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Tocilizumab (TCZ) is the only biologic therapy approved for giant cell arteritis (GCA). There is general agreement on the initial/maintenance dose, duration of TCZ therapy is not well established. In GiACTA trial, after one year on TCZ, most patients had GCA relapse after withdrawal. The aim of this study is to assess the effectiveness and safety of TCZ therapy optimisation in a large unselected series of patients with GCA in a clinical practice scenario.Multicentre study on 471 GCA patients treated with TCZ. Once prolonged remission was achieved (n=231) and based on a decision between patient and physician, TCZ was optimised (n=125). We compared optimised (TCZOPT) and not optimised (TCZNON-OPT) groups. Prolonged remission defined as normalisation of clinical and laboratory data for 6 months. Optimisation was carried out by decreasing TCZ dose and/or increasing dosing interval.We evaluated 231 GCA patients on TCZ in prolonged remission. At TCZ onset, no differences in demographic, clinical, or laboratory data were observed. First TCZ optimisation was performed after a median follow-up of 12[6-17] months. Intravenous TCZ was optimised from 8 to 4mg/kg/4weeks in 44% patients, while subcutaneous TCZ was optimised from 162mg/w to 162mg/every-other-week in 65% cases. At the end of follow-up, prolonged remission (78.2% vs. 84.2%; p=0.29) and relapses (5.6% vs. 10.4%, p=0.177) were similar in TCZOPT vs. TCZNON-OPT. Severe infections were more frequent in TCZNON-OPT (12.9% vs. 6.6%; p=0.009).TCZ optimisation may be done once complete remission is achieved by reducing dose or increasing dosing interval. This seems to be effective, safe and cost-effective therapeutic scheme.

With the arrival of the SARS-CoV-2 pandemic in 2020, it was proposed to make the change from intravenous (IV) tocilizumab (TCZ) to its subcutaneous formulation, in order to avoid rheumatological patients having to go to the day hospital and guarantee enough IV TCZ for those critical patients with COVID who needed it. The aim of this study was to describe the rate and reasons for switching back to IV TCZ from subcutaneous TCZ.We included patients from the rheumatology service that were on treatment with IV TCZ in February 2020 and were followed up until March 2021. Patients that remained on subcutaneous TCZ were compared with those who switched back to IV TCZ (switch-back group). A subgroup analysis according to rheumatic disease was performed.Fifty-five patients switched to subcutaneous TCZ: 28 rheumatoid arthritis, 19 giant cell arteritis, 4 polymyalgia rheumatica, 2 juvenile idiopathic arthritis, and 2 systemic sclerosis. Seventeen patients switched back to IV TCZ due to ineffectiveness (n = 8), patient preference (n = 4), adverse events (n = 4), and difficulty with the SC administration route (n = 1). In the analysis by disease, 4 of 23 patients switched back to IV TCZ in giant cell arteritis/polymyalgia rheumatica group due to ineffectiveness (n = 2), injection site reaction (n = 1), or patient preference (n = 1). In rheumatoid arthritis group, 11 of 28 patients switched back to IV TCZ: ineffectiveness (n = 5), patient preference (n = 3), headache (n = 1), injection site reaction (n = 1), and due to difficulty with the SC administration route (n = 1).Mass switch from IV to subcutaneous TCZ during the SARS-CoV-2 pandemic has been safe, effective, and well tolerated after 1 year of follow-up.

Visual involvement is the most feared complication of giant cell arteritis (GCA). Information on the efficacy of tocilizumab (TCZ) for this complication is scarce and controversial. We assessed a wide series of GCA treated with TCZ, to evaluate its role in the prevention of new visual complications and its efficacy when this manifestation was already present before the initiation of TCZ. This is an observational multicenter study of patients with GCA treated with TCZ. Patients were divided into two subgroups according to the presence or absence of visual involvement before TCZ onset. Visual manifestations were classified into the following categories: transient visual loss (TVL), permanent visual loss (PVL), diplopia, and blurred vision. Four hundred seventy-one GCA patients (mean age, 74±9years) were treated with TCZ. Visual manifestations were observed in 122 cases (26%), of which 81 were present at TCZ onset: PVL (n=60; unilateral/bilateral: 48/12), TVL (n=17; unilateral/bilateral: 11/6), diplopia (n=2), and blurred vision (n=2). None of the patients without previous visual involvement or with TVL had new episodes after initiation of TCZ, while only 11 out of 60 (18%) patients with PVL experienced some improvement. The two patients with diplopia and one of the two patients with blurred vision improved. TCZ may have a protective effect against the development of visual complications or new episodes of TVL in GCA. However, once PVL was established, only a few patients improved., The authors disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: This work was partially supported by RETICS Program (RD16/0012/0009) (Instituto de Salud Carlos III, co-funded by the European Regional Development Fund) from ‘Instituto de Salud Carlos III’ (ISCIII) (Spain).

Objective To compare the efficacy and safety of TCZ in monotherapy (TCZMONO) vs. combined with conventional immunosuppressive drugs (TCZCOMBO) in Giant Cell Arteritis (GCA) in a clinical practice scenario. Methods Multicenter study of 134 patients with refractory GCA. Patients on TCZMONO (n = 82) were compared with those on TCZCOMBO (n = 52). Drugs were methotrexate (MTX) (n = 48), azathioprine (n = 3), and leflunomide (n = 1). The main outcomes were: prolonged remission (normalization of clinical and laboratory parameters for at least 6 months) and the number of relapses. Results Patients on TCZCOMBO were younger (68.8 ± 8.0 vs 71.2 ± 9.0 years; p = 0.04), with a trend to a longer GCA duration (median [IQR],18.5 [6.25–34.0] vs. 13.0 [7.75–33.5] months; p = 0.333), higher C-reactive protein (CRP) levels (2.1[1–4.7] vs 1.2 [0.2–2.4] mg/dL; p = 0.003), and more prevalence of extra-cranial large-vessel vasculitis (LVV) (57% vs. 34.1%; p = 0.007). In both groups, rapid and sustained improvement was observed. Despite the longer GCA duration, and the higher CRP levels and prevalence of LVV in the TCZCOMBO, the improvement was similar in both groups at 12 months. Moreover, in the TCZCOMBO group, prolonged remission was significantly higher at 12-month. Relapses and serious adverse events were similar in both groups. Conclusion In clinical practice, TCZ in monotherapy or combined with conventional immunosuppressive agents is effective and safe in patients with GCA. Nevertheless, the addition of immunosuppressive drugs, usually MTX, seems to allow a higher rate of prolonged remission, even in patients with a longer GCA duration, more extra-cranial LVV involvement, and higher acute-phase reactants.

Resumen Objetivos Describir pacientes con aortitis no infecciosa y su respuesta al tratamiento de un hospital comarcal. Metodos Revision de pacientes con aortitis no infecciosa, tecnica diagnostica utilizada y tratamiento inmunosupresor recibido. Resultados Se encontraron 8 pacientes (7 mujeres y un hombre) diagnosticados de aortitis mediante tomografia por emision de positrones (PET). La mediana de edad fue de 69 anos (IQR 62-72,2). Tocilizumab (TCZ) mejoro los sintomas, la VSG y la PCR a los 3 meses del tratamiento (p Conclusion TCZ fue eficaz y seguro en los pacientes que fueron refractarios a esteroides y a otros tratamientos inmunosupresores.

Background:Visual involvement is one of the most feared complication of Giant Cell Arteritis (GCA).Tocilizumab (TCZ) has shown efficacy and safety in large-vessel vasculitis (LVV) including GCA (1-4).Objectives:To assess the efficacy of TCZ to: a) prevent the appearance of new ocular involvement, and b) to improve visual symptoms if present.Methods:Observational, multicenter study of 312 patients with GCA treated with TCZ. Patients were diagnosed with GCA accordingly to a) ACR criteria, and/or b) biopsy of temporal artery, and/or c) presence of LVV by imaging.Patients were divided into two subgroups: a) with, and b) without visual involvement at any time. Visual manifestations were classified as: a) Transient visual loss (TVL) (amaurosis fugax), b) Permanent visual loss (PVL) (longer than 24 hours) (partial or complete; unilateral or bilateral), c) diplopia, and d) blurred vision. Accordingly to visual duration up to TCZ onset, we considered: a) 1-10 days, b) 11-30 days, and c) more than 30 days.Results:We studied 312 (218 women/94 men; mean age73.4±9.6 years). Visual manifestations at any time (before and/or after TCZ) were observed in 78 (25%). In 47 of them visual manifestations were present at TCZ onset, and in the remaining 31 patients had had a complete recovery. Main clinical features of GCA with and without visual involvement are shown in TABLE. Patients with visual involvement were older, with other ischemic complications, and requiring more corticosteroids dose.Table 1.Main features of 312 patients at TCZ onset.OverallN= 312GCA with visual involvement(n= 78)GCA without visual involvement(n=234)pGeneral featuresAge (mean±SD)73.4±9.676.6±8.072.4±9.80.001*Female/Male(% of female), n218/94 (70)47/31 (60)171/63(73)0.046*Time from GCA diagnosis to TCZ onset (months), median [IQR]8 [3-24]5 [1-14]10 [3-24]0.040*Positive TAB, n (%)137/229 (60)33/60 (55)104/169 (61)0.444Ischemic manifestationsVisual involvement, n (%)47 (15)47 (60)00.000*Headache, n (%)166 (53)59 (76)107 (46)0.000*Jaw claudication60 (19)26 (33)34 (14)0.001*Systemic manifestationsFever, n (%)27 (9)8 (10)19 (8)0.743Constitutional syndrome, n (%)115 (37)30 (38)85 (36)0.878PmR, n (%)188 (60)46 (59)142 (61)0.830Acute phase reactantsESR, mm/1st hour, median [IQR]27 [10-50]34.5 [15.2-58]26.0 [10.0-48.0]0.193CRP (mg/dL), median [IQR]1.4 [0.4-3.3]1.5 [0.3-5.5]1.3 [0.4-2.9]0.134Prednisone dose, mg/day, mean±SD22.3±16.627.1±18.620.8±15.60.008*TCZmono/TCZcombo, n (%)211/10157/21154/800.295Follow-up (months), mean±SD28.4±21.825.8±22.429.3±21.60.119After TCZ onset, none patient developed new visual involvement. At TCZ onset 47 patients had the following visual manifestations; PVL (n= 28; unilateral/bilateral; 22/6), TVL (n=15; unilateral/bilateral; 9/6), diplopia (n=2) and blurred vision (n=2).None of the patients with TVL presented new episodes after TCZ onset, while 8 out of 28 patients with PVL experienced partial improvement (FIGURE). The 2 patients with diplopia and 1 of 2 patients with blurred vision improved.Figure 1.Efficacy of TCZ in 47 patients with GCA and visual involvement at TCZ onset.Conclusion:TCZ seems to prevent the appearance of new ocular manifestations. When they are present, TCZ may improve totally TVL and partially PVL.References:[1]Stone JH, et al. N Engl J Med. 2017; 377: 317-28.[2]Calderón-Goercke M, et al. Semin Arthritis Rheum 2019;49:126-35. https://doi.org/10.1016/j.semarthrit.2019.01.003.[3]Prieto Peña D et al. Clin Exp Rheumatol 2020 Nov 27. PMID: 33253103.[4]Loricera J, et al. Clin Exp Rheumatol 2016; 34:S44-53. PMID: 27050507.Disclosure of Interests:Lara Sanchez-Bilbao: None declared, Javier Loricera: None declared, Vicente Aldasoro: None declared, Rafael Melero: None declared, Santos Castañeda: None declared, Olga Maiz: None declared, Clara Moriano: None declared, Ignacio Villa-Blanco: None declared, Eztizen Labrador-Sánchez: None declared, Cristina Hidalgo: None declared, Sara Manrique Arija: None declared, Eva Galíndez-Agirregoikoa: None declared, Eva Perez-Pampín: None declared, Andrea García-Valle: None declared, Cristina Campos Fernández: None declared, Juan Ramón De Dios: None declared, Carlota Laura Iñíguez: None declared, José Luis Andréu Sánchez: None declared, Julio Sánchez: None declared, Monica Calderón-Goercke: None declared, Miguel A González-Gay Speakers bureau: Abbvie, Pfizer, Roche, Sanofi, Lilly, Celgene and MSD, Consultant of: 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, Consultant of: Abbvie, Lilly, Pfizer, Roche, Bristol-Myers, Janssen, UCB Pharma and MSD, Grant/research support from: Abbvie, MSD and Roche

Background Tocilizumab (TCZ) has been approved for the treatment of Giant Cell Arteritis (GCA). It showed to be effective to induce remission, prevent relapses and decrease the cumulative prednisone dose. However, the glucocorticoids are the mainstay in the acute treatment of GCA. Objectives Our aim was to compare the efficacy and safety of the initial dose of prednisone at the onset of TCZ treatment. Methods Retrospective, multicenter study on 134 patients with GCA in treatment with TCZ. We compared two subgroups of patients according to the initial dose of prednisone at TCZ onset. Clinical efficacy, analytical improvement and safety was studied. Results We studied 134 patients (101 w/33 m) and made a comparative study between 2 groups: a) TCZ and ≤ 15 mg of prednisone; 68 (50.7%) cases, and b) TCZ and > 15 mg of prednisone, 66 (49,3%) patients. It is summarized in TABLE 1. It was no statistical significance according to age, sex and evolution time of disease. In the group receiving > 15 mg of prednisone, the patients presented more visual involvement (p 15 mg of prednisone (p=0.001 and p=0.006, respectively). TABLE 2 summarizes the infections of our patients. Conclusion According with our results, we can conclude that TCZ is equally effective; in terms of prolonged remission and relapses, with doses ≤ 15 mg of prednisone at treatment onset. Being the most important data, the higher risk to develop adverse effects, as well as infections with higher doses of prednisone. References [1] Proven A. Glucocorticoid therapy in giant cell arteritis: duration and adverse outcomes. Arthritis Rheum. 2003; 49:703-8. [2] Calderon-Goercke M. Tocilizumab in giant cell arteritis. Observational, open-label multicenter study of 134 patients in clinical practice. Semin Arthritis Rheum. 2019 Jan 5. pii: S0049-0172(18)30571-7. doi: 10.1016/j.semarthrit.2019.01.003. [Epub ahead of print] Disclosure of Interests Monica Calderon-Goercke: None declared, J. Loricera: None declared, D. Prieto-Pena: None declared, Vicente Aldasoro: None declared, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Ignacio Villa-Blanco: None declared, Alicia Humbria: None declared, Clara Moriano: None declared, Susana Romero-Yuste: None declared, J. Narvaez Consultant for: Bristol-Myers Squibb, Catalina Gomez-Arango: None declared, Eva Perez-Pampin: None declared, Rafael Melero: None declared, Elena Becerra-Fernandez: None declared, Marcelino Revenga: None declared, Noelia Alvarez-Rivas: None declared, Carles Galisteo: None declared, Francisca Sivera: None declared, Alejandro Olive: None declared, Maria Alvarez del Buergo: None declared, Luisa Marena Rojas: None declared, Carlos Fernandez-Lopez: None declared, Francisco Navarro: None declared, Enrique Raya: None declared, Eva Galindez: None declared, Beatriz Arca: None declared, Roser Solans-Laque: None declared, Arantxa Conesa: None declared, Cristina Hidalgo: None declared, Carlos Vazquez: None declared, Jose Andres Roman-Ivorra: None declared, Pau Lluch: None declared, Sara Manrique Arija Speakers bureau: ABBvie, MSD, Janssen, Lillly, Roche, Pfyzer, Novartis., Paloma Vela-Casasempere Grant/research support from: UCB, Abbvie, Pfizer, Roche, Bristol-Myer-Squibb (another research, not BIOBADASER related), Consultant for: UCB, Lilly, Pfizer, Roche, Bristol-Myer-Squibb, Speakers bureau: Roche, UCB, MSD, Pfizer, GSK, BMS, Lilly, Eugenio de Miguel: None declared, Carmen Torres-Martin: None declared, Juan Carlos Nieto: None declared, Carmen Ordas-Calvo: None declared, Eva Salgado-Perez: None declared, Cristina Luna-Gomez: None declared, Francisco J. Toyos Saenz de Miera: None declared, Nagore Fernandez-Llanio: None declared, Antonio Garcia: None declared, Carmen Larena: None declared, Natalia Palmou-Fontana: None declared, Vanesa Calvo-Rio: None declared, Carmen Gonzalez-Vela: None declared, Alfonso Corrales: None declared, Maria Varela-Garcia: None declared, Elena Aurrecoechea: None declared, Raquel Dos-Santos: None declared, Angel Garcia-Manzanares: None declared, Norberto Ortego: None declared, Sabela Fernandez: None declared, Francisco Ortiz-Sanjuan: None declared, Montserrat Corteguera: None declared, J. Luis Hernandez: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background In Giant Cell Arteritis (GCA) two dominant cytokine clusters have been linked to disease activity, IL-6 – IL-17 axis (Th17) and IL-12 – IFN γ axis (Th1). The first one related to systemic symptoms and the second route responsible for ischemic symptoms. Tocilizumab (TCZ) performs its effect mainly by inhibiting Th17 axis and terminally Th1 route. Objectives Our aim was to evaluate the effect of TCZ on ischemic and systemic symptoms throughout the follow-up. Methods Retrospective, multicenter study of 134 patients diagnosed of GCA on treatment with TCZ. We evaluate the efficacy of TCZ by improving ischemic (visual involvement, headache, jaw claudication) and systemic symptoms (fever, constitutional syndrome, polymyalgia rheumatica (PMR)). Results We evaluated 134 patients (101 w/33 m) and its main symptoms at TCZ onset, TABLE. 73 (54.5%) patients presented PMR followed by headache in 70 (52.2%) cases, constitutional syndrome in 31 (23.1%) and visual involvement in 28 (20.9%) patients. After one month of treatment there was an important clinical improvement, persisting in 13,6% of patients PMR, 10.6% headache and 10.6% visual involvement. Throughout the follow-up, the improvement of ischemic symptoms was slower. At month 12, in 5.6% (4) of patients persisted with visual impairment, and 2.8% (2) patients presented headache and constitutional syndrome. However, the analytical improvement was statistically significant from the first month and sustained during follow-up. Conclusion According to the results of our study, we can conclude that in clinical practice, ischemic symptoms take longer to improve than systemic symptoms; being visual affectation the most frequent symptom after 12 months of follow-up. References [1] Soriano A, Muratore F, Pipitone N, Boiardi L, Cimino L, Salvarani C. Visual loss and other cranial ischaemic complications in giant cell arteritis. Nat Rev Rheumatol. 2017; 13:476-84. [2] Ciccia F, Rizzo A, Ferrante A, Guggino G, Croci S, Cavazza A, et al. New insights into the pathogenesis of giant cell arteritis. Autoimmun Rev. 2017; 16:675-83. [3] Calderon-Goercke M. Tocilizumab in giant cell arteritis. Observational, open-label multicenter study of 134 patients in clinical practice. Semin Arthritis Rheum. 2019Jan5. pii: S0049-0172(18)30571-7. Doi: 10.1016/j.semarthrit.2019.01.003. [Epub ahead of print] Disclosure of Interests Monica Calderon-Goercke: None declared, J. Loricera: None declared, D. Prieto-Pena: None declared, Vicente Aldasoro: None declared, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Ignacio Villa-Blanco: None declared, Alicia Humbria: None declared, Clara Moriano: None declared, Susana Romero-Yuste: None declared, J. Narvaez Consultant for: Bristol-Myers Squibb, Catalina Gomez-Arango: None declared, Eva Perez-Pampin: None declared, Rafael Melero: None declared, Elena Becerra-Fernandez: None declared, Marcelino Revenga: None declared, Noelia Alvarez-Rivas: None declared, Carles Galisteo: None declared, Francisca Sivera: None declared, Alejandro Olive: None declared, Maria Alvarez del Buergo: None declared, Luisa Marena Rojas: None declared, Carlos Fernandez-Lopez: None declared, Francisco Navarro: None declared, Enrique Raya: None declared, Eva Galindez: None declared, Beatriz Arca: None declared, Roser Solans-Laque: None declared, Arantxa Conesa: None declared, Cristina Hidalgo: None declared, Carlos Vazquez: None declared, Jose Andres Roman-Ivorra: None declared, Pau Lluch: None declared, Sara Manrique Arija Speakers bureau: ABBvie, MSD, Janssen, Lillly, Roche, Pfyzer, Novartis., Paloma Vela-Casasempere Grant/research support from: UCB, Abbvie, Pfizer, Roche, Bristol-Myer-Squibb (another research, not BIOBADASER related), Consultant for: UCB, Lilly, Pfizer, Roche, Bristol-Myer-Squibb, Speakers bureau: Roche, UCB, MSD, Pfizer, GSK, BMS, Lilly, Eugenio de Miguel: None declared, Carmen Torres-Martin: None declared, Juan Carlos Nieto: None declared, Carmen Ordas-Calvo: None declared, Eva Salgado-Perez: None declared, Cristina Luna-Gomez: None declared, Francisco J. Toyos Saenz de Miera: None declared, Nagore Fernandez-Llanio: None declared, Antonio Garcia: None declared, Carmen Larena: None declared, Natalia Palmou-Fontana: None declared, Vanesa Calvo-Rio: None declared, Carmen Gonzalez-Vela: None declared, Alfonso Corrales: None declared, Maria Varela-Garcia: None declared, Elena Aurrecoechea: None declared, Raquel Dos-Santos: None declared, Angel Garcia-Manzanares: None declared, Norberto Ortego: None declared, Sabela Fernandez: None declared, Francisco Ortiz-Sanjuan: None declared, Montserrat Corteguera: None declared, J. Luis Hernandez: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background Giant cell arteritis (GCA) can be refractory to corticosteroid therapy. Tocilizumab (TCZ) has been approved in the treatment of GCA. There are no studies comparing the efficacy and safety when using TCZ as monotherapy or in combination with conventional immunosuppressive drugs in GCA. Objectives Our aim was to compare efficacy and safety of TCZ combined or in monotherapy in GCA. Methods Multicenter study on 134 patients with refractory GCA who received TCZ therapy as monotherapy or combined with conventional immunosuppressants. Prolonged remission, absence of clinical symptoms and signs and normalization of the acute phase reactants for at least 6 months. Relapse, recurrence of signs or symptoms of GCA and/or ESR >20 mm/h in men or >25 mm/h in women, and/or serum CRP >0.5 mg/dL related to GCA, both before and after starting TCZ therapy. Results We evaluated 134 patients (101 w/33 m) with a mean age of 73.0±8.8 years. TCZ was prescribed as monotherapy in 82 (62.2%) cases and combined with conventional immunosuppressants in 52 (38.8%) patients: MTX (n=48), AZA (n=3), and LFN (n=1). A comparative study between both groups is summarized in TABLE. Patients who received combined TCZ were younger and had a higher C-reactive protein (CRP) and a higher presence of aortitis in imaging techniques. After TCZ was started, prolonged remission was reached with combined therapy (statistical significance at 12 and 24 months). The corticosteroids sparing effect was similar in both groups. And in terms of side effects no significant difference was seen between TCZ as monotherapy or combined with conventional immunosuppressants. Conclusion Patients receiving combined conventional immunosuppressants with TCZ in the clinical practice study showed a higher prolonged remission. The incidence of serious infections and/or relevant adverse events was not affected according to the treatment. As well as the corticoid-sparing effect was achieved in the same way in both groups. References [1] Goercke .Calderon-M. Tocilizumab in giant cell arteritis. Observational, open-label multicenter study of 134 patients in clinical practice. Semin Arthritis Rheum. 2019 Jan 5. pii: S0049-0172(18)30571-7. doi: 10.1016/j.semarthrit.2019.01.003. [Epub ahead of print] [2] Loricera J et al. Semin Arthritis Rheum.2015;44:717-723

Background Recently, based on the GiACTA trial results, weekly subcutaneous Tocilizumab (TCZ) has been approved for the treatment of Giant Cell Arteritis (GCA). It has showed to be effective and safety. Objectives Our aim was to compare the efficacy of TCZ according the route of administration. Methods Multicenter study of 134 GCA patients in treatment with TCZ. It was performed a comparative study between 2 groups according the route of administration of TCZ, intravenous (IV) or subcutaneous (SC). Results We study 134 patients divided in 2 groups: a) IV TCZ, 104 cases and, b) SC TCZ, 30 cases, with a mean age 73.4±8.2 years vs 71.9±10.6 years, respectively (p=0.501). Disease duration, clinical manifestations and acute phase reactants at TCZ onset were similar in both groups with non-statistical difference. 91.7% patients who received SC TCZ achieved prolonged remission after 12 months of treatment (p=0.043). And the glucocorticoid sparing effect of TCZ was greater in the same group, reaching a statistical difference at 3 and 24 months (p=0.017 and p=0.021). Patients under IV TCZ treatment suffered more adverse event during follow up (p=0.043). TABLE 1 and 2 summarizes the comparative study. Conclusion Patients in treatment with SC TCZ, reached prolonged remission after 12 months of treatment and were able to discontinue prednisone dose after 24 months of follow up. The incidence of adverse events was more frequent in the IV TCZ group, without difference in relation to infections. References [1] Stone JH, Tuckwell K, Dimonaco S, Klearman M, Aringer M, Blockmans D, et al. Trial of Tocilizumab in Giant-Cell Arteritis. N Engl J Med. 2017; 377:317-28. [2] Calderon-Goercke M. Tocilizumab in giant cell arteritis. Observational, open-label multicenter study of 134 patients in clinical practice. Semin Arthritis Rheum. 2019 Jan 5. pii: S0049-0172(18)30571-7. doi: 10.1016/j.semarthrit.2019.01.003. [Epub ahead of print] Disclosure of Interests Monica Calderon-Goercke: None declared, J. Loricera: None declared, D. Prieto-Pena: None declared, Vicente Aldasoro: None declared, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Ignacio Villa-Blanco: None declared, Alicia Humbria: None declared, Clara Moriano: None declared, Susana Romero-Yuste: None declared, J. Narvaez Consultant for: Bristol-Myers Squibb, Catalina Gomez-Arango: None declared, Eva Perez-Pampin: None declared, Rafael Melero: None declared, Elena Becerra-Fernandez: None declared, Marcelino Revenga: None declared, Noelia Alvarez-Rivas: None declared, Carles Galisteo: None declared, Francisca Sivera: None declared, Alejandro Olive: None declared, Maria Alvarez del Buergo: None declared, Luisa Marena Rojas: None declared, Carlos Fernandez-Lopez: None declared, Francisco Navarro: None declared, Enrique Raya: None declared, Eva Galindez: None declared, Beatriz Arca: None declared, Roser Solans-Laque: None declared, Arantxa Conesa: None declared, Cristina Hidalgo: None declared, Carlos Vazquez: None declared, Jose Andres Roman-Ivorra: None declared, Pau Lluch: None declared, Sara Manrique Arija Speakers bureau: ABBvie, MSD, Janssen, Lillly, Roche, Pfyzer, Novartis., Paloma Vela-Casasempere Grant/research support from: UCB, Abbvie, Pfizer, Roche, Bristol-Myer-Squibb (another research, not BIOBADASER related), Consultant for: UCB, Lilly, Pfizer, Roche, Bristol-Myer-Squibb, Speakers bureau: Roche, UCB, MSD, Pfizer, GSK, BMS, Lilly, Eugenio de Miguel: None declared, Carmen Torres-Martin: None declared, Juan Carlos Nieto: None declared, Carmen Ordas-Calvo: None declared, Eva Salgado-Perez: None declared, Cristina Luna-Gomez: None declared, Francisco J. Toyos Saenz de Miera: None declared, Nagore Fernandez-Llanio: None declared, Antonio Garcia: None declared, Carmen Larena: None declared, Natalia Palmou-Fontana: None declared, Vanesa Calvo-Rio: None declared, Carmen Gonzalez-Vela: None declared, Alfonso Corrales: None declared, Maria Varela-Garcia: None declared, Elena Aurrecoechea: None declared, Raquel Dos-Santos: None declared, Angel Garcia-Manzanares: None declared, Norberto Ortego: None declared, Sabela Fernandez: None declared, Francisco Ortiz-Sanjuan: None declared, Montserrat Corteguera: None declared, J. Luis Hernandez: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background Aortitis can be idiopathic or associated with other conditions. It is frequently refractory to conventional immunosuppressive therapy. Tocilizumab (TCZ), an anti-IL-6 receptor antibody seems to be effective and safe. Objectives Our aim was to assess the efficacy and safety of TCZ at short and long follow-up in a series of patients with Aortitis. Methods Retrospective, multicenter study of 79 patients diagnosed of inflammatory aortitis based on imaging techniques (PET/CT, CT angiography and/or MR angiography). Results We study 79 patients (61 w/ 18 m). 59 (74.7%) cases were Aortitis secondary to Giant Cell Arteritis (GCA), while 20 (25.3%) were idiopathic. The mean age was 71±8.5 years vs 64.2±7.1 years, respectively (p=0.001). At time of disease diagnosis more than a half of patients (59.5%) presented as main symptom polymyalgia rheumatica (PMR). Aortitis was diagnosed with PET/CT (71 patients), angioRMN (12 patients) and angioCT (8 patients). Prior to TCZ treatment, 61 (77.2%) patients had received conventional immunosuppressive drugs, 59 (74.7%) of them received MTX. After 24 months of treatment with TCZ, more than 75% of patients reached a prolonged remission in both groups (p=0.527), with only 4% of relapses after the same follow-up period (p=1.000). 40 (50.6%) patients had a control image technique (PET/CT) throughout follow up. 4 (3 secondary to GCA and 1 idiopathic) patients reached a complete improvement in uptake after one year of treatment. Conclusion Our results show that idiopathic aortitis occurs in younger patients compared with aortitis secondary to GCA. TCZ proved to be effective in both pathologies, allowing clinical and analytical improvement, as well as a reduction of corticoid dose, without increasing the risk of relapse. However, the improvement in imaging techniques seems to be slower. Reference [1] Loricera J, Blanco R, Castaneda S, Humbria A, Ortego-Centeno N, Narvaez J, et al. Tocilizumab in refractory aortitis: study on 16 patients and literature review. Clin Exp Rheumatol. 2014; 32:79-89. Disclosure of Interests Monica Calderon-Goercke: None declared, J. Loricera: None declared, D. Prieto-Pena: None declared, Vicente Aldasoro: None declared, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Ignacio Villa-Blanco: None declared, Alicia Humbria: None declared, Clara Moriano: None declared, Susana Romero-Yuste: None declared, J. Narvaez Consultant for: Bristol-Myers Squibb, Catalina Gomez-Arango: None declared, Eva Perez-Pampin: None declared, Rafael Melero: None declared, Marcelino Revenga: None declared, Noelia Alvarez-Rivas: None declared, Francisca Sivera: None declared, Maria Alvarez del Buergo: None declared, Luisa Marena Rojas: None declared, Eva Galindez: None declared, Beatriz Arca: None declared, Roser Solans-Laque: None declared, Carlos Vazquez: None declared, Pau Lluch: None declared, Eva Salgado-Perez: None declared, Cristina Luna-Gomez: None declared, Francisco J. Toyos Saenz de Miera: None declared, Nagore Fernandez-Llanio: None declared, Antonio Garcia: None declared, Carmen Larena: None declared, Natalia Palmou-Fontana: None declared, Vanesa Calvo-Rio: None declared, Carmen Gonzalez-Vela: None declared, Alfonso Corrales: None declared, Maria Varela-Garcia: None declared, Elena Aurrecoechea: None declared, Raquel Dos-Santos: None declared, Jose Luis Martin-Varillas: None declared, Sabela Fernandez: None declared, J. Luis Hernandez: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background Giant cell arteritis (GCA) is a large vessel vasculitis with a predisposition for the cranial branches of the external carotid artery. However, aorta and/or its main branches may also be involved. Objectives In a series of patients with GCA who presented extracranial vessel involvement, our aim was to assess a) the vascular territories most frequently affected and b) correlation of a major extension of extracranial vascular involvement with a more severe clinical and analytical features. Methods Multicenter study of 68 patients with GCA who presented a compromise of extracranial vessels confirmed by PET/CT. Visual analysis of vascular uptake was performed on supra-aortic trunks (SAT), aortic arch (AA), thoracic aorta (TA), abdominal aorta (AbA), iliac arteries (IA), lower limb arteries (LLA), and upper limb arteries (ULA). Results We evaluated 68 patients with GCA (51w/17m) with a mean age of 68.0±8.3 years. The vascular territories affected were: TA (n=58, 85.29%), SAT (n=38, 55.88%), AbA (n=28, 41.18%), AA (n=18, 26.47%), LLA (n=17, 25%), IA (n=13, 19.12%) and ULA (n=6, 8.82%). We considered 3 groups according to the number of vascular territories affected: a) 1 or 2 territories, b) 3 or 4 territories and c) 5 or more territories and made a comparative study between this groups. In patients with ≥5 vascular territories affected, we observed a higher baseline ESR, and the most frequent systemic manifestations were polymyalgia rheumatica and constitutional symptoms with statistical significance (TABLE). Distribution of categorical variables was compared by the Pearson Chi-squared test. Quantitative variables were analyzed using the ANOVA test. Conclusion In patients with GCA the involvement of TA is very frequent, followed by the SAT and the AbA. Regarding the laboratory findings, patients with higher levels of ESR presented a major extension of extracranial vascular involvement, as well as presenting PMR and/or constitutional symptoms was also related to more affection of extracranial territories. References [1] Loricera J, Blanco R, Hernaandez JL, et al. Use of positron emission tomography (PET) for the diagnosis of large-vessel vasculitis. Rev Esp Med Nucl Imagen Mol. 2015; 34: 372-377. [2] Loricera J, Blanco R, Hernaandez JL, et al. Non-infectious aortitis: a report of 32 cases from a single tertiary centre in a 4-year period and literature review. Clin Exp Rheumatol. 2015; 33: S19-31. Disclosure of Interests Monica Calderon-Goercke: None declared, J. Loricera: None declared, D. Prieto-Pena: None declared, J. Narvaez Consultant for: Bristol-Myers Squibb, Santos Castaneda Consultant for: Amgen, BMS, Pfizer, Lilly, MSD, Roche, Sanofi, UCB, Elena Aurrecoechea: None declared, Ignacio Villa-Blanco: None declared, Catalina Gomez-Arango: None declared, Eva Perez-Pampin: None declared, Vicente Aldasoro: None declared, Noelia Alvarez-Rivas: None declared, Nagore Fernandez-Llanio: None declared, Maria Alvarez del Buergo: None declared, Luisa Marena Rojas: None declared, Francisca Sivera: None declared, Eva Galindez: None declared, Roser Solans-Laque: None declared, Susana Romero-Yuste: None declared, Norberto Ortego: None declared, Marcelino Revenga: None declared, Rafael Melero: None declared, Eva Salgado-Perez: None declared, Sabela Fernandez: None declared, Vanesa Calvo-Rio: None declared, Natalia Palmou-Fontana: None declared, Jose Ignacio Banzo: None declared, Isabel Martinez-Rodriguez: None declared, Carmen Gonzalez-Vela: None declared, Raquel Dos-Santos: None declared, J. Luis Hernandez: None declared, Miguel A Gonzalez-Gay Grant/research support from: Prof. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, Jansen and Roche., Speakers bureau: Consultation fees/participation in company sponsored speaker’s bureau from Pfizer, Lilly, Sobi, Celgene, Novartis, Roche and Sanofi., Ricardo Blanco Grant/research support from: Abbvie, MSD, and Roche, Consultant for: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen

Background: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

ResumenCaso clínicoPaciente diagnosticada de artritis reumatoide (AR) en el año 2006, con buen control global. En el año 2013 comenzó con dolor, hiperemia conjuntival, prurito y visión borrosa, siendo diagnosticada de queratitis ulcerativa periférica (PUK), encontrándose estable articular y analíticamente. Se intensificó tratamiento con metotrexate, y se inició tratamiento con infliximab, con gran mejoría.DiscusiónLa PUK es una enfermedad rara, pero a tener en cuenta en los pacientes con AR. En la mayoría de los casos, el tratamiento de fondo debe ser intensificado; en este sentido deben considerarse los tratamientos biológicos por su eficacia y efecto rápido de acción.AbstractCase reportPatient diagnosed with rheumatoid arthritis (RA) in 2006 with good overall control. In 2013 she began with pain, conjunctival hyperemia, pruritus, and blurred vision being diagnosed with peripheral ulcerative keratitis (PUK), being well controlled articulately and analytically. Treatment with methotrexate was intensified and infliximab treatment was started with great improvement.DiscussionPUK is a rare condition but it must be considered in patients with RA. In most cases maintenance treatment must be intensified; in this sense, biological agents should be considered for their efficacy and rapid onset of action.

Background:Infections are the most common adverse event of Tocilizumab (TCZ) in Giant Cell Arteritis (GCA). In GiACTA study(1),serious infections were observed in 7% (9.6/100 patient-years) of patients who received TCZ weekly. Randomized clinical trials (RCTs) are conducted under highly standardized design excluding some real-world patients. Therefore, adverse events may be underestimated in RCTs. In our series of real-life, serious infections occurred in 11.9% (10.6/100 patient-years)(2).Objectives:In a wide series of GCA of clinical practice treated with TCZ, we assess the frequency, type and predisposing factors of serious infections.Methods:Multicenter study of 134 patients diagnosed with GCA, all of them refractory to conventional therapy, treated with TCZ. Serious infection was considered when a life-threatening infection, fatal, or requiring hospitalization occurred, intravenous antibiotics were required, or the infectious process led to persistent or significant disability.Results:16 of 134 (11.9%, 10.6/100 patient-years) patients developed serious infections during follow-up. The most frequent infections were pneumonia (n=4), urinary tract infection (n=4), and facial herpes zoster (n=2). At TCZ onset, serious infections were more frequent in older patients (74.3±9.6 vs 72.9±8.7 years), with a longer GCA evolution (20 [4.3-45.6] vs 13 [5-29.3] months), with visual manifestations (43.75% vs 17.8%) and a higher dose of prednisone at TCZ onset (30.4±15.5 vs 21.1±16.1 mg/day) (TABLE). Presence of comorbidities were similar in both groups. 13 of the 16 patients who had infections received a dose of prednisone greater than 15 mg/day (16.3/100 patient-years) compared to 3 patients under treatment with less than 15 mg/day of prednisone (4.2/100 patient-years).Conclusion:The age, GCA duration, ocular involvement and the dose of glucocorticoids, at TCZ onset, seem to be predisposing factors related to an increased risk of developing serious infections in GCA patients.References:[1]Stone JH, et al. N Engl J Med. 2017; 377:317-28.[2]Calderón-Goercke M et al. Semin Arthritis Rheum 2019 Aug;49(1): 126-135.TABLESERIOUS INFECTIONS(n=16)WITHOUT SERIOUS INFECTIONS(n=118)pBASAL FEATURES AT TCZ ONSETGENERAL FEATURES Age, years, mean± SD74.3±9.672.9±8.70.552 Sex, female/male n(%)13/388/300.760 Time from GCA diagnosis to TCZ onset (months), median [IQR]20[4.3-45.6]13[5-29.3]0.604COMORBIDITIES Hypertension, n(%)9(56)86(73)0.551 Diabetes, n(%)3(19)39(33)0.677 Chronic kidney disease, n(%)3(19)27(23)0.512CLINICAL FEATURES OF GCA PMR, n(%)9(56.25)64(54.2)0.879 Aortitis, n(%)5(31.25)53(45)0.301 Visual manifestations, n(%)7(43.75)21(17.8)0.017CORTICOSTEROIDS AT TCZ ONSET Prednisone dose mg/d, mean (SD)30.4±15.521.1±16.10.031Disclosure of Interests:Monica Calderón-Goercke: None declared, D. Prieto-Peña: None declared, Santos Castañeda: None declared, Clara Moriano: None declared, Elena Becerra-Fernández: None declared, Marcelino Revenga: None declared, Noelia Alvarez-Rivas: None declared, Carles Galisteo: None declared, Águeda Prior-Español: None declared, E. Galindez: None declared, Cristina Hidalgo: None declared, Sara Manrique Arija: None declared, Eugenio de Miguel Grant/research support from: Yes (Abbvie, Novartis, Pfizer), Consultant of: Yes (Abbvie, Novartis, Pfizer), Paid instructor for: yes (AbbVie, Novartis, Pfizer, MSD, BMS, UCB, Roche, Grunental, Janssen, Sanofi), Speakers bureau: yes (AbbVie, Novartis, Pfizer, MSD, BMS, UCB, Roche, Grunental, Janssen, Sanofi), Eva Salgado-Pérez: None declared, Vicente Aldasoro Speakers bureau: Roche, Abbvie, MSD, UCB, Pfizer, Menarini, Grunenthal, Gebro, Novartis, Janssen, Ignacio Villa-Blanco Consultant of: UCB, Speakers bureau: Novartis, MSD, Lilly, Susana Romero-Yuste: None declared, J. Narváez: None declared, Catalina Gomez-Arango: None declared, Eva Perez-Pampín: None declared, Rafael Melero: None declared, Francisca Sivera: None declared, Carlos Fernández-Díaz Speakers bureau: Brystol Meyers Squibb, Alejandro Olive: None declared, María Álvarez del Buergo: None declared, Luisa Marena Rojas: None declared, Carlos Fernández-López: None declared, Francisco Navarro: None declared, Enrique Raya: None declared, Beatriz Arca: None declared, Roser Solans-Laqué: None declared, Arantxa Conesa: None declared, Carlos Vázquez: None declared, Jose Andrés Román-Ivorra: None declared, Pau Lluch: None declared, Paloma Vela-Casasempere: None declared, Carmen Torres-Martín: None declared, Juan Carlos Nieto Speakers bureau: Pfizer, Abbvie, MSD, Novartis, Janssen, Lilly, Nordic Pharma, BMS, Gebro, FAES Farma, Roche, Sanofi, Carmen Ordas-Calvo: None declared, Cristina Luna-Gomez: None declared, Francisco J. Toyos Sáenz de Miera: None declared, Nagore Fernández-Llanio: None declared, Antonio García: None declared, Carmen González-Vela: None declared, Javier García-Fernández: None declared, Patricia Vicente-Gómez: None declared, Ángel García-Manzanares: None declared, Norberto Ortego: None declared, Francisco Ortiz-Sanjuán: None declared, Montserrat Corteguera: None declared, J. Luis Hernández: None declared, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Ricardo Blanco Grant/research support from: AbbVie, MSD, and Roche, Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, and MSD

Background:Tocilizumab (TCZ) is the only biological agent approved in Giant Cell Arteritis (GCA). 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 scarce.Objectives:Our aim was to assess efficacy and safety of TCZ therapy optimization in an unselected wide series of GCA in clinical practice.Methods:Multicenter study, 134 patients with GCA who received TCZ due to inefficacy/adverse events of previous therapy. Once complete remission was reached and based on a shared decision between patient and physician TCZ was optimized in some cases. Optimization was done by decreasing the dose and/or prolonging the TCZ dosing interval progressively.Results:134 GCA patients treated with TCZ (101w/33m); mean age 73.0±8.8 years. TCZ was administered IV to 106 (79.1%) patients and SC to 28 (20.9%). TCZ was optimized in 43 (32.1%) patients. No demographic, clinical manifestations or laboratory data differences had been found at TCZ onset (TABLE). After a follow up of 12 [6-15.5] months, and a complete remission for 6 [3-12] months; the first TCZ optimization was performed. Median prednisone dose at first TCZ optimization was 2.5 [0-5] mg/day. TCZ IV was optimized from 8 to 4 mg/kg/4weeks in 12 of 106 (11.3%) and from 162 mg/SC/week to 162 mg/SC/2weeks in 9 of 28 (32.1%) cases. Five (11.6%) of the 43 optimized cases relapsed. In 4 cases, the relapses were treated increasing TCZ up to the pre-optimization dose, in 1 case the route of administration was change (4 mg/kg/4week to 162 mg/SC/week). In 8 of 43 optimized patients (18.6%), it was possible to withdraw TCZ after complete remission for 30 [16.25-45.75] months. Regarding adverse events and severe infections were similar in both groups. The mean TCZ treatment costs were lower in the optimized group.Conclusion:Once remission is reached in GCA patients under TCZ treatment, optimization of TCZ may be performed. Based on our experience it could be performed by reducing the dose with IV TCZ or by prolonging dosing interval with SC TCZ.References:[1]Calderón-Goercke M et al. Semin Arthritis Rheum 2019 Aug;49(1): 126-135.TABLE.OPTIMIZED-TCZ GROUP (n=43)NON-OPTIMIZED TCZ GROUP (n=91)pBASAL FEATURES AT TCZ ONSETGENERAL FEATURESAge, years, mean± SD68.9±8.771.4±8.50.125Sex, female/male n(%)32/1068/240.779Time from GCA diagnosis to TCZ onset (months), median [IQR]19.5[7.75-45]10.5[4 – 25]0.047SYSTEMIC MANIFESTATIONSFever, n(%)1(2.4)8(8.7)0.176Constitutional syndrome, n(%)11(26.2)19(20.7)0.476PMR, n(%)18(42.9)56(60.9)0.052ISCHEMIC MANIFESTATIONSVisual involvement, n(%)5(11.9)23(25)0.084Headache, n(%)26(61.9)42(45.7)0.081Jaw claudication, n(%)1(2.4)11(12)0.072CORTICOSTEROIDS AT TCZ ONSETPrednisone dose, mg/d mean (SD)15.1±11.125±17.40.001FOLLOW-UP ON TCZ THERAPY (MONTHS), MEDIAN [IQR]24[18-27]6 [3-18]0.000Relapses, n(%)5(11.6)5(5.5)0.207End follow-up remission, n(%)40(93)84(92)0.99Severe side efects, n(%)14(32.6)22(24.2)0.307Seriuos infections, n(%)6(14)10(11)0.878Cost, (mean) euros per yearIVSC7 538.47 329.011 726.411 726.4--Disclosure of Interests:Monica Calderón-Goercke: None declared, D. Prieto-Peña: None declared, Santos Castañeda: None declared, Clara Moriano: None declared, Elena Becerra-Fernández: None declared, Marcelino Revenga: None declared, Noelia Alvarez-Rivas: None declared, Carles Galisteo: None declared, Águeda Prior-Español: None declared, E. Galindez: None declared, Cristina Hidalgo: None declared, Sara Manrique Arija: None declared, Eugenio de Miguel Grant/research support from: Yes (Abbvie, Novartis, Pfizer), Consultant of: Yes (Abbvie, Novartis, Pfizer), Paid instructor for: yes (AbbVie, Novartis, Pfizer, MSD, BMS, UCB, Roche, Grunental, Janssen, Sanofi), Speakers bureau: yes (AbbVie, Novartis, Pfizer, MSD, BMS, UCB, Roche, Grunental, Janssen, Sanofi), Eva Salgado-Pérez: None declared, Vicente Aldasoro Speakers bureau: Roche, Abbvie, MSD, UCB, Pfizer, Menarini, Grunenthal, Gebro, Novartis, Janssen, Ignacio Villa-Blanco Consultant of: UCB, Speakers bureau: Novartis, MSD, Lilly, Susana Romero-Yuste: None declared, J. Narváez: None declared, Catalina Gomez-Arango: None declared, Eva Perez-Pampín: None declared, Rafael Melero: None declared, Francisca Sivera: None declared, Alejandro Olive: None declared, María Álvarez del Buergo: None declared, Luisa Marena Rojas: None declared, Carlos Fernández-López: None declared, Francisco Navarro: None declared, Enrique Raya: None declared, Beatriz Arca: None declared, Roser Solans-Laqué: None declared, Arantxa Conesa: None declared, Carlos Vázquez: None declared, Jose Andrés Román-Ivorra: None declared, Pau Lluch: None declared, Paloma Vela-Casasempere: None declared, Carmen Torres-Martín: None declared, Juan Carlos Nieto Speakers bureau: Pfizer, Abbvie, MSD, Novartis, Janssen, Lilly, Nordic Pharma, BMS, Gebro, FAES Farma, Roche, Sanofi, Carmen Ordas-Calvo: None declared, Cristina Luna-Gomez: None declared, Francisco J. Toyos Sáenz de Miera: None declared, Nagore Fernández-Llanio: None declared, Antonio García: None declared, J. Luis Hernández: None declared, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Ricardo Blanco Grant/research support from: AbbVie, MSD, and Roche, Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, and MSD

Objective: Tocilizumab (TCZ) has shown efficacy in clinical trials on giant cell arteritis (GCA). Real-world data are scarce. Our objective was to assess efficacy and safety of TCZ in unselected patients with GCA in clinical practice Methods: Observational, open-label multicenter study from 40 national referral centers of GCA patients treated with TCZ due to inefficacy or adverse events of previous therapy. Outcomes variables were improvement of clinical features, acute phase reactants, glucocorticoid-sparing effect, prolonged remission and relapses. A comparative study was performed: (a) TCZ route (SC vs. IV); (b) GCA duration (6 months); (c) serious infections (with or without); (d) < 15 vs. >15 mg/day at TCZ onset. Results: 134 patients; mean age, 73.0 +/- 8.8 years. TCZ was started after a median [IQR] time from GCA diagnosis of 13.5 [5.0-33.5] months. Ninety-eight (73.1%) patients had received immunosuppressive agents. After 1 month of TCZ 93.9% experienced clinical improvement. Reduction of CRP from 1.7 [0.4-3.2] to 0.11 [0.05-0.5] mg/dL (p < 0.0001), ESR from 33 [14.5-61] to 6 [2-12] mm/1st hour (p < 0.0001) and decrease in patients with anemia from 16.4% to 3.8% (p < 0.0001) were observed. Regardless of administration route or disease duration, clinical improvement leading to remission at 6, 12, 18, 24 months was observed in 55.5%, 70.4%, 69.2% and 90% of patients. Most relevant adverse side-effect was serious infections (10.6/100 patients-year), associated with higher doses of prednisone during the first three months of therapy. Conclusion: In clinical practice, TCZ yields a rapid and maintained improvement of refractory GCA. Serious infections appear to be higher than in clinical trials. (C) 2019 Elsevier Inc. All rights reserved.

Background Giant cell arteritis (GCA) is a large vessel vasculitis with a predisposition for the cranial branches of the external carotid artery. However, aorta and/or its main branches may also be involved (1–3. Objectives To assess the vascular territories most frequently affected in a series of patients with GCA who presented extracranial vessel involvement. Methods Retrospective study of patients with GCA who presented compromise of extracranial vessels confirmed by PET/CT. Visual analysis of vascular uptake was performed on supra-aortic trunks (SAT), aortic arch (AA), thoracic aorta (TA), abdominal aorta (AA), iliac arteries (IA), lower limb arteries (LLA), and upper limb arteries (ULA). We carried out a comparative study between both sexes to see if there was any difference in the pattern of affectation. Results We evaluated 68 patients with GCA (51W/17M) with a mean age of 68.06±8.33 years. The vascular territories affected were: TA (n=58, 85.29%), SAT (n=38, 55.88%), AA (n=28, 41.18%), AA (n=18, 26.47%), LLA (n=17, 25%), IA (n=13, 19.12%) and ULA (n=6, 8.82%). We also made a study of the number of vascular territories affected: 1 vascular territory (n=13, 19.12%), 2 territories (n=22, 32.35%), 3 territories (n=18, 26.47%), 4 territories (n=12, 17.65%) and more than 4 territories (n=3, 4.41%). Likewise, a comparative study between both sexes was conducted, in which only statistical significance was achieved in the involvement of ULA, which was more frequent in men (table 1). Conclusions In patients with GCA the involvement of TA is very frequent, followed by the SAT and the AA. To a lesser extent, the AA and the LLA vessels are affected. The involvement of the IA and the ULA vessels is less frequent; the latter more frequently in men. On the other hand, the involvement of 2–3 vascular territories are the most frequent patterns. References [1] Loricera J, Blanco R, Hernandez JL, et al. Use of positron emission tomography (PET) for the diagnosis of large-vessel vasculitis. Rev Esp Med Nucl Imagen Mol. 2015;34:372–377. [2] Loricera J, Blanco R, Hernandez JL, et al. Non-infectious aortitis: a report of 32 cases from a single tertiary centre in a 4-year period and literature review. Clin Exp Rheumatol. 2015;33:S19–31. [3] Loricera J, Blanco R, Hernandez JL, et al. Tocilizumab in giant cella arteritis: Multicenter open-label study of 22 patients. Semin Arthritis Rheum. 2015;44:717–723. Disclosure of Interest None declared

Background Giant cell arteritis (GCA) is a large vessel vasculitis that has a special predilection for extracranial branches of the external carotid artery. Among its most fearsome complications is visual affectation. Tocilizumab (TCZ) is a monoclonal antibody directed against the interleukin 6 receptor that has shown utility in the treatment of GCA. Objectives Our aim was to assess the evolution of visual clinic in patients with GCA treated TCZ. Methods Retrospective multicentre study of 20 GCA patients with visual involvement treated with TCZ. The efficacy of this drug on visual symptoms was evaluated. Results We evaluated 20 patients (14 women and 6 men) with a mean age ±SD of 73.7±10.1 years with GCA and visual symptoms. In total there were 23 affected eyes. The symptoms reported were: unilateral blindness (n=6), unilateral blurred vision (n=6), unilateral amaurosis fugax (n=3), unilateral hemianopsia (n=2), bilateral blindness (n=1), bilateral blurred vision (n=1), bilateral hemianopsia (n=1). Before starting treatment with TCZ all patients had received high doses of prednisone, with a mean ±SD of 54.2±13.8 mg/day (range of doses: 40–80 mg/day). In addition, 9 of them also received intravenous corticosteroid boluses. In addition, 13 patients received traditional immunosuppressants: methotrexate (MTX) (n=12), cyclophosphamide (n=2), leflunomide (n=1) and azathioprine (n=1). Regardless of corticosteroids, TCZ was administered as monotherapy in 14 patients, while in 6 it was administered in combination with MTX. The TABLE shows the evolution of the visual affectation of these patients. Throughout a median follow-up [RIC] of 94–18 months, none of the 7 patients who had a blindness regained vision. The 2 patients who presented unilateral hemianopsia recovered vision. The patient with bilateral hemianopsia and the patient with bilateral blurred vision experienced a partial improvement. The rest of the patients achieved a complete recovery. Conclusions Although TCZ seems to be also useful in the treatment of visual manifestations of ACG, once blindness is established, it does not seem to be effective. Disclosure of Interest None declared

Background Giant cell arteritis (GCA) is a large-vessel vasculitis which can involve the aorta and/or its major branches. Tocilizumab (TCZ) seems to be effective in giant cell arteritis (GCA).1–4 Objectives Our aim was to assess if the clinical and analytical improvement yielded in patients with GCA treated with TCZ is accompanied by a reduction of the vascular inflammation evaluated by PET/CT. Methods Study of 36 patients who had a baseline and follow-up PET/CT from a multicenter series of 134 patients with GCA in treatment with TCZ. The evolution of the vascular involvement objectified by PET/CT was assessed. In addition clinical efficacy, analytical improvement (acute phase reactants) and the reduction of corticosteroid dose was studied. Results The 36 patients (28 women and 8 men) had an mean age of 69.83±8.66 years. After TCZ onset, a rapid and maintained clinical improvement was observed (table 1). In addition, during the first twenty-four months of follow-up, C-reactive protein decreased from 2.4 [0.9–6.8] to 0.1 [0.0–0.5] mg/dL and the erythrocyte sedimentation rate from 41.5 [16.7–58.5] to 4 [2–12.5] mm/1 st hour. On the other hand, the levels of haemoglobin experienced an increase from 12.3 [11.3–13.0] to 13.3 [13.0–13.9] g/dL. The median dose of prednisone decreased from 12.5 [9.4–26.2] to 0.0 [0.0–0.0] mg/day. However, the decrease in F18-fluordeoxyglucose uptake in the PET/CT study was not as evident. Conclusions Although TCZ seems to be an important therapeutic agent in the treatment of GCA, achieving a rapid and sustained clinical and analytical improvement, the decrease in vessel inflammation assessed by F18-fluordeoxyglucose uptake seems to take a slower course. References [1] Loricera J, et al. Tocilizumab in giant cell arteritis: Multicenter open-label study of 22 patients. Semin Arthritis Rheum2015;44:717–723. [2] Loricera J, et al. Tocilizumab in refractory aortitis: Study on 16 patients and literature review. Clin Exp Rheumatol2014;32(3 Suppl 82):S79–89. [3] Loricera J, et al. Use of positron emission tomography (PET) for the diagnosis of large-vessel vasculitis. Rev Esp Med Nucl Imagen Mol2015;34:372–377. [4] Loricera J, Blanco R, Hernandez JL, et al. Tocilizumab in patients with Takayasu arteritis: A retrospective study and literature review. Clin Exp Rheumatol2016;34(3 Suppl 97):S44–53. Disclosure of Interest None declared

Resumen Objetivo Describir las caracteristicas demograficas y trastornos de pacientes con diagnostico de sindrome de activacion macrofagica (SAM) en el periodo comprendido entre diciembre de 2008-enero de 2014. Metodos Se revisaron las historias clinicas desde el diagnostico de SAM y tras su alta hospitalaria hasta enero de 2014. Los pacientes se agruparon en 4 grupos: autoinmunes (AI), hemato-oncologicas (HO), infecciosas (Inf) y oncologicas (Onc). Las variables fueron analizadas entre los 4 grupos y entre AI y HO. Resultados Trece pacientes (7 hombres, con una mediana de 54 anos [32-63]) se estudiaron. Las etiologias encontradas fueron: 5 AI, 5 HO, 2 Inf y una Onc. Se encontraron celulas hemofagociticas en el liquido ascitico en uno de los pacientes. Se encontro un paciente con SAM secundario a enfermedad relacionada con la IgG4. Conclusiones La mortalidad, el pronostico y la evolucion de la enfermedad puede verse influida por el retraso en el diagnostico, el inicio del tratamiento y la etiologia del SAM. Los pacientes con enfermedades HO presentaron peor pronostico.

Objectives Describe patients with noninfectious aortitis and their response to treatment in a regional hospital. Methods Review of patients with noninfectious aortitis, diagnostic technique used and immunosuppressive therapy received. Results We report 8 patients (7 women and one man) diagnosed with aortitis by positron emission tomography (PET). The mean age was 69 years (interquartile range [IQR] 62–72.2). Three months of treatment with tocilizumab improved symptoms, erythrocyte sedimentation rate and C -reactive protein level (P Conclusions Tocilizumab was an effective and safe treatment in those patients diagnosed with aortitis refractory to steroids and conventional immunosuppressive therapy.

Introduction Hemophagocytic syndrome (HS) occurs in autoimmune diseases and belongs to the hemophagocytic lymphohistiocytosis group of diseases. This paper describes the features of 2 patients with systemic lupus erythematosus (SLE) who presented HS as the initial clinical manifestation. Clinical observations Both patients had prolonged fever not associated to an infectious process and did not respond to broad-spectrum antibiotics. Discussion The diagnosis of HS secondary to SLE is complicated, because it has some features in common, but HS is characterized by hyperferritinemia, hipofibrinogemia, hypertriglyceridemia and a decrease in the erythrocyte sedimentation rate, unlike SLE. HS treatment when associated to SLE is not well established, but steroids and/or immunoglobulins are effective as the initial treatment, and in refractory cases, cyclosporine or cyclophosphamide may be associated. Conclusions HS can be the initial manifestation of SLE and should be suspected in patients with organ enlargement, cytopenias, clotting disorders, liver disorders and prolonged fever unresponsive to antibiotics. Anakinra may be a treatment option in adult HS associated to SLE.

Objective Describe the demographic characteristics and disorders of patients with diagnosis of macrophage activation syndrome (MAS) in the December 2008–January 2014 period. Methods Medical records were reviewed from diagnosis of MAS and after discharge until January 2014. Patients were divided into 4 groups according to the primary disease: autoimmune (AI), hemato-oncologic (HO), infectious (Inf) and oncologic (Onc). The variables were analyzed among the 4 groups and between AI and HO. Results Thirteen patients [7 men, with a median of 54 years (32–63)] were studied. The etiologies were: 5 AI, 5 HO, 2 Inf and 1 Onc disease. Hemophagocytic cells were found in the ascitic fluid of one patient. A patient with MAS secondary to IgG4-related disease was found. Conclusions Mortality, prognosis and disease progression may be influenced by the delay in diagnosis, treatment initiation and etiology of MAS. HO ill patients had a worse prognosis.