep;70(9):1408-1418.Table 1.WITHDRAWAL (months)BACK TO ENBREL (n)CONTINUEENBREL (n)TIME TO SWITCH (months)REASON FOR WITHDRAWALInefficacyA.event*CancerDeathRA2211853 14521PsA166653,515001SpA888455300TOTAL4625225234822*Infections (1), Heart failure (1), skin reaction (3), injection point reaction (1)RA: rheumatoid arthritis; PsA: psoriatic artrhritis; SpA: spondyloarthritisDisclosure of Interests:None declared

Background:The Remitting Seronegative Symmetrical Synovitis with Pitting Edema Syndrome (RS3PE) is a rare rheumatological disease, considered a benign process.Objectives:This study aims to describe its clinical features and serological markers, and also to analyze its possible association with neoplasms.Methods:An observational retrospective study was performed to assess demographic and clinical characteristics of patients diagnosed from RS3PE at a reference hospital amongst the Rheumatology and Internal Medicine departments, from 2010 to 2021.Results:Twenty-seven patients were included, with a mean age of 82.74 y.o. (IC95% 80.45-85.04; range 66 to 93), and a 51.85% proportion of males. Only 22.22% were from rural areas.All patients presented bilateral hand edema although some associated feet edema (40.74%) or morning stiffness (70.37%). Blood tests demonstrated anemia in 44.44% of patients. Inflammatory markers were elevated, such as C-Reactive Protein (29.23 mg/L, IC95% 18.17-40.29), erythrocyte-sedimentation rate (33.74 mm/hour, IC95% 24.22-43.26) and fibrinogen (531.6 mg/dL, IC95% 482.91-580.30). Only a few patients presented any autoimmune serological marker such as antinuclear antibodies (18.18%) or rheumatoid factor (8.70%).X-ray screening was realized to 22 patients. 14 showed of osteoarthritis radiologic presentation, 4 had radiological findings of chondrocalcinosis and one of them presented both. Only one patient had bone erosion.Malignancy screening was performed at diagnosis in only 29.63% of patients (all negative). During follow-up only two tumors were detected (mean accumulated follow-up: 40.37 months, IC95% 26.70-54.04; range 1 to 122) and there were adenocarcinoma primary neoplasms.All but one patient received low-dose corticosteroids, with a good and rapid response in all cases. Three patients received treatment with methotrexate (2) or leflunomide (1).Conclusion:RS3PE must be contemplated in elderly patients presenting with bilateral hand pitting edema and articular symptoms. No specific biomarkers have been described, but inflammatory reaction is often found in the absence of rheumatoid arthritis biomarkers. Rapid response to corticosteroids is prevalent. Only two neoplasms were detected during follow-up.References:[1]Paira S, Graf C, Roverano S, Rossini J. Remitting seronegative symmetrical synovitis with pitting oedema: a study of 12 cases. Clin Rheumatol. 2002 May;21(2):146-9. doi: 10.1007/pl00011218. PMID: 12086166.[2]Cobeta García JC, Martínez Burgui J. RS3PE syndrome or benign edematous polysynovitis in the elderly. Study of 8 cases. Rev Clin Esp. 1999 Dec;199(12):785-9. Spanish. PMID: 10687410.[3]Moreno Obregón F, Del Castillo Madrigal M, Díaz Narváez F, Pérez Delgado FJ. RS3PE syndrome with positive rheumatoid factor. Reumatol Clin. 2019 Nov-Dec;15(6):e168-e169. English, Spanish. doi: 10.1016/j.reuma.2017.11.009. Epub 2017 Dec 15. PMID: 29254743Disclosure of Interests:None declared

Background:With the arrival of biosimilar drugs and savings policies to make the health system sustainable, hospital managers have chosen to make changes from original molecules to biosimilar drugs.Objectives:This work aims to reflect what happens when making these switchings.Methods:We reviewed 235 patients who started Etanercept original in Rheumatology at Navarra Hospital Complex and Henares University Hospital and their switch to Etanercept biosimilar with a follow-up of 6 months.Results:The switch was performed in 174 patients with psoriatic arthritis (PsA), rheumatoid arthritis (RA), ankylosing spondylitis (AS), juvenile idiopathic arthritis, SAPHO and spondyloarthritis.9.8% discontinued treatment: 6 RA (8.1%), 5 PsA (9.8 %) and 6 AS (20.7%); all of them in the injection presentation. 12 patient stopped treatment due to ineffecacy, 2 due to reaction at the injection site, 2 due to diarrhea and 1 due to headache. Among 88.2% of patients who returned to Etanercept original, 28.6% did not achieve good response and had to change of treatment. The median time from Etanercept original beginning until the moment of switching was 54 (40-87) months.Conclusion:In our series, approximately 10% of switching patients failed after a 6-month of follow-up; when trying to return to Etanercept original 28.6% did not achieve response.The median persistence time in the original molecule and the percentage of failures observed in AS could be two conditions to consider before switching.A longer-term follow-up and a greater number of patients are necessary to ratify these data.Table 1DISEASESTOPPED CAUSEBACK TO Etanercept originalCONTINUE Etanercept originalTIME UNTIL SWITCH (meses)ASReaction at the injection siteYesYes28ASHeadacheYesYes54ASIneffecacyYesYes126RAIneffecacyYesNo25ASDiarrheaYesYes87RAIneffecacyYesYes37PsAIneffecacyNoNo43RAIneffecacyYesYes54PsAIneffecacyYesNo93PsAIneffecacyNoNo132PsAIneffecacyYesYes62RAIneffecacyYesYes142RAReaction at the injection siteYesYes51RAIneffecacyYesNo56PsAIneffecacyYesNo40ASIneffecacyYesYes26ASDiarrheaYesYes48Disclosure of Interests:Vicente Aldasoro Speakers bureau: Roche, Abbvie, MSD, UCB, Pfizer, Menarini, Grunenthal, Gebro, Novartis, Janssen, Javier Mendizabal: None declared, Sara Perez Garcia: None declared, Guillen Sada Urmeneta: None declared, Juliana Restrepo Vélez: None declared, Natividad del Val del Amo: None declared, Inmaculada Paniagua Zudaire: None declared, Ricardo Gutiérrez Polo: None declared, Loreto Horcada: None declared, Laura Garrido Courel: None declared, C. Fito-Manteca: None declared

Background Giant cell arteritis (GCA) can be refractory to corticosteroids1–3. Tocilizumab (TCZ) demonstrated to be effective in two short-term clinical trials. Objectives To assess efficacy of TCZ in refractory GCA or with side effects to corticosteroids in clinical practice. Methods Multicenter study on 134 patients with GCA in treatment with TCZ due to lack of efficacy and/or unacceptable adverse events of previous therapy. Results 134 patients (101 w/33 m); mean age of 73.0±8.8 years. Main clinical features at TCZ onset were: PMR (n=73), constitutional syndrome (n=31), headache (n=70), visual (n=28) and jaw (n=14) affection. Besides steroids, 98 patients also received immunosuppressive agents. table 1 shows evolution during follow-up period. After a median follow-up of 12 [3.7–24] months, it was observed a decrease in:a)CRP from 1.7 [0.4–3.2] to 0.1 [0.0–0.3] mg/dL b)ESR from 33 [14.5–61] to 42–9 mm/1st hour and c) Prednisone dose from 1510–30 to 5 [0–7.5] mg/day. Outcome of patients was:a)discontinuation of TCZ (n=15) due to sustained remission, b)dose reduction due to improvement (n=17) or side effects (n=11), c)withdrawal of TCZ because of side effects (n=12) and d)same dose that at onset (n=73). TCZ had to be discontinued due to: infections, haematological and cardiovascular alterations, neoplasms and heptic toxicity among the most frequent. Table 1 Conclusions TCZ leads to a rapid and maintained improvement in patients with refractory GCA and/or with unacceptable side effects related to corticosteroids. However, the risk of neutropenia and infection should be kept in mind. References [1] Loricera J, et al. Semin Arthritis Rheum2015;44:717–723. [2] Loricera J, et al. Clin Exp Rheumatol2014;32(3Suppl82):S79–89. [3] Loricera J, et al. Clin Exp Rheumatol2016; 34 (3 Suppl 97):S44–53. Disclosure of Interest None declared

Objectives To describe patients with Systemic Lupus Erythematosus (SLE) treated with Belimumab (BLM). Material and methods Review of patients with SLE treated with BLM from 2012 to 2017. Results We enrolled 18 patients, 16 women and 2 men. The median age at of SLE diagnose was 34.3 (IQR 27–45.7) years and the median age at BLM start was 2.2 (IQR 38.9–47) years. The received sDMARDs median number was 2.5 (IQR 2–4). Before starting BLM 7 patients had received rituximab, 4 cyclophosphamide, 1 abatacept and 1 one efalizumab. At BLM begin 17 patients were taking prednisone, 13 hydroxycloroquine, 7 methotrexate, 1 azatioprine, 1 mycophenolate and 1 leflunomide (table 1). The median BLM bolus received was 8.5 (IQR 2–32.7) and it was stopped in 4 patients due to lack of efficacy; this 4 patients had persistence of arthritis and non of them had received bDMARD before. 13 patients had at least one antiphospholipid autoantibody positive and 4 developed antiphospholipid syndrome. The median titer of antinuclear antibodies (ANA) was 1/360 (IQR 1/160–1/1280). All patients but one had ANA positive; among them 9 patients were antiRo positive, 4 antiLa, 4 antiSm and 4 antiRNP positive antibodies. 9 patients were antiDNA positive and 5 were positive for rheumatoid factor (RF) able 1 shows SLEDAI, C3 and C4, antiDNA levels. At BLMin 5 patients had renal involvement; 3 of them improved after 3 months of treatment. Among 14 patients with joint involvement, 3 improved by month 3, 2 by month 6 and 1 by month 7 after treatment beginning. 3 patients had heart and lung disease; no one of them improved with BLM. Among 9 patients with skin disease only one showed improvement after 12 months of treatment. 9 patients had haematological disorders: 1 improved by month one, 1 by month 3, one by month 5 and 2 my month 6 of treatment. Conclusion In this series BLM improved SLEDAI and complement levels allowing sparing of maintenance corticosteroid in SLE patients.

Resumen Caso clinico Paciente diagnosticada de escleritis necrotizante c-ANCA+, seudotumor orbitario y posible esclerosis multiple; en 2003 realizo tratamiento con ciclofosfamida oral y esteroides con respuesta parcial. Entre 2005-2010 sufrio episodios oculares autolimitados. En 2010 se realizo un primer trasplante escleral, con mala evolucion. Se inicio tratamiento con rituximab y se realizo segundo injerto con buena evolucion. A los 12 meses no se observaron cambios en resonancia magnetica y el segundo injerto cicatrizo. Discusion La enfermedad de Wegener con afectacion limitada a la orbita y/o el ojo es una entidad poco frecuente. La anatomia patologica, analitica de sangre, clinica y buena respuesta al tratamiento son clave para su diagnostico.

Case report A patient diagnosed with necrotizing scleritis, c-ANCA+, an orbital pseudotumour, and possible multiple sclerosis in 2003 was treated with oral cyclophosphamide and steroids with partial response. Between 2005 and 2010 she suffered self-limited episodes. In 2010 a first scleral transplant was performed with poor outcome. She was treated with rituximab, and a second graft was performed with good results. At 12 months there was no change in magnetic resonance and the second graft healed. Discussion Wegener's disease with limited involvement of the orbit and/or the eye is a rare condition. The histopathology, blood analysis, symptoms and good response to treatment are the key to its diagnosis.

Background Non-infectious aortitis associated with polymyalgia rheumatica (PMR) is a rare entity (1). Sometimes the conventional immunosuppressive therapy is not enough, forcing us to find new therapeutic options (2,3). We present our experience in a local hospital. Objectives To evaluate symptoms, clinic, diagnosis and therapeutic options. Methods During the years 2010–2015 we search for patients diagnosed with PMR which at the same time were diagnosed with non infectious aortitis. Their medical records were reviewed and the following data were collected: initial clinical diagnosis, diagnosis method used, time elapsed since the onset of symptoms to diagnosis, ESR and CRP at diagnosis and after the use of treatment. Results 5 patients were found: 4 women and 1 man. The average age onset of initial symptoms was 67.2 (74–58) years and the average delay in the diagnosis of the disease was 26 (1–50) months. The most common onset clinic was classic PMR symptom. Patients 2, 3, 4 and 5 also showed parietooccipital or parietal headache. Other symptoms were lower limb claudication, temporomandibular joint pain, dyspnea, fatigue and pseudofibromyalgia. All patients underwent temporal artery biopsy. In patient 5 bilateral temporal artery biopsy was performed. The definitive diagnosis test was PET (4). In patient 2 PET was compatible with aortitis being angioMRI negative. The average ESR at the beginning and after the treatment was 87.4 (114–65) and 10.2 (15.6) respectively. The initial and final average CRP (mg /dL) was 8.03 (14.54–1.86) and 1.2 (3 to 0.5) respectively. All patients underwent autoimmune analytical battery, being this study negative. 1,2,3 and 4 patients performed concomitant weekly methotrexate (MTX) with maximum dose of 25 mg weekly sc. Patients 1 and 5 made concomitant treatment with steroids and azathioprine at doses of 100 and 150 mg daily, respectively. Patients 1,2,3 and 5 were treated with tozilizumab which was well tolerated and without adverse effects to date. The average time from diagnosis of the disease until the start of treatment was 9.75 months (2,3). Patient 4 at the time of publication of these data remained asymptomatic with weekly MTX 15 mg sc and 5 mg of prednisone. Conclusions Non-infectious aortitis associated with PMR may go unnoticed due to high variability of presentation. This can make, sometimes, a delayed diagnosis (1). PET seems to be a good tool for the diagnosis (4) and tozilizumab seems to be a suitable treatment for those patients refractory to steroids and DMARDs (2,3). More patients are needed to generalize these data. References Non-infectious aortitis: a report of 32 cases from a single tertiary centre in a 4-year period and literature review. Loricera J, Blanco R, et al. Clin Exp Rheumatol. 2015 Mar-Apr;33(2 Suppl 89):S-19–31. Tocilizumab in refractory aortitis: study on 16 patients and literature review. Loricera J, Blanco R, et al. Clin Exp Rheumatol. 2014 May-Jun;32(3 Suppl 82):S79–89. Biologic therapy in ANCA-negative vasculitis. Loricera J, Blanco R, et al. Int Immunopharmacol. 2015 Aug;27(2):213–9. (18)F-FDG PET/CT for the detection of large vessel vasculitis in patients with polymyalgia rheumatica. Lavado-Perez C, Martinez-Rodriguez I, et al. Rev Esp Med Nucl Imagen Mol. 2015 Sep-Oct;34(5):275–81. Disclosure of Interest None declared

A patient diagnosed with necrotizing scleritis, c-ANCA+ an orbital pseudotumour, and possible multiple sclerosis in 2003 was treated with oral cyclophosphamide and steroids with partial response. Between 2005-2010 she suffered self-limited episodes. In 2010 a first scleral transplant was performed with poor outcome. She was treated with rituximab, and a second graft was performed with good results. At 12 months there was no change in magnetic resonance and the second graft healed.Wegener's disease with limited involvement of the orbit and/or the eye is a rare condition. The histopathology, blood analysis, symptoms and good response to treatment are the key to its diagnosis.

Background Secondary macrophage activation syndrome (MAS) is a group of diseases, especially due to autoimmune (AI) and hemato-oncology (HO). It would be interesting to find any clinical and analytics data to differentiate both etiologies. Objectives To describe and compare demographics, clinical and laboratories features and mortality of patients diagnosed with secondary MAS due to HO and AI diseases at the Donostia University Hospital. Methods A cohort of patients diagnosed with MAS was studied by reviewing medical reports in the period Dec/2008-Jan/2015. We analyzed and compared only patients with AI and HO diseases. The variable studied were diagnosis, age, sex, fever, organomegaly, hospital and overall mortality, analytical findings, hospital stay, days from admission to diagnosis and days from diagnosis to the end of the study or death after discharge. Quantitative variables are shown with the median and interquartile range. For the bivariate analysis Wilcoxon and Chi square test were used. Median survival in months and Hazar Ratio (HR) was calculated with the Kaplan Meier plot. Results Nineteen patients were found diagnosed with MAS, 6 and 9 were due to AI and HO diseases respectively. The AI diseases found were: 3 Systemic Lupus Erythematosus, 2 Adult Still9s Disease and 1 disease associated with IgG4. HO diseases found were: 3 acute myeloid leukemias, 2 B cells Not Hogkin Lymphoma (NHL), 1 T anb B cells NHL, 1 Natural Killers cells extranodal lymphoma, 1 myelodysplastic syndrome and 1 gastric plasmacytoma. The table shows the descriptive analysis of 19 patients and the bivariate analysis between AI and HO. The median survival of HO diseases was 1.2 months and AI diseases can not be calculated. The mortality of HO and AI diseases was 77.8% and 16.7% respectively with a p=0.04 in the log rank test. The HR of mortality among HO against AI diseases was 6.84. Conclusions In the present study the following differences were found: 1) Patients with MAS due to AI diseases have a higher elevated liver enzymes compared to HO disease. 2) Patients with MAS due to HO diseases have a greater pancytopenia compared to AI diseases, especially in leukocytes and neutrophils. 3) Patients with MAS due to HO have a higher mortality compared to AI diseases. Disclosure of Interest None declared

Background Hemophagocytic Lymphohistiocytosis (HLH) is a severe and acute inflammatory syndrome, underdiagnosed, difficult to treat and can occur at any stage of life. The macrophage activation syndrome is a variant of secondary HLH occurs in autoimmune diseases.1 Objectives Describe the clinical, laboratory, treatment and outcome of patients diagnosed with secondary HLH, identifying probable etiological and triggers causes. Methods A retrospective study was performed between 2008 and 2012 at the Donostia University Hospital, Spain. Inclusion criteria were to met diagnostic criteria for HLH and had a bone marrow biopsy with hemophagocytics cells. Mains endpoints were: demographics, diagnostic criteria, probable etiology, triggers causes and treatments. Secondary endpoints were: time delay in diagnosis, days of hospitalization, need for admission to the Intensive Care Unit (I.C.U.) and the cause, and overall mortality. Results We recruited 11 patients (7 men and 4 women) with a mean age of 48.9 years (16 - 78 years). The below table describes the probable etiologies and triggers of secondary HLH. As no data in the literature described, we found as etiologies and triggers causes of secondary HLH: Campylobacter jejuni in a previously healthy patient without comorbidities; Pneumocystis jirovecii in a patient with Human immunodeficiency virus (H.I.V.); and patient with grade IV glioblastoma multiforme after starting chemotherapy with temozolomide. Hemophagocytics cells were found in ascitic fluid in one patient with Adult Still´s Disease. The mean delay in diagnosis was 14.5 days (3 – 31 days) and average time of hospitalization was 46.7 days (10 – 130 days). The 45% of patient required admission in I.C.U, the leading cause was the multiple organ failure (MOF). The overall mortality rate was 36.4% (4 MOF and 1 massive hemoptysis). Treatment given was steroids, synthetic immunosuppressants and biological drugs. Conclusions The secondary HLH should be suspected in any patient with prolonged fever unresponsive to broad-spectrum antibiotics, hepatosplenomegaly, cytopenias, coagulation and liver disorders. Hemophagocytics cells might be found in pathological body fluids before a bone marrow biopsy had been done or when there are doubts in the result of it. Meet all the diagnostic criteria is not necessary to start treatment when you have a high clinical suspicion and a bone marrow biopsy with hemophagocytics cells. Mortality can be influenced by etiology, trigger cause, and diagnosis and treatment delay. References Henter JI, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48:124. Disclosure of Interest None Declared