Norwegian society of rheumatology recommendations on diagnosis and treatment of patients with Polymyalgia Rheumatica: a narrative review.

BACKGROUND: To provide evidence-based, up-to-date recommendations for physicians in primary and specialist healthcare setting in diagnosing and treating patients with polymyalgia rheumatica (PMR). METHODS: The PMR working group conducted a narrative review of the available evidence in the field and wrote the draft guidelines. These guidelines were discussed and revised according to the standard operating procedures within the Norwegian Society of Rheumatology. The European Alliance of Associations for Rheumatology (EULAR) and American College of Rheumatology (ACR) recommendations for the management of PMR, the British Society for Rheumatology (BSR) guidelines for the management for PMR, the treat-to-target recommendations in giant cell arteritis and PMR and the 2023 recommendations for early referral of individuals with suspected polymyalgia rheumatica were used in particular for purpose of harmonization. RESULTS: A total of 10 recommendations have been formulated covering initial diagnostic investigations, comorbidity assessment, imaging, specialist referral criteria, treatment involving glucocorticoids and steroid-sparing agents and follow-up care. CONCLUSION: Norwegian recommendations for diagnostics and treatment to improve management and outcome in patients with PMR were developed.

Vascular ultrasound as a follow-up tool in patients with giant cell arteritis: a prospective observational cohort study.

Objectives: To evaluate relapses in giant cell arteritis (GCA), investigate the utility of vascular ultrasound to detect relapses, and develop and assess a composite score for GCA disease activity (GCAS) based on clinical symptoms, ultrasound imaging activity, and C-reactive protein (CRP). Methods: Patients with GCA were prospectively followed with scheduled visits, including assessment for clinical relapse, protocol ultrasound examination, and CRP. At each visit, patients were defined as having ultrasound remission or relapse. GCAS was calculated at every visit. Results: The study included 132 patients, with a median follow-up time of 25 months [interquartile range (IR) 21]. The clinical relapse rate was 60.6%. There were no differences in relapse rates between GCA subtypes (cranial-GCA, large vessel (LV)-GCA, and mixed-GCA) (p = 0.83). Ultrasound yielded a sensitivity of 61.2% and a specificity of 72.3% for diagnosing GCA- relapse in our cohort. In 7.7% of follow-up visits with clinical relapses, neither high CRP nor findings of ultrasound relapse were registered. In comparison, in 10.3% of follow-up visits without symptoms of clinical relapse, there were both a high CRP and findings of ultrasound relapse. Conclusion: We found moderate sensitivity and specificity for ultrasound as a monitoring tool for relapse in this prospective cohort of GCA patients. The extent or subtype of vasculitis at the diagnosis did not influence the number of relapses. Based on a combination of clinical symptoms, elevated CRP, and ultrasound findings, a composite score for GCA activity is proposed.

Risk loci involved in giant cell arteritis susceptibility: a genome-wide association study.

BACKGROUND: Giant cell arteritis is an age-related vasculitis that mainly affects the aorta and its branches in individuals aged 50 years and older. Current options for diagnosis and treatment are scarce, highlighting the need to better understand its underlying pathogenesis. Genome-wide association studies (GWAS) have emerged as a powerful tool for unravelling the pathogenic mechanisms involved in complex diseases. We aimed to characterise the genetic basis of giant cell arteritis by performing the largest GWAS of this vasculitis to date and to assess the functional consequences and clinical implications of identified risk loci. METHODS: We collected and meta-analysed genomic data from patients with giant cell arteritis and healthy controls of European ancestry from ten cohorts across Europe and North America. Eligible patients required confirmation of giant cell arteritis diagnosis by positive temporal artery biopsy, positive temporal artery doppler ultrasonography, or imaging techniques confirming large-vessel vasculitis. We assessed the functional consequences of loci associated with giant cell arteritis using cell enrichment analysis, fine-mapping, and causal gene prioritisation. We also performed a drug repurposing analysis and developed a polygenic risk score to explore the clinical implications of our findings. FINDINGS: We included a total of 3498 patients with giant cell arteritis and 15 550 controls. We identified three novel loci associated with risk of giant cell arteritis. Two loci, MFGE8 (rs8029053; p=4·96 × 10-8; OR 1·19 [95% CI 1·12-1·26]) and VTN (rs704; p=2·75 × 10-9; OR 0·84 [0·79-0·89]), were related to angiogenesis pathways and the third locus, CCDC25 (rs11782624; p=1·28 × 10-8; OR 1·18 [1·12-1·25]), was related to neutrophil extracellular traps (NETs). We also found an association between this vasculitis and HLA region and PLG. Variants associated with giant cell arteritis seemed to fulfil a specific regulatory role in crucial immune cell types. Furthermore, we identified several drugs that could represent promising candidates for treatment of this disease. The polygenic risk score model was able to identify individuals at increased risk of developing giant cell arteritis (90th percentile OR 2·87 [95% CI 2·15-3·82]; p=1·73 × 10-13). INTERPRETATION: We have found several additional loci associated with giant cell arteritis, highlighting the crucial role of angiogenesis in disease susceptibility. Our study represents a step forward in the translation of genomic findings to clinical practice in giant cell arteritis, proposing new treatments and a method to measure genetic predisposition to this vasculitis. FUNDING: Institute of Health Carlos III, Spanish Ministry of Science and Innovation, UK Medical Research Council, and National Institute for Health and Care Research.

Vasculitis distribution and clinical characteristics in giant cell arteritis: a retrospective study using the new 2022 ACR/EULAR classification criteria.

Introduction: Giant cell arteritis (GCA) is the most common vasculitis of the elderly. In recent years, advanced imaging has to a certain extent replaced temporal artery biopsy (TAB) to aid diagnosis in many institutions and helped to identify three major phenotypes of GCA, namely, cranial GCA (c-GCA), large-vessel non-cranial GCA (LV-GCA), and a combination of these two patterns called mixed-GCA, which all show different clinical patterns. Recent 2022 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria respect the changing conception and clinical practice during the last two decades. In this cohort study, we present vasculitis distribution and baseline characteristics using the 2022 ACR/EULAR classification criteria as well as the EULAR core data set. Methods: In this retrospective study from Southern Norway, we identified all patients diagnosed with GCA between 2006 and 2019 in our single-center fast-track clinic (FTC). We included all patients who were examined using ultrasound (US) of cranial as well as non-cranial large vessels at diagnosis to depict vascular distribution. EULAR core data set, ACR 1990, and 2022 ACR/EULAR classification criteria were used to characterize the cohort. Results: Seventy-seven patients were diagnosed with GCA at our institution in the aforementioned period. Seventy-one patients (92.2%) were diagnosed with the help of US and included in the further analysis. The 2022 ACR/EULAR classification criteria allocated 69 patients (97.2%), while the ACR 1990 classification criteria allocated 49 patients (69.0%) in our cohort as having GCA. Mixed-GCA was the most common type in 33 patients (46.5%). Weight loss was significantly more common in patients with large-vessel non-cranial vasculitis in LV-GCA and mixed-GCA. Headache, on the other hand, was significantly more common in patients with involvement of cranial vessels. Conclusion: Mixed GCA was the most common form of GCA in our cohort. In our study, the 2022 ACR/EULAR classification criteria seem to be a more useful tool compared with the old ACR 1990 classification criteria to allocate GCA patients diagnosed and treated at our US-based FTC as having GCA.

Exploring the impact of the national tender system on the use of costly drugs treating rheumatoid arthritis patients in ten rheumatology centers in Norway (2010-2019).

BACKGROUND: Biologic and targeted synthetic disease-modifying antirheumatic drugs (b/tsDMARDs) are highly effective in treating rheumatoid arthritis (RA), albeit high drug cost has restricted their use in many countries. As a countermeasure, Norway implemented pharmaceutical tendering as a cost-reducing strategy. The aim of this study was to assess the annual proportion of different b/tsDMARDs registered to treat RA patients under the influence of a Norwegian pharmaceutical tendering between 2010 and 2019. METHOD: The data is collected from ten Norwegian outpatient centers. The included patients are categorized as naïve, non-naïve, and current b/tsDMARD users. 13 individual b/tsDMARDs are assessed and compared with the tender rankings from each year. Overview of subcutaneous (sc) with per oral vs. intravenous (iv) and biosimilars vs. non-biosimilar are also described. RESULT: The tender-winning b/tsDMARD was the most or second most used drug in nine out of ten years for naïve users, seven for non-naïve users, and twice for current users. The average sum of the highest and second highest proportion among naïve, non-naïve, and current b/tsDMARD users were 75%, 53%, and 50% during the ten years, respectively. The tender-winning drug was iv in eight out of ten years. However, the average total proportion of sc and per oral b/tsDMARDs was about 70% for naïve b/tsDMARD users, 50% for non-naïve b/tsDMARD users, and 60% for current b/tsDMARD users. The main contributors to sc and per oral b/tsDMARD were etanercept (reference and biosimilar) and certolizumab pegol. The main contributors to iv b/tsDMARD were rituximab reference and infliximab biosimilar. Despite low-ranking offers, rituximab reference (offered as a second-line drug) often achieved a high proportion among non-naïve and current b/tsDMARD users. After the introduction of biosimilars, their average proportion was about 40%, 40%, and 20% for naïve, non-naïve, and current b/tsDMARD users, respectively. CONCLUSION: Based on observed data, a higher tender rank was associated with a higher proportion among naïve and non-naïve b/tsDMARD users. However, in most cases, sc b/tsDMARDs achieved a higher proportion with lower tender ranks than iv b/tsDMARDs with higher tender ranks.

The provisional OMERACT ultrasonography score for giant cell arteritis.

OBJECTIVES: To develop an Outcome Measures in Rheumatology (OMERACT) ultrasonography score for monitoring disease activity in giant cell arteritis (GCA) and evaluate its metric properties. METHODS: The OMERACT Instrument Selection Algorithm was followed. Forty-nine members of the OMERACT ultrasonography large vessel vasculitis working group were invited to seven Delphi rounds. An online reliability exercise was conducted using images of bilateral common temporal arteries, parietal and frontal branches as well as axillary arteries from 16 patients with GCA and 7 controls. Sensitivity to change and convergent construct validity were tested using data from a prospective cohort of patients with new GCA in which ultrasound-based intima-media thickness (IMT) measurements were conducted at weeks 1, 3, 6, 12 and 24. RESULTS: Agreement was obtained (92.7%) for the OMERACT GCA Ultrasonography Score (OGUS), calculated as follows: sum of IMT measured in every segment divided by the rounded cut-off values of IMTs in each segment. The resulting value is then divided by the number of segments available. Thirty-five members conducted the reliability exercise, the interrater intraclass correlation coefficient (ICC) for the OGUS was 0.72-0.84 and the median intrareader ICC was 0.91. The prospective cohort consisted of 52 patients. Sensitivity to change between baseline and each follow-up visit up to week 24 yielded standardised mean differences from -1.19 to -2.16, corresponding to large and very large magnitudes of change, respectively. OGUS correlated moderately with erythrocyte sedimentation rate, C reactive protein and Birmingham Vasculitis Activity Score (corrcoeff 0.37-0.48). CONCLUSION: We developed a provisional OGUS for potential use in clinical trials.

Extended ultrasound examination identifies more large vessel involvement in patients with giant cell arteritis.

OBJECTIVES: To compare limited with a more extended ultrasound examination (anteromedial ultrasound, A2-ultrasound) to detect large vessel (LV) involvement in patients with newly diagnosed GCA. METHODS: Patients with new-onset GCA were included at the time of diagnosis. All patients were examined using limited ultrasound (ultrasound of the axillary artery as visualized in the axilla) and an extended A2-ultrasound method (which also includes the carotid, vertebral, subclavian and proximal axillary arteries), in addition to temporal artery ultrasound. RESULTS: One hundred and thirty-three patients were included in the study. All patients fulfilled the criteria according to a proposed extension of the 1990 ACR classification criteria for GCA and had a positive ultrasound examination at diagnosis. Ninety-three of the 133 GCA patients (69.9%) had LV involvement when examined by extended A2-ultrasound, compared with only 56 patients (42.1%) by limited ultrasound (P < 0.001). Twelve patients (9.0%) had vasculitis of the vertebral arteries as the only LVs involved. Five patients (3.8%) would have been missed as having GCA if only limited ultrasound was performed. Forty patients (30.0%) had isolated cranial GCA, 21 patients (15.8%) had isolated large vessel GCA and 72 patients (54.1%) had mixed-GCA. CONCLUSION: Extended A2-ultrasound examination identified more patients with LV involvement than the limited ultrasound method. However, extended A2-ultrasound requires high expertise and high-end equipment and should be performed by ultrasonographers with adequate training.

Predictive models for thromboembolic events in giant cell arteritis: A US veterans health administration population-based study.

Giant cell arteritis (GCA) that affects older patients is an independent risk factor for thromboembolic events. The objective of this study was to identify predictive factors for thromboembolic events in patients with GCA and develop quantitative predictive tools (prognostic nomograms) for pulmonary embolism (PE) and deep venous thrombosis (DVT). A total of 13,029 patients with a GCA diagnosis were included in this retrospective study. We investigated potential predictors of PE and DVT using univariable and multivariable Cox regression models. Nomograms were then constructed based on the results of our Cox models. We also assessed the accuracy and predictive ability of our models by using calibration curves and cross-validation concordance index. Age, inpatient status at the time of initial diagnosis of GCA, number of admissions before diagnosis of GCA, and Charlson comorbidity index were each found to be independent predictive factors of thromboembolic events. Prognostic nomograms were then prepared based on these predictors with promising prognostic ability. The probability of developing thromboembolic events over an observation period of 5 years was estimated by with time-to-event analysis using the method of Kaplan and Meier, after stratifying patients based on predicted risk. The concordance index of the time-to-event analysis for both PE and DVT was > 0.61, indicating a good predictive performance. The proposed nomograms, based on specific predictive factors, can accurately estimate the probability of developing PE or DVT among patients with GCA.

Exploring drug cost and disease outcome in rheumatoid arthritis patients treated with biologic and targeted synthetic DMARDs in Norway in 2010-2019 - a country with a national tender system for prescription of costly drugs.

BACKGROUND: In Norway, an annual tender system for the prescription of biologic and targeted synthetic disease-modifying antirheumatic drugs (b/tsDMARDs) has been used since 2007. This study aimed to explore annual b/tsDMARDs costs and disease outcomes in Norwegian rheumatoid arthritis (RA) patients between 2010 and 2019 under the influence of the tender system. METHODS: RA patients monitored in ordinary clinical practice were recruited from 10 Norwegian centers. Data files from each center for each year were collected to explore demographics, disease outcomes, and the prescribed treatment. The cost of b/tsDMARDs was calculated based on the drug price given in the annual tender process. RESULTS: The number of registered RA patients increased from 4909 in 2010 to 9335 in 2019. The percentage of patients receiving a b/tsDMARD was 39% in 2010 and 45% in 2019. The proportion of b/tsDMARDs treated patients achieving DAS28 remission increased from 42 to 67%. The estimated mean annual cost to treat a patient on b/tsDMARDs fell by 47%, from 13.1 thousand euros (EUR) in 2010 to 6.9 thousand EUR in 2019. The mean annual cost to treat b/tsDMARDs naïve patients was reduced by 75% (13.0 thousand EUR in 2010 and 3.2 thousand EUR in 2019). CONCLUSIONS: In the period 2010-2019, b/tsDMARD treatment costs for Norwegian RA patients were significantly reduced, whereas DAS28 remission rates increased. Our data may indicate that the health authorities' intention to reduce treatment costs by implementing a tender system has been successful.

Norwegian society of rheumatology recommendations on diagnosis and treatment of patients with giant cell arteritis.

Objective: To provide clinical guidance to Norwegian Rheumatologists and other clinicians involved in diagnosing and treating patients with giant cell arteritis (GCA). Methods: The available evidence in the field was reviewed, and the GCA working group wrote draft guidelines. These guidelines were discussed and revised according to standard procedures within the Norwegian Society of Rheumatology. The European Alliance of Associations for Rheumatology (EULAR) recommendations for imaging and treatment in large vessel vasculitis and the British Society for Rheumatology (BSR) guidelines for diagnostics and treatment in GCA informed the development of the current guidelines. Results: A total of 13 recommendations were developed. Ultrasound is recommended as the primary diagnostic test. In patients with suspected GCA, treatment with high doses of Prednisolone (40-60 mg) should be initiated immediately. For patients with refractory disease or relapse, Methotrexate (MTX) should be used as the first-line adjunctive therapy, followed by tocilizumab (TCZ). Conclusion: Norwegian recommendations for diagnostics and treatment to improve management and outcome in patients with GCA were developed.

Vascular Ultrasound for Giant Cell Arteritis: Establishing a Protocol Using Vascular Sonographers in a Fast-Track Clinic in the United States.

OBJECTIVE: We developed a fast-track clinic (FTC) to expedite the evaluation of patients suspected of having giant cell arteritis (GCA) using vascular ultrasound. Though FTCs have demonstrated efficacy in Europe, no protocolized clinic in the United States has been developed. This study introduces a new FTC model unique to the United States, using vascular sonographers, and describes the protocols used to develop reliable findings. We evaluate clinical outcomes using vascular ultrasound and temporal artery biopsy (TAB). METHODS: A retrospective review included all subjects referred to the University of Washington FTC aged 50 years old or older who received both ultrasound and TAB between November 2017 and November 2019. Ultrasound was performed by a vascular sonographer trained in GCA detection. Ultrasound results were read by a vascular surgeon and reviewed by four rheumatologists certified in musculoskeletal ultrasound who had completed a course in vascular ultrasound use in GCA and large-vessel vasculitis. RESULTS: A total of 43 subjects underwent both vascular ultrasound and TAB. Six subjects had both positive ultrasound and TAB results. There were also seven positive ultrasound results in patients with negative TAB results, most due to detection of large-vessel GCA (LV-GCA). All 29 subjects with negative ultrasound results had negative TAB results. CONCLUSION: This is the first study in the United States to demonstrate a reliable FTC protocol using vascular sonographers. This protocol demonstrated good agreement between ultrasound and TAB and allowed for the detection of additional cases of LV-GCA by vascular ultrasound. Vascular ultrasound improved the rate of GCA diagnosis primarily by detecting additional cases of LV-GCA.

Enhanced Liver Fibrosis Score as a Biomarker for Vascular Damage Assessment in Patients with Takayasu Arteritis-A Pilot Study.

Takayasu Arteritis (TA) is characterized by granulomatous panarteritis, vessel wall fibrosis, and irreversible vascular impairment. The aim of this study is to explore the usefulness of the Enhanced Liver Fibrosis score (ELF), procollagen-III aminoterminal propeptide (PIIINP), tissue inhibitor of matrix metalloproteinase-1 (TIMP-1), and hyaluronic acid (HA) in assessing vascular damage in TA patients. ELF, PIIINP, TIMP-1, and HA were measured in 24 TA patients, and the results were correlated with the clinical damage indexes (VDI and TADS), an imaging damage score (CARDS), and disease activity scores (NIH and ITAS2010). A mean ELF score 8.42 (±1.12) and values higher than 7.7 (cut-off for liver fibrosis) in 21/24 (87.5%) of patients were detected. The VDI and TADS correlated significantly to ELF (p < 0.01). Additionally, a strong association across ELF and CARDS (p < 0.0001), PIIINP and CARDS (p < 0.001), and HA and CARDS (p < 0.001) was observed. No correlations of the tested biomarkers with inflammatory parameters, NIH, and ITAS2010 scores were found. To our knowledge, this is the first study that suggests the association of the serum biomarkers PIIINP, HA, and ELF score with damage but not with disease activity in TA patients. The ELF score and PIIINP may be useful biomarkers reflecting an ongoing fibrotic process and quantifying vascular damage.

Decentralizing healthcare in Norway to improve patient-centered outpatient clinic management of rheumatoid arthritis - a conceptual model.

A growing population of older adults and improved effective treatments for inflammatory rheumatic diseases will increase the demand for more healthcare resources that already struggle with staggering outpatient clinic waiting times. Transformative delivery care models that provide sustainable healthcare services are urgently needed to meet these challenges. In this mini-review article, a proposed Lifelong Treatment Model for a decentralized follow-up of outpatient clinic patients living with rheumatoid arthritis is presented and discussed.Our conceptual model follows four steps for a transformative care delivery model supported by an Integrated Practice Unit; (1) Diagnosis, (2) Treatment, (3) Patient Empowered Disease Management, and (4) Telehealth. Through an Integrated Practice Unit, a multidisciplinary team could collaborate with patients with rheumatoid arthritis to facilitate high-value care that addresses most important outcomes of the patients; (1) Early Remission, (2) Decentralization, (3) Improved Quality of Life, and (4) Lifelong Sustain Remission.The article also addresses the growing challenges for the healthcare delivery system today for patients with rheumatoid arthritis and proposes how to reduce outpatient clinic visits without compromising quality and safety.

The fast-track outpatient clinic significantly decreases hospitalisation rates among polymyalgia rheumatica patients.

OBJECTIVES: This study aimed to investigate the hospitalisation rates and the reasons for hospitalisation in patients with polymyalgia rheumatica (PMR). Furthermore, it aimed to clarify the impact of a newly established Fast Track Clinic (FTC) approach on hospitalisation rates in connection with PMR diagnosis. METHODS: Patients diagnosed with PMR at South-West Jutland Hospital, Denmark, between 2013 and 2018 were included retrospectively. Only patients fulfilling the 2012 EULAR/ACR classification criteria were included in our cohort. An FTC for patients suspected of having PMR was established in the rheumatologic department of South-West Jutland Hospital in January 2018. RESULTS: Over 6 years (2013 to 2017), 254 patients were diagnosed with PMR, 56 of them while hospitalised. Hospitalised patients were more likely to have a higher initial CRP mean ± standard deviation (SD) 99.53 ± 59.36 vs 45.82 ± 36.96 mg/lt (p <  0.0001) and a shorter duration of symptoms (p = 0.0018). After implementing the FTC, a significant decrease in hospitalisation rates (from 20.4% to 3,5%) and inpatient days of care (mean ± SD 4.15 ± 3.1 vs 1 ± 0) were observed. No differences between the two groups were observed regarding clinical symptoms, laboratory values and initial prednisolone dose. CONCLUSION: A substantial number of patients are hospitalised in connection with the PMR diagnosis. The FTC approach can decrease the hospitalisation rates significantly among these patients. TRIAL REGISTRATION: Retrospectively registered.

Diagnosing giant cell arteritis: a comprehensive practical guide for the practicing rheumatologist.

GCA is the most common large vessel vasculitis in the elderly population. In recent years, advanced imaging has changed the way GCA can be diagnosed in many locations. The GCA fast-track clinic approach combined with US examination allows prompt treatment and diagnosis with high certainty. Fast-track clinics have been shown to improve prognosis while being cost effective. However, all diagnostic modalities are highly operator dependent, and in many locations expertise in advanced imaging may not be available. In this paper, we review the current evidence on GCA diagnostics and propose a simple algorithm for diagnosing GCA for use by rheumatologists not working in specialist centres.

OMERACT definition and reliability assessment of chronic ultrasound lesions of the axillary artery in giant cell arteritis.

OBJECTIVES: To define chronic ultrasound lesions of the axillary artery (AA) in long-standing giant cell arteritis (GCA) and to evaluate the reliability of the new ultrasound definition in a web-based exercise. METHODS: A structured Delphi, involving an expert panel of the Large Vessel Vasculitis subgroup of the Outcome Measures in Rheumatology (OMERACT) Ultrasound Working Group was carried out. The reliability of the new definition was tested in a 2-round web-based exercise involving 23 experts and using 50 still images each from AA of long-standing and acute GCA patients, as well as 50 images from healthy individuals. RESULTS: The final OMERACT ultrasound definition of chronic changes was based on measurement and appearance of the intima-media complex. The overall reliability of the new definition for chronic ultrasound changes in longstanding GCA of the AA was good to excellent with Light's kappa values of 0.79-0.80 for inter-reader reliability and mean Light's-kappa of 0.88 for intra-reader reliability. The mean inter-rater and intra-rater agreements were 86-87% and 92%, respectively. Good reliabilities were observed comparing the vessels with longstanding versus acute GCA with a mean agreement and kappa values of 81% and 0.63, respectively. CONCLUSION: The new OMERACT ultrasound definition for chronic vasculitis of the AA in GCA revealed a good to excellent inter- and intra-reader reliability in a web-based exercise of experts.

Incidence Trends and Mortality of Giant Cell Arteritis in Southern Norway.

OBJECTIVE: Southern Norway consists of a homogeneous population of nearly 300,000 inhabitants and is an ideal epidemiologic setting. We aimed to explore potential changes in incidence of giant cell arteritis (GCA) in Southern Norway from 2000-2013, with comparisons of previous reports from the same population cohort from 1987-1994 and 1992-1996, and to investigate the mortality rates of GCA over a period of 14 years. METHODS: All patients diagnosed with GCA during January 1, 2000 to December 31, 2013 were identified through the electronic health records and biopsy findings databases at our clinic. The diagnosis of GCA and information about death was confirmed by reviewing the patients' hospital records. Inclusion criteria were: 1) fulfillment of the American College of Rheumatology 1990 criteria for GCA, or 2) histologically proven GCA, or 3) confirmed arteritis of the large or medium-sized vessels by imaging. RESULTS: A total of 206 patients were included, and 147 (72%) were females. The annual incidence rate of GCA per 100,000 inhabitants age ≥50 years was 16.8 (95% confidence interval [95% CI] 14.6-19.2), 24.5 for females (95% CI 19.2-26.5), and 10.2 for males (95% CI 7.9-13.2). Forty-six patients (22%) died (24 women, 22 men). The overall standardized mortality ratio was 1.05 (95% CI 0.77-1.38), 0.92 for females (95% CI 0.61-1.35), and 1.38 for males (95% CI 0.88-2.05). Overall survival rate was significantly higher in females compared to males (P < 0.001). CONCLUSION: GCA incidence is not increasing. We did not find excess mortality; however, males seem to have a worse survival rate compared to females.