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Objective: The spectrum of giant cell arteritis (GCA) includes different vascular phenotypes. Tocilizumab (TCZ) is the only biologic currently approved regardless these phenotypes. We aimed to assess the effectiveness of TCZ in different phenotypes., Methods: Multicentre observational study of GCA patients treated with TCZ. They were divided into three phenotypes: a) cranial (cGCA), b) extracranial (ecGCA) and c) mixed (mixGCA). Outcomes included clinical remission, EULAR complete remission, relapses, absence of inflammation in imaging techniques, and safety., Results: We studied 471 patients (342 women; mean age 74.0 ± 9.0 years). The phenotypic distribution was: cGCA (n = 217; 46%), mixGCA (174; 37%) and ecGCA (80; 17%). Patients with ecGCA were younger (66.5 ± 10.1 years) than cGCA (74.8 ± 8.1) and mixGCA (71.4 ± 8.5), and had a longer delayed GCA diagnosis (median [IQR]) (6 [1-14] vs 1 [1-3] vs 2 [1-6] months, respectively). Systemic manifestations were similar in the 3 groups, while ischemic manifestations were more frequent in cGCA. Combined TCZ -besides glucocorticoids- was used more frequently in ecGCA (36%). Clinical remission was observed in 51%/43%/47% in cGCA/ecGCA/mixGCA, respectively, at first month, and in 79%/81%/89% at 24 months. Complete EULAR remission in 35%/27%/28% (1st month) and 72%/73%/67% (24 months).Absence of inflammation in imaging techniques was of 15%/26% (12 months) and of 22%/7% (ecGCA/mixGCA (24 months). Relevant adverse events were observed in 109 (23.1%) patients., Conclusion: TCZ shows a rapid and maintained effectiveness in all GCA phenotypes in clinical and EULAR complete remission. By contrast, absence of inflammation in imaging techniques was much lower in ecGCA and mixGCA., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objective: Aortitis in Giant Cell Arteritis (GCA-aortitis) is a frequent complication that may lead to aneurysms. Tocilizumab (TCZ) was approved in GCA, but the efficacy in GCA-aortitis and aneurysms has not been analyzed to date. Our aim was to assess the effectiveness and safety of TCZ in a wide series of GCA-aortitis and aneurysms., Methods: Multicentre observational study with GCA-aortitis treated with TCZ. GCA was diagnosed by: a) ACR criteria, b) temporal artery biopsy, and/or c) imaging techniques. Aortitis was diagnosed mainly by PET/CT. Main outcomes were EULAR and imaging remission. Others were clinical remission, analytical normalization, corticosteroid-sparing effect, and the prevention and improvement of aneurysms., Results: 196 patients with GCA-aortitis treated with TCZ. After 6 months, 72.2% reached EULAR remission but only 12% an imaging remission; increasing up-to 81.4% and 31.8%, respectively, at 24 months. A rapid clinical remission, ESR and CRP normalization was observed in 47.4%, 84.3% and 55.6%, at 1 month, increasing to 89.6%, 85.3% and 80.3% at 24 months, respectively. Aneurysms were present in 10 (5%) patients. Five of them required early surgery, while 3 others enlarged. No patient on TCZ therapy developed aneurysms during follow-up., Conclusion: In patients with GCA-aortitis treated with TCZ, a rapid and maintained clinical and analytical improvement was observed. However, there was an uncoupling between clinical and EULAR remission with imaging remission., Competing Interests: Declaration of competing interest Disclosures that might be interpreted as constituting possible conflict(s) of interest for the study: Javier Loricera had consultation fees/participation in company-sponsored speaker´s bureau from Roche, Galápagos, Novartis, UCB Pharma, MSD, Celgene, Astra Zeneca, and Grünenthal and received support for attending meetings and/or travel from Janssen, Abbvie, Roche, Novartis, MSD, UCB Pharma, Celgene, Lilly, Pfizer, Galápagos. Paloma Vela research funding/consulting and conferences fees from: Abbvie, Roche, Amgen, Novartis, Lilly, Pfizer, GSK. Susana Romero-Yuste has received grants/recents supports from Abbvie, Astra-Zeneca, Bristol, Janssen, Lilly, Roche, Sandoz, Sanofi, UCB. Eugenio De Miguel Research funding/consulting and conferences fees from: Abbvie, Novartis, Pfizer, Roche, Janssen, Lilly, MSD, BMS, UCB, Grünenthal and Sanofi. Iván Ferraz-Amaro would like to acknowledge that he has received grants/research supports from Abbvie, MSD, Janssen, and Roche, as well as consultation fees from company sponsored speakers bureaus associated with Abbvie, Pfizer, Roche, Sanofi, Celgene, and MSD. Fernando López-Gutiérrez received support for attending meetings and/or travel from Janssen, Abbvie, Roche, Novartis, MSD, UCB Pharma, Celgene, Lilly, Pfizer, Galápagos. Santos Castañeda has received research support from MSD and Pfizer and had consultation fees/participation in company-sponsored speaker's bureau from BMS, Eli-Lilly, Roche, Gedeon-Richter, Grünenthal Pharma and UCB. Ricardo Blanco received grants/research support from AbbVie, MSD, and Roche, and had consultation fees/participation in a company-sponsored speaker's bureau from AbbVie, Pfizer, Roche, GSK, Lilly, UCB, Bristol-Myers, Novartis, Janssen, UCB and MSD. The following authors did not declare financial disclosure: Adrián Martín-Gutiérrez, Javier Narváez, Vicente Aldasoro, Olga Maiz, Eva Galíndez-Agirregoikoa, Jesús C. Fernández-López, Julio Sánchez-Martín, Patricia Moya, Cristina Campos., (Copyright © 2024 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.)

Objectives: The aim of this study is to analyse the diagnostic value of positron emission tomography (PET) in patients with giant cell arteritis (GCA) despite glucocorticoid (GC) therapy before PET acquisition., Methods: Consecutive patients with strongly suspected GCA according to 2022 EULAR/ACR criteria were included. The physician diagnosis of GCA after 6 months of follow-up was the gold standard. PET was performed at baseline and 6 months later. In patients with negative results at 60 min, delayed imaging was performed at 180 min., Results: Twenty-six patients were included with a median (IQR) age of 70.5 (57-88) years. Baseline PET was positive in all but one: 18 patients at 60 min and 7 patients after delayed imaging at 180 min. The median (IQR) GC dose at the time of baseline PET was 45 mg/d (26.2-45) of prednisone equivalent with a median exposure of 14 days (7-76.2). At 6 months of follow-up, PET was performed in 22 patients, with positive results in 16. Delayed imaging was performed in 6 patients due to negative PET at 60 min, with positive results in all cases, despite treatment with GC and/or biological therapy., Conclusions: In patients on GC therapy, delayed imaging protocols applying procedural recommendations for vascular quantification could improve diagnostic accuracy. Therefore, we suggest performing imaging only at 180 min in patients who have been on GCs for more than 3 days as well as in those with highly suspected GCA but negative findings in baseline PET at 60 min.

Objectives: 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., Methods: We carried out a 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., Results: 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)., Conclusions: 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.

Background: 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., Objective: 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., Design: This is an observational multicenter study of patients with GCA treated with TCZ., Methods: 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., Results: Four hundred seventy-one GCA patients (mean age, 74 ± 9 years) 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., Conclusion: 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., Competing Interests: Competing interests: The authors declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Disclosures that might be interpreted as constituting of possible conflict(s) of interest for the study: J. Loricera had consultation fees/participation in company-sponsored speaker’s bureau from Roche, Novartis, UCB Pharma, MSD, Celgene, and Grünenthal and received support for attending meetings and/or travel from Janssen, AbbVie, Roche, Novartis, MSD, UCB Pharma, Celgene, Lilly, Pfizer, Galápagos. S. Castañeda is assistant professor of the UAM-Roche, EPID-Future chair, Department of Medicine, Universidad Autónoma de Madrid, Madrid, Spain, and received support for attending meetings and/or travel from Janssen, AbbVie, Roche, Novartis, MSD, UCB Pharma, Celgene, Lilly, Pfizer. V. Aldasoro had consultation fees/participation in company sponsored speaker’s bureau from Janssen, BMS, MSD, Roche, Sanofi, Pfizer, Novartis, Amgen, Lilly, AbbVie, Gebro, Nordic, Lacer, Alter, UCB, ASAC Pharma, Menarini and Celgene and received support for attending meetings and/or travel from Janssen, BMS, MSD, Roche, Sanofi, Pfizer, Novartis, Amgen, Lilly, AbbVie, Gebro, Nordic, Lacer, Alter, UCB, ASAC Pharma, Menarini, and Celgene. O. Maiz received support for attending meetings and/or travel from Janssen, AbbVie, Roche, Novartis, MSD, UCB Pharma, Celgene, Lilly, Pfizer. P. Vela received grants/research supports from AbbVie, Pfizer, BMS, Novartis, MSD, and Roche and had consultation fees/participation in company sponsored speaker’s bureau from Pfizer, BMS, Lilly, UCB, and MSD and received support for attending meetings and/or travel from Pfizer, BMS, Lilly, UCB, and MSD. S. Romero-Yuste had consultation fees/participation in company sponsored speaker’s bureau from AbbVie, Astra-Zeneca, BMS, Fresenius, Janssen, K. Pharma, Lilly, MSD, Novartis, Pfizer, Roche, Rubió, Sandoz, Sanofi, UCB and attendance at conferences AbbVie, Astra-Zeneca, BMS, Fresenius, Janssen, K. Pharma, Lilly, MSD, Novartis, Pfizer, Roche, Rubió, Sandoz, Sanofi, UCB. E. de Miguel had consultation fees/participation in company sponsored speaker’s bureau from AbbVie, Novartis, Pfizer, BMS, MSD, UCB, Roche, Grünenthal, and Janssen and received support for attending meetings and/or travel from AbbVie, Novartis, Pfizer, BMS, MSD, UCB, Roche, Grünenthal, and Janssen. E. Galíndez-Agirregoikoa had consultation fees/participation in company sponsored speaker’s bureau from Celgene, AbbVie, Pfizer, Roche, Lilly, MSD, Janssen, and Bristol and received support for attending meetings and/or travel from Celgene, AbbVie, Pfizer, Roche, Lilly, MSD, Janssen, and Bristol. F. Sivera received grants from Roche. Iván Ferraz-Amaro received grants/research supports from AbbVie, MSD, Janssen, and Roche and received consultation fees from company-sponsored speaker’s bureau associated with AbbVie, Pfizer, Roche, Sanofi, Sobi, Amgen, Celgene, and MSD and received support for attending meetings and/or travel from AbbVie, Pfizer, Roche, Sanofi, Sobi, Amgen, Celgene, and MSD. Mónica Calderón-Goercke received support for attending meetings and/or travel from Lilly, AbbVie, Pfizer. José L. Hernández received grants from Amgen and had participation in company-sponsored speaker’s bureau from Amgen, MSD, Bayer, and Esteve. Miguel A. Gonzalez-Gay has received grants/research supports from AbbVie, MSD, Janssen, and Roche and had consultation fees/participation in company-sponsored speaker’s bureau from AbbVie, Pfizer, Roche, Sanofi, Lilly, Celgene, and MSD and received support for attending meetings and/or travel from AbbVie, Pfizer, Roche, Sanofi, Lilly, Celgene, and MSD. R. Blanco received grants/research supports from AbbVie, MSD, and Roche, and had consultation fees/participation in company-sponsored speaker’s bureau from AbbVie, Pfizer, Roche, Bristol-Myers, Lilly, Janssen, and MSD and received support for attending meetings and/or travel from AbbVie, Pfizer, Roche, Bristol-Myers, Lilly, Janssen, and MSD. The other co-authors have declared no competing interests., (© The Author(s), 2022.)

Objective: To compare the efficacy and safety of TCZ in monotherapy (TCZ MONO ) vs. combined with conventional immunosuppressive drugs (TCZ COMBO ) in Giant Cell Arteritis (GCA) in a clinical practice scenario., Methods: Multicenter study of 134 patients with refractory GCA. Patients on TCZ MONO (n = 82) were compared with those on TCZ COMBO (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 TCZ COMBO 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 TCZ COMBO , the improvement was similar in both groups at 12 months. Moreover, in the TCZ COMBO 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., Competing Interests: Declaration of Competing Interest Disclosures that might be interpreted as constituting possible conflict(s) of interest for the study: Dr. Mónica Calderón-Goercke attendance at conferences Lilly, Abbvie, and Pfizer. Dr. S. Castañeda is assistant professor, cátedra EPID-Future, funded by UAM-Roche, Universidad Autónoma de Madrid (UAM). Dr. Vicente Aldasoro had consultation fees/participation in company-sponsored speaker´s bureau from Janssen, BMS, MSD, Roche, Sanofi, Pfizer, Novartis, Amgen, Lilly, Abbvie, Gebro, Nordic, Lacer, Alter, UCB, ASAC Pharma, Menarini and Celgene. Dr. Eva Pérez-Pampín had consultation fees/participation in company-sponsored speaker´s bureau from MSD, BMS, Janssen, Abbvie, Novartis, Pfizer, Roche, UCB, Lilly, Celgene, and Nordic. Dr. Francisca Sivera received grants from Roche. Dr. Eugenio De Miguel had consultation fees/participation in company-sponsored speaker´s bureau from Abbvie, Novartis, Pfizer, BMS, MSD, UCB, Roche, Grünenthal and Janssen. Dr. Diana Prieto-Peña attendance at conferences Lilly, Roche, and Pfizer. Prof. José L. Hernández received grants from Amgen and had participation in company-sponsored speaker´s bureau from Amgen, MSD, Bayer, and Esteve. Dr. MA Gonzalez-Gay received grants/research supports from Abbvie, MSD, and Roche, and had consultation fees/participation in company-sponsored speaker´s bureau from Pfizer, Celgene, Novartis, Roche, Sanofi, and Lilly. Dr. R Blanco received grants/research supports from Abbvie, MSD, and Roche, and had consultation fees/participation in company-sponsored speaker´s bureau from Abbvie, Pfizer, Roche, BMS, Janssen, and MSD. No financial disclosures declared: Ignacio Villa, Clara Moriano, Susana Romero-Yuste, Javier Narváez, Catalina Gómez-Arango, Rafael Melero, Elena Becerra-Fernández, Marcelino Revenga, Noelia Álvarez-Rivas, and Carles Galisteo., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objectives: A potential point of concern among clinicians is whether results derived from the clinical trials can be reasonably applied or generalised to a definable group of patients seen in real world. It can be the case of the GiACTA study that is a phase III randomised controlled trial of tocilizumab (TCZ) in giant cell arteritis (GCA). To address this question, we compared the clinical features and the responses to TCZ from the GiACTA trial patients with those from a series of GCA seen in the daily clinical practice., Methods: Comparative study of clinical features between patients from the GiACTA trial (overall n=251) and those from a multicentre series of real-world GCA patients undergoing TCZ therapy (n=134). The diagnosis of GCA in the GiACTA trial was established by the ACR modified criteria whereas in the series of real-world patients it was made by using the ACR criteria, a positive biopsy of temporal artery or the presence of imaging techniques consistent with large-vessel vasculitis in individuals who presented cranial symptoms of GCA. GiACTA trial patients received subcutaneous TCZ (162 mg every 1 or 2 weeks) whereas those from the clinical practice series were treated using standard IV dose (8 mg/kg/month) or subcutaneous (162 mg/week)., Results: Real-life patients undergoing TCZ were older with longer disease duration and higher values of ESR and had received conventional immunosuppressive therapy (mainly methotrexate) more commonly than those included in the GiACTA trial. Despite clinical differences, TCZ was equally effective in both GiACTA trial and clinical practice patients. However, serious infections were more commonly observed in GCA patients recruited from the clinical practice., Conclusions: Despite clinical differences with patients recruited in clinical trials, data from real-life patients confirm the efficacy of TCZ in GCA.

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 69years (interquartile range [IQR] 62-72.2). Three months of treatment with tocilizumab improved symptoms, erythrocyte sedimentation rate and C-reactive protein level (P<.001 and P<.012, respectively) in the 6 patients in whom it was used., Conclusions: Tocilizumab was an effective and safe treatment in those patients diagnosed with aortitis refractory to steroids and conventional immunosuppressive therapy., (Copyright © 2018 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

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 vs. >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., (Copyright © 2019 Elsevier Inc. All rights reserved.)

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. Hemophagocitc 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., (Copyright © 2014 Elsevier España, S.L.U. All rights reserved.)

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., (Copyright © 2013 Elsevier España, S.L. All rights reserved.)