The Joint Vasculitis Registry in German-speaking countries (GeVas): subgroup analysis of 195 GCA patients.

OBJECTIVES: Giant cell arteritis (GCA) is one of the most common forms of vasculitis. There is an abundance of studies which are conducted in a randomised controlled trial setting but limited with respect to cohort size and follow-up time. GeVas is the first large-scale registry for vasculitides in German-speaking countries that enables to evaluate this rare disease. Herein we focus on the subgroup of GCA patients including follow-up data up to one year. METHODS: GeVas is a prospective, web-based, multicentre registry for the documentation of organ manifestations, outcomes, and therapy regimens in vasculitides. Recruitment started in June 2019. By April 2023, 15 centres were initiated and have started to enrol patients. RESULTS: After 4 years, 195 GCA-patients were included in the registry, of which 64% were female and 36% were male. The average age was 76 years at the time of recruitment (IQR=69-82). Seventy-nine percent were included in the registry because of a newly diagnosed GCA and 21% because of a relapse. At the first assessment most of the patients (89%) described general symptoms. Thirty-one percent stated ocular symptoms. Cranial symptoms were documented in 78% of the cases. All patients were documented with immunosuppressive treatment at start, of whom 95% received prednisolone, 16% cyclophosphamide, 20% methotrexate, and 48% tocilizumab. After three months 62% and after one year 91% of the patients achieved remission. CONCLUSIONS: Regarding demographics, clinical manifestations and diagnostics, our study showed a similar composition compared to other studies. However, our data differed in terms of treatment regimens.

Análisis coste/efectivo del diagnóstico de la arteritis de la temporal / Cost-effectiveness analysis of the diagnosis of temporal arteritis

La arteritis de la temporal (AT) es la forma más frecuente de vasculitis sistémica, su diagnóstico está basado en criterios propuestos por el Colegio Americano de Reumatología (1990), y su tratamiento son corticoides a dosis elevadas. Nuestro objetivo es valorar el gasto del diagnóstico de la AT, y secundariamente análisis coste/efectivo de distintas estrategias diagnósticas (clínica, biopsia, eco-Doppler) y terapéuticas (suspensión del corticoide). Material y método: Estudio observacional, retrospectivo de pacientes con AT (2012-2021). Se recogieron datos demográficos, comorbilidades, signos y síntomas sugestivos de AT. Se diagnosticó AT con una puntuación ≥3 según los criterios del American College of Reumatology (ACR-SCORE). Se analizaron los gastos del diagnóstico y modificación de tratamiento. Resultados: Setenta y cinco pacientes, mediana edad 77 (6-87) años. Cefalea, dolor temporal y claudicación mandibular fueron significativos para el diagnóstico de AT. Los pacientes con halo en eco-Doppler y biopsia positiva, presentaron elevación de VSG y PCR de forma significativa en comparación con los pacientes que no. El gasto diagnóstico de AT fue de 414,7€/paciente. Si empleamos ACR-SCORE≥3-eco-Doppler serían 167,2€/paciente (ahorro del 59,6%) y ACR-SCORE≥3-biopsia 339,75€/paciente (ahorro del 18%). Si se retiraba corticoide y se realizaba biopsia hubiesen sido 21,6€/paciente (ahorro del 94,7%), si se retiraba corticoide y se realizaba eco-Doppler hubiesen sido 10,6€/paciente (ahorro del 97,4%). Conclusiones: Cefalea, dolor temporal y claudicación mandibular son predictores de AT. La elevación de VSG y PCR son predictores de biopsia positiva y presencia de halo en la ecografía.El empleo de ACR-SCORE≥3 con eco-Doppler o con biopsia, y con suspensión del corticoide son coste/efectivos.(AU)

Temporal arteritis (TA) is the most common form of systemic vasculitis. Its diagnosis is based on criteria proposed by the American College of Rheumatology (1990), and its treatment is high-dose corticosteroids. Our objective is to assess the cost of diagnosing TA, and secondarily, cost-effective analysis of different diagnostic strategies (clinical, biopsy, Doppler ultrasound) and therapeutic strategies (corticosteroid suspension).Material and method: Observational, retrospective study has been carried out on patients with TA (2012–2021). Demographic data, comorbidities, signs and symptoms suggestive of TA were collected. TA was diagnosed with a score ≥3 according to American College of Rheumatoloy criteria (ACR-SCORE). The costs of diagnosis and treatment modification were analysed. Results: Seventy-five patients have been included, median age 77 (46-87) years. Headache, temporal pain and jaw claudication were significant for the diagnosis of TA. Patients with a halo on Doppler ultrasound and a positive biopsy have significantly elevated ESR and CRP compared to patients who do not.: The cost of the TA diagnosis was 414.7 euros/patient. If we use ACR-SCORE≥3-echodoppler it is 167.2 €/patient (savings 59.6%) and ACR-SCORE≥3-biopsy 339.75 €/patient (savings 18%). If the corticosteroid was removed and a biopsy was performed, 21.6 €/patient (94.7% savings), if the corticosteroid was removed and Doppler ultrasound was performed, 10.6 €/patient (97.4% savings).Conclusions: Headache, temporary pain and jaw claudication are predictors of TA. Elevated ESR and CRP are predictors of positive biopsy and presence of halo on ultrasound. The uses of ACR-SCORE≥3 with Doppler ultrasound or biopsy, and with corticosteroid suspension, are cost-effective.(AU)

Cost-effectiveness analysis of the diagnosis of temporal arteritis.

Temporal arteritis (TA) is the most common form of systemic vasculitis. Its diagnosis is based on criteria proposed by the American College of Rheumatology (1990), and its treatment is high-dose corticosteroids. Our objective is to assess the cost of diagnosing TA, and secondarily, cost-effective analysis of different diagnostic strategies (clinical, biopsy, doppler ultrasound) and therapeutic strategies (corticosteroid suspension). MATERIAL AND METHOD: Observational, retrospective study has been carried out on patients with AT (2012-2021). Demographic data, comorbidities, signs and symptoms suggestive of AT were collected. AT was diagnosed with a score ≥ 3 according to American College of Rheumatoloy criteria (ACR-SCORE). The costs of diagnosis and treatment modification were analysed. RESULTS: Seventy-five patients have been included, median age 77 (46-87) years. Headache, temporal pain and jaw claudication were significant for the diagnosis of TA. Patients with a halo on Doppler ultrasound and a positive biopsy have significantly elevated ESR and CRP compared to patients who do not. The cost of the AT diagnosis was 414.7 euros/patient. If we use ACR-SCORE ≥ 3-echodoppler it is 167.2 є/patient (savings 59.6%) and ACR-SCORE ≥ 3-biopsy 339.75 є/patient (savings 18%). If the corticosteroid was removed and a biopsy was performed, 21.6 є/patient (94.7% savings), if the corticosteroid was removed and Doppler ultrasound was performed, 10.6 є/patient (97.4% savings). CONCLUSIONS: Headache, temporary pain and jaw claudication are predictors of AT. Elevated ESR and CRP are predictors of positive biopsy and presence of halo on ultrasound. The uses of ACR-SCORE ≥ 3 with Doppler ultrasound or biopsy, and with corticosteroid suspension, are cost-effective.

Myocardial infarction in a population-based cohort of patients with biopsy-confirmed giant cell arteritis in southern Sweden.

OBJECTIVES: To determine the incidence rate (IR) of myocardial infarction (MI), relative risk of MI, and impact of incident MI on mortality in individuals with biopsy-confirmed giant cell arteritis (GCA). METHODS: MIs in individuals diagnosed with GCA 1998-2016 in Skåne, Sweden were identified by searching the SWEDEHEART register, a record of all patients receiving care for MI in a coronary care unit (CCU). The regional diagnosis database, with subsequent case review, identified GCA patients receiving care for MI outside of a CCU. A cohort of 10 reference subjects for each GCA case, matched for age, sex and area of residence, was used to calculate the incidence rate ratio (IRR) of MI in GCA to that in the general population. RESULTS: The GCA cohort comprised 1134 individuals. During 7958 person-years of follow-up, 102 were diagnosed with incident MI, yielding an IR of 12.8 per 1000 person-years (95% CI 10.3 to 15.3). The IR was highest in the 30 days following GCA diagnosis and declined thereafter. The IRR of MI in GCA to that of the background population was 1.29 (95% CI 1.05 to 1.59). Mortality was higher in GCA patients who experienced incident MI than in those without MI (HR 2.8; 95% CI 2.2 to 3.6). CONCLUSIONS: The highest incidence of MI occurs within the 30 days following diagnosis of GCA. Individuals with GCA have a moderately increased risk of MI compared with a reference population. Incident MI has a major impact on mortality in GCA.

Incidence and clinical manifestations of giant cell arteritis in Spain: results of the ARTESER register.

OBJECTIVE: This study aimed to estimate the incidence of giant cell arteritis (GCA) in Spain and to analyse its clinical manifestations, and distribution by age group, sex, geographical area and season. METHODS: We included all patients diagnosed with GCA between 1 June 2013 and 29 March 2019 at 26 hospitals of the National Health System. They had to be aged ≥50 years and have at least one positive results in an objective diagnostic test (biopsy or imaging techniques), meet 3/5 of the 1990 American College of Rheumatology classification criteria or have a clinical diagnosis based on the expert opinion of the physician in charge. We calculated incidence rate using Poisson regression and assessed the influence of age, sex, geographical area and season. RESULTS: We identified 1675 cases of GCA with a mean age at diagnosis of 76.9±8.3 years. The annual incidence was estimated at 7.42 (95% CI 6.57 to 8.27) cases of GCA per 100 000 people ≥50 years with a peak for patients aged 80-84 years (23.06 (95% CI 20.89 to 25.4)). The incidence was greater in women (10.06 (95% CI 8.7 to 11.5)) than in men (4.83 (95% CI 3.8 to 5.9)). No significant differences were found between geographical distribution and incidence throughout the year (p=0.125). The phenotypes at diagnosis were cranial in 1091 patients, extracranial in 337 patients and mixed in 170 patients. CONCLUSIONS: This is the first study to estimate the incidence of GCA in Spain at a national level. We found a predominance among women and during the ninth decade of life with no clear variability according to geographical area or seasons of the year.

Concomitant Polymyalgia Rheumatica and Giant Cell Arteritis Associated with Cystic Echinococcosis: A Rare Geriatric Case.

INTRODUCTION: Parasitic infections could be an important triggering factor for autoimmune diseases. We present a clinical case of concomitant polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) induced with cystic echinococcosis (CE). CASE PRESENTATION: A 74-year-old male was admitted with a 2-month history of progressive pain at the shoulders and hip, movement restriction, and constitutional symptoms. As a result of the examinations performed due to rheumatological complaints, PMR and GCA were diagnosed. The cystic appearance, which was incidentally detected in the liver 6 months ago and not examined at that time, was found to be hydatid cyst. Medical treatment was initiated for all three conditions and the patient's symptoms improved significantly. DISCUSSION: Parasite infections may cause various autoimmune diseases because of molecular mimicry or sustained immune activation. Echinococcus granulosus is a very complex multicellular parasite and highly immunogenic for humans. Some body parts of the parasite, the outer surface and secreted particles, stimulate the host immune system strongly. CONCLUSION: The first case in the literature of coexistence of PMR and GCA associated with CE. Autoimmune diseases should be evaluated in patients with CE. Furthermore, CE should be considered in patients with autoimmune diseases in the presence of a cyst.

[Challenges and perspectives for the treatment of giant cell arteritis]. / Défis et perspectives pour le traitement de l'artérite à cellules géantes.

Early diagnosis and prompt initiation of treatment are crucial to avoid severe complications in giant cell arteritis (GCA). The European Alliance of Associations for Rheumatology (EULAR) recommendations for the use of imaging in large vessel vasculitis have helped better define the role of different techniques for diagnosing and monitoring the disease. Regarding the treatment, corticosteroids remain the standard, and tocilizumab is the preferred corticosteroid-sparing treatment. New corticosteroid-sparing treatments and "ultra-light" corticosteroid usage regimens are also under study and could represent valid therapeutic alternatives in the future.

L'artérite à cellules géantes est une vascularite pouvant avoir de graves conséquences, telles que la cécité. Le diagnostic et l'introduction d'un traitement dans les meilleurs délais sont cruciaux pour éviter ces complications. Les recommandations de l'Alliance des associations européennes pour la rhumatologie (EULAR) sur l'utilisation de l'imagerie dans les vascularites des gros vaisseaux ont permis de mieux définir le rôle des différentes techniques pour le diagnostic et le suivi de la maladie. Concernant le traitement, les corticostéroïdes restent la référence et le tocilizumab le traitement d'épargne cortisonique de choix. De nouveaux traitements d'épargne cortisonique et des schémas d'utilisation des glucocorticoïdes à doses « ultra-faibles ¼ sont aussi en phase d'étude et pourraient représenter de futures alternatives thérapeutiques.

Glucocorticoid discontinuation rate and risk factors for relapses in a contemporary cohort of patients with giant cell arteritis.

The rates of relapses and therapy discontinuation in patients with giant cell arteritis (GCA) in the modern therapeutic era have not been defined. We aimed to evaluate the glucocorticoid (GC) discontinuation rate and the factors associated with relapses in a contemporary GCA cohort. Patient and treatment data were collected cross-sectionally at first evaluation and 2 years later (second evaluation), in a multicenter, prospective GCA cohort. Predictors of relapses were identified by logistic regression analyses. 243 patients with GCA were initially included (67% women, mean age at diagnosis: 72.1 years, median disease duration: 2 years) while 2 years later complete data for 160 patients were available and analyzed. All patients had received GCs at diagnosis (mean daily prednisolone dose: 40 mg) while during follow-up, 37% received non-biologic and 16% biologic agents, respectively. At second evaluation, 72% of patients were still on therapy (GCs: 58% and/or GC-sparing agents: 29%). Relapses occurred in 27% of patients during follow-up; by multivariable logistic regression analysis, large vessel involvement at diagnosis [odds ratio (OR) = 4.22], a cardiovascular event during follow-up (OR = 4.60) and a higher initial GC daily dose (OR = 1.04), were associated with these relapses. In this large, real-life, contemporary GCA cohort, the rates of GC discontinuation and relapses were 40% and 27%, respectively. Large vessel involvement, a higher GC dose at diagnosis and new cardiovascular events during follow-up were associated with relapses.

Temporal artery ultrasonography for the diagnosis of giant cell arteritis: a case report.

Orofacial pain is a worldwide pain problem, with many patients unable to find appropriate diagnosis and treatment. Orofacial pain includes pain arising from the odontogenic and nonodontogenic structures in the head and neck region. Dental clinicians need to have a thorough knowledge and skill to diagnose, manage, and treat patients with odontogenic pain or refer patients for treatment of nonodontogenic pain to specialists such as orofacial pain specialists, neurologists, otolaryngologists, and rheumatologists. More often, dental practitioners diagnose patients with a temporomandibular disorder (TMD), and when treatment is ineffective, term it "atypical facial pain." The first requirement for effective treatment is an accurate diagnosis. Dental clinicians must be aware of giant cell arteritis (GCA), a chronic large-vessel vasculitis, primarily affecting adults over the age of 50 years, as it frequently mimics and is misdiagnosed as TMD. GCA is associated with loss of vision, and stroke and can be a life-threatening disorder. Therefore, diagnostic testing for GCA and differential diagnosis should be common knowledge in the armamentarium of all dental clinicians. Historically, temporal artery biopsy was considered the definitive diagnostic test for GCA. Temporal artery ultrasound (TAUSG), a safe and noninvasive imaging modality, has replaced the previous diagnostic gold standard for GCA, the temporal artery biopsy, owing to its enhanced diagnostic capabilities and safety profile. The present case report describes a patient with GCA, and the role TAUSG played in the diagnosis. Case report: A 72-year-old woman presented with left-sided facial pain, jaw claudication, dysesthesia of the tongue, and episodic loss of vision of 2 years' duration. She was diagnosed with and treated for a myriad of dental conditions including endodontia and temporomandibular joint therapy with no benefit. A thorough history and physical examination, combined with serologic analysis, led to the diagnosis of GCA and TAUSG, which confirmed the diagnosis. Conclusion: This report underscores the responsibility of differential diagnosis and early recognition of GCA facilitated by TAUSG in optimizing treatment outcomes as a viable, noninvasive diagnostic tool. (Quintessence Int 2024;55:336-343; doi: 10.3290/j.qi.b4938419).

Reappraisal of large artery involvement in giant cell arteritis: a population-based cohort over 70 years.

OBJECTIVE: To evaluate the incidence and outcomes of large artery (LA) involvement among patients with giant cell arteritis (GCA) and to compare LA involvement to non-GCA patients. METHODS: The study included Olmsted County, Minnesota, USA residents with incident GCA between 1950 and 2016 with follow-up through 31 December 2020, death or migration. A population-based age-matched/sex-matched comparator cohort without GCA was assembled. LA involvement included aortic aneurysm, dissection, stenosis in the aorta or its main branches diagnosed within 1 year prior to GCA or anytime afterwards. Cumulative incidence of LA involvement was estimated; Cox models were used. RESULTS: The GCA cohort included 289 patients (77% females, 81% temporal artery biopsy positive), 106 with LA involvement.Reported cumulative incidences of LA involvement in GCA at 15 years were 14.8%, 30.2% and 49.2% for 1950-1974, 1975-1999 and 2000-2016, respectively (HR 3.48, 95% CI 1.67 to 7.27 for 2000-2016 vs 1950-1974).GCA patients had higher risk for LA involvement compared with non-GCA (HR 3.22, 95% CI 1.83 to 5.68 adjusted for age, sex, comorbidities). Thoracic aortic aneurysms were increased in GCA versus non GCA (HR 13.46, 95% CI 1.78 to 101.98) but not abdominal (HR 1.08, 95% CI 0.33 to 3.55).All-cause mortality in GCA patients improved over time (HR 0.62, 95% CI 0.41 to 0.93 in 2000-2016 vs 1950-1974) but remained significantly elevated in those with LA involvement (HR 1.89, 95% CI 1.39 to 2.56). CONCLUSIONS: LA involvement in GCA has increased over time. Patients with GCA have higher incidences of LA involvement compared with non-GCA including thoracic but not abdominal aneurysms. Mortality is increased in patients with GCA and LA involvement highlighting the need for continued surveillance.

Giant Cell Arteritis: Advances in Understanding Pathogenesis and Implications for Clinical Practice.

Giant cell arteritis (GCA) is a noninfectious granulomatous vasculitis of unknown etiology affecting individuals older than 50 years. Two forms of GCA have been identified: a cranial form involving the medium-caliber temporal artery causing temporal arteritis (TA) and an extracranial form involving the large vessels, mainly the thoracic aorta and its branches. GCA generally affects individuals with a genetic predisposition, but several epigenetic (micro)environmental factors are often critical for the onset of this vasculitis. A key role in the pathogenesis of GCA is played by cells of both the innate and adaptive immune systems, which contribute to the formation of granulomas that may include giant cells, a hallmark of the disease, and arterial tertiary follicular organs. Cells of the vessel wall cells, including vascular smooth muscle cells (VSMCs) and endothelial cells, actively contribute to vascular remodeling responsible for vascular stenosis and ischemic complications. This review will discuss new insights into the molecular and cellular pathogenetic mechanisms of GCA, as well as the implications of these findings for the development of new diagnostic biomarkers and targeted drugs that could hopefully replace glucocorticoids (GCs), still the backbone of therapy for this vasculitis.

Resumen ejecutivo sobre la optimización del abordaje multidisciplinar e integrado de la polimialgia reumática y la arteritis de células gigantes en la Comunidad de Madrid / Executive summary on the optimization of the multidisciplinary and integrated approach to polymyalgia rheumatica and giant cell arteritis in Madrid region

La polimialgia reumática y la arteritis de células gigantes pueden suponer una emergencia médica en la que el retraso en su correcto diagnóstico y manejo terapéutico pueden asociar complicaciones graves. Con el objetivo de mejorar la atención de los pacientes con estas patologías en el entorno de la Comunidad de Madrid, se diseñó un estudio para identificar las causas y las posibles soluciones para hacer frente los problemas relacionados con el diagnóstico de estas patologías. Tras un análisis preliminar, se identificaron 11 áreas de mejora relacionadas con cuatro aspectos diferenciados del proceso asistencial: coordinación y protocolos, equipamientos, formación y concienciación sobre las patologías y experiencia del paciente. De todas ellas, se priorizó resolver aquellas relacionadas con la generación de protocolos de abordaje integral de las patologías y que contemplen todas las especialidades y niveles asistenciales implicados. Otro aspecto crucial es el incremento del grado de sospecha clínica de estas patologías. (AU)

Polymyalgia rheumatica and giant cell arteritis can be a medical emergency in which a delay in correct diagnosis and therapeutic management can cause serious complications. With the aim of improving the care of patients with these pathologies in the Community of Madrid, a study was designed to identify the causes and possible solutions to address the problems related to the diagnosis of these pathologies. After the analysis, 11 areas of improvement related to four different aspects of the care process were identified: coordination and protocols, equipment, training and awareness of pathologies, and patient experience. Of all the areas identified, it was considered a priority to resolve those related to the generation of protocols for the comprehensive management of the pathologies, which include all the specialties and levels of care involved. Another crucial aspect is the increase in the degree of clinical suspicion of these pathologies. (AU)

Resumen ejecutivo sobre la optimización del abordaje multidisciplinar e integrado de la polimialgia reumática y la arteritis de células gigantes en la Comunidad de Madrid / Executive summary on the optimization of the multidisciplinary and integrated approach to polymyalgia rheumatica and giant cell arteritis in Madrid region

La polimialgia reumática y la arteritis de células gigantes pueden suponer una emergencia médica en la que el retraso en su correcto diagnóstico y manejo terapéutico pueden asociar complicaciones graves. Con el objetivo de mejorar la atención de los pacientes con estas patologías en el entorno de la Comunidad de Madrid, se diseñó un estudio para identificar las causas y las posibles soluciones para hacer frente los problemas relacionados con el diagnóstico de estas patologías. Tras un análisis preliminar, se identificaron 11 áreas de mejora relacionadas con cuatro aspectos diferenciados del proceso asistencial: coordinación y protocolos, equipamientos, formación y concienciación sobre las patologías y experiencia del paciente. De todas ellas, se priorizó resolver aquellas relacionadas con la generación de protocolos de abordaje integral de las patologías y que contemplen todas las especialidades y niveles asistenciales implicados. Otro aspecto crucial es el incremento del grado de sospecha clínica de estas patologías. (AU)

Polymyalgia rheumatica and giant cell arteritis can be a medical emergency in which a delay in correct diagnosis and therapeutic management can cause serious complications. With the aim of improving the care of patients with these pathologies in the Community of Madrid, a study was designed to identify the causes and possible solutions to address the problems related to the diagnosis of these pathologies. After the analysis, 11 areas of improvement related to four different aspects of the care process were identified: coordination and protocols, equipment, training and awareness of pathologies, and patient experience. Of all the areas identified, it was considered a priority to resolve those related to the generation of protocols for the comprehensive management of the pathologies, which include all the specialties and levels of care involved. Another crucial aspect is the increase in the degree of clinical suspicion of these pathologies. (AU)