BackgroundTraditionally, giant cell arteritis (GCA) was described as a disease of the temporal arteries. It is now accepted that the clinical spectrum has become broader and different diagnostic tools such as PET and US have made possible to diagnose patients who have not been included in the last decades, especially in extracranial forms. Moreover, recently, new diagnosed criteria (2022 ACR/EULAR) have been proposed. This implies an evident change of the clinical epidemiology of GCA.ObjectivesTo assess the clinical epidemiology of GCA accordingly to new diagnostic tools and 2022 ACR/EULAR criteria.MethodsObservational study of patients diagnosed with GCA who underwent temporal artery biopsy (TAB) between January 2016 and December,2022. The patients were divided into 3 groups:a) only cranial (cGCA),b) only extracranial (ecGCA), andc) mixed affection (mixGCA). The diagnosis of GCA was made according toa) temporal artery biopsy, and/orb) EULAR/ACR2022 criteria, and/orc) imaging techniques. Demographic, clinical, analytical and imaging techniques characteristics were studied.ResultsWe included 191 patients (120 females/71 males), with a mean±SD age of 74.9±9.6 years. The main characteristics of the patients and the differences among phenotypes are shown inTable 1. Only a 27.2% of the patients had a positive TAB with a mean±SD length of 16.4±6.3mm.The GCA phenotype most frequent was the cranial GCA (cGCA). Headache (79.6%) was the most common ischemic manifestations, followed by the abnormal examination of temporal artery (46.6%) and visual symptoms (including 8.4% patients with blindness), jaw or lingual claudication (26.3%), and scalp tenderness (25.1%). Polymyalgia rheumatica (PmR) was the most frequent systemic manifestations, observing in 59.2% of the patients. The values of serum CRP and ESR are shown inTable 1.ConclusionGCA is a vasculitis which has increased its clinical spectrum with extracranial involvement, affecting people with a mean age of more than 70 years and with a female predilection. PmR appears to be present in more than half of the patients, mainly in extracranial phenotype.Table 1.Main features of the GCA patients.TOTAL (n=191)cGCA (n=128)ecGCA (n=28)mixGCA (n=36)p (cGCA vs ecGCA)Age (years), mean±SD75 ± 1075 ± 1074 ± 974 ± 90.55Sex, female (% females)120 (63)79 (62)16 (57)25 (69)0.62TAB+, n (%)52 (27)39 (31)2 (7)11 (31)0.010ACR1990 Criteria, n (%)128 (67)95 (75)6 (21)27 (75)<0.001EULAR/ACR2022 Criteria, n (%)155 (81)108 (85)14 (50)33 (92)<0.001Ischemic manifestations, n (%)-Headache-Scalp tenderness-Abnormal examination of TA-Visual impairment-Jaw/lingual claudication152(80)48(25)89(47)76(40)50(26)115(91)39(31)64(50)62(49)40(32)7(25)0(0)7(25)4(14)3(11)30 (83)9 (25)18 (50)10 (28)7 (19)<0.001<0.0010.0150.0010.034Systemic manifestations, n (%)-Fever-PmR27 (14)113 (59)16 (13)62 (49)5 (18)24 (86)6 (17)27 (75)0.54<0.001Laboratory-CPR, mg/dL, median [IQR]-ESR, mm 1ºh, mean±SD2.5 [0.5-7.7]56 ± 352.9 [0.4-7.7]54 ± 342.2 [0.6-8.0]63 ± 412.3[0.5-6.6]56 ± 300.920.26Predinsone therapy- Dose (mg/day), median [IQR]- Cumulative dose (mg), median [IQR]- Time (days) from GC onset and TAB, median [IQR]40 [20-45]569 [240-1875]9 [4-21]40 [30-50]625 [285-1925]9 [3-17]30 [10-40]330 [169-473]8 (5-30)40 [15-45]650 [200-1875]10 [5-25]0.150.820.45Abbreviations: ACR: American College of Rheumatology, cGCA: cranial giant cell arteritis, ecGCA: extracranial giant cell arteritis, EULAR: European League Against Rheumatism, GCA: giant cell arteritis, mixGCA: mixed giant cell arteritis, PmR: polymyalgia rheumatica, TA: temporal artery, TAB: temporal artery biopsy.Figure 1.Distribution of GCA phenotypes in different age groups. All data are in % (n).REFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsNone Declared.

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

BackgroundGlucocorticoids (GC) are the mainstay therapy in Giant Cell Arteritis (GCA), initially at high doses (40-60 mg/day) followed by gradual glucocorticoid tapering. This treatment, especially in older patients, is associated with numerous adverse effects (AE). In addition, there are frequent relapses. Therefore, conventional synthetic immunosuppressants such as methotrexate (MTX), leflunomide, azathioprine, cyclophosphamide or mycophenolate, have been used with controversial results. Studies with biological immunosuppressants, such as TNFi have been ineffective; in contrast, tocilizumab (TCZ) has obtained positive results and was approved for the treatment of GCA.ObjectivesIn the ARTESER study we describe a) treatment with GC, synthetic or biological immunosuppressants; b) AE of CG; and c) evolution.MethodsARTESER is a retrospective observational study sponsored by the Spanish Society of Rheumatology. 26 Spanish centers participated and all new patients diagnosed with GCA from June 1, 2013 to March 29, 2019 were included. Data on GC and immunosuppressants were collected at the beginning and during the follow-up of GCA patients. For the calculation of the cumulative dose of GC, an application was developed that, by including the periods of time, dose and type of GC received during follow-up, performs the automatic calculation in mg of prednisone.ResultsOf the 1675 patients included, GC treatment was adequately recorded in 1650 patients (Table 1). All received oral treatment, being prednisone the most frequently drug used (N=1602, 97.09%). In addition, 426 (25.82%) patients received at least one iv pulse of methylprednisolone, being the 1000 mg regimen the most frequent (n=217; 50.9%). The total mean duration of GC treatment was 22.65 months. The mean cumulative dose per patient at the end of follow-up was 8514.98 mg of prednisone.Table 1.Corticosteroid treatment and immunosuppressive treatmentPatients taking oral corticosteroid1650 Prednisone, n (%)1602 (97.09) Methylprednisolone, n (%)164 (9.94) Deflazacort, n (%)64 (3.88)Patients receiving intravenous corticosteroid, n (%)426 (25.82)Mean duration of steroid treatment, mean (SD)22.65 (17.36)Mean cumulative dose at the end of follow-up per patient, mg of prednisone, mean (SD)8514.98 (6570.21)Methotrexate at diagnosis*, n (%)165 (9.9)Leflunomide at diagnosis*, n (%)2 (0.1)Azathioprine at diagnosis*, n (%)3 (0.2)Cyclophosphamide at diagnosis*, n (%)7 (0.4)Mycophenolate at diagnosis*, n (%)1 (0.1)Tocilizumab at diagnosis*, n (%)22 (1.3)Methotrexate during follow-up, n (%)532 (31.8)Leflunomide during follow-up, n (%)19 (1.2)Azathioprine during follow-up, n (%)26 (1.5)Cyclophosphamide during follow-up, n (%)10 (0.6)Mycophenolate during follow-up, n (%)10 (0.6)Tocilizumab during follow-up, n (%)153 (9.1)The most widely used immunosuppressant was MTX both at diagnosis (n=165; 9.9%) and during follow-up (n=532; 31.8%), followed by TCZ, at diagnosis (22; 1.3%) and at follow-up (153; 9.1%).AE with GC were described in 393 patients (23.8%), highlighting serious infections (n=67; 10.03%) followed by diabetes mellitus (n=63; 9.43%), steroid myopathy (n=53; 7.9%), vertebral fractures (n=47; 7.04%), non-vertebral fractures (n=36; 5.39%), heart failure (n=36; 5.39%), arterial hypertension (n=34; 5.09%) and neuropsychiatric alterations (n=27; 4.04%).During the follow-up, 334 (19.9%) patients had relapses, 532 (31.8%) were hospitalized on some occasion, and 142 patients (8.48%) died. The main cause of death were infections (n=44; 30.99%), neoplasms (n=23; 16.2%), cardiovascular (n=15; 10.56%), and cerebrovascular (n=10; 7.04%).ConclusionThe main treatment for GCA was oral GC, which were required for almost two years on average, in a quarter of patients associated with IV pulses. The cumulative steroid dose was high as well as the side effects. MTX was the most widely used immunosuppressant and TCZ was prescribed in 10%. Relapses and admissions at the hospital were relatively frequent.AcknowledgementsThis study has been funded by ROCHE Farma. The funder has not participated in the design, analysis, or interpretation of the resultsDisclosure of InterestsNone declared