Bilateral juvenile temporal arteritis: a case-based review.

Juvenile Temporal Arteritis (JTA) is a rare non-granulomatous vasculitis affecting the superficial temporal arteries, mostly in individuals under 45 years old. It is often misdiagnosed due to its benign nature and the absence of systemic symptoms. Herein, we present a case report of a 40-year-old woman who initially presented with painless nodules in the left temporal area. Following a biopsy, the patient developed additional nodules not only in the same temple but also on the contralateral side. Remarkably, these nodules underwent spontaneous regression without further treatment, highlighting the variability in JTA's course and distinctive response to intervention. In addition, through a systematic literature review of 43 case reports - 17 with bilateral involvement - we aimed to thoroughly understand the clinical and histopathological findings, diagnostic processes, and treatment responses in JTA, with an emphasis on cases with bilateral involvement. Findings indicate that JTA typically presents as painless or painful temporal nodules, rarely accompanied by other non-specific symptoms, making histopathological examination crucial for accurate diagnosis. Collectively, our work provides the most extensive account of bilateral JTA cases to date. It emphasizes the need for clinical awareness of this condition, contributes valuable data to the limited information available on this rare condition and serves as a stepping-stone for further inquiry. The main takeaway from this review is the variable nature of JTA and the importance of histopathology in diagnosis, which helps clinicians avoid excessive testing and overtreatment and anticipate possible spontaneous resolution.

Self-limited giant cell arteritis: The dilemma associated with its diagnosis and treatment.

Giant cell arteritis (GCA) can result in visual loss and other sequelae. An 81-year-old man presented with a one-week history of fever. He had bilateral temporal headache, jaw claudication, tenderness of the temporal arteries and a recent skin rash. A temporal artery biopsy showed typical GCA, but the symptoms were self-limiting. We continued close observation, without administering prednisolone treatment. Five months later, the symptoms did not recur, and prednisolone again was not administered. Our patient presented with an atypical course of GCA that created a clinical dilemma. The final diagnosis was self-limiting GCA.

An iconodiagnosis for Joos Vijd, as painted by the van Eycks in the Ghent Altarpiece.

The Ghent Altarpiece, a jewel of Gothic art painted by the van Eyck brothers in the fifteenth century, is particularly noteworthy for its use of an innovative dilution of oil, giving it a realistic scope that is particularly conducive to iconodiagnostic hypotheses. For the first time in the literature, we are taking a medical look at this masterpiece, and more specifically at the representation of its patron, whose identity is well known: Joos Vijd, a powerful notable from the town of Ghent, in modern-day Belgium. A vascular turgidity of the temporal artery, which can be suggestive of temporal arteritis, Hertoghe's sign and a slight ear crease were observed. These signs might be vascular lesions accentuated by Vijd's age and attest to van Eyck's virtuosity and anatomic accuracy.

Predictive Factors for Biopsy-Negative Giant Cell Arteritis and Alternative Diagnoses in a Neuro-Ophthalmology Context.

Giant cell arteritis is a challenging diagnosis for patients given the high prevalence of negative temporal artery biopsies (TAB). Despite the lack of histopathological evidence of giant cell arteritis in the TAB, patients can still have TAB-negative giant cell arteritis. The purpose of this paper is to analyse the predictors for TAB-negative giant cell arteritis and the alternative diagnosis of biopsy-negative patients without a giant cell arteritis diagnosis. A retrospective electronic database review of all TABs performed at the Royal Victorian Eye and Ear Hospital from February 2015 to May 2020. Logistic regression analysis was performed to determine predictive factors for a diagnosis of TAB-negative giant cell arteritis. In all cases, a clinical diagnosis of TAB-negative giant cell arteritis was determined by a neuro-ophthalmologist. Alternative diagnoses for negative TABs were identified and explored. A total of 368 TABs were analysed with 287 (78%) negative for histopathological evidence of GCA. Twenty-seven (9.4%) patients were diagnosed and treated as TAB-negative giant cell arteritis. The clinical predictors of a TAB-negative giant cell arteritis diagnosis were the presence of jaw claudication (OR 2.77, 95% CI 1.10-6.98) and CRP (OR 1.02, 95% CI 1.00-1.03). Alternative diagnoses included non-specific headache, non-arteritic anterior ischaemic optic neuropathy, retinal vessel occlusions, and ocular nerve palsies. Predictive factors for a diagnosis of TAB-negative giant cell arteritis were jaw claudication and an elevated CRP. Several alternative diagnoses can be considered for patients with a negative TAB in a neuro-ophthalmology context.

[Unclear cause of unilateral blindness with normal inflammation parameters]. / Unklare Ursache einseitiger Erblindung bei normwertigen Entzündungsparametern.

A 70-year-old female patient presented with unilateral blindness of the right eye. As C­reactive protein (CRP) and the erythrocyte sedimentation rate (ESR) were inconspicuous, a nonarteritic embolic occlusion was assumed; however, after detailed anamnesis large vessel vasculitis (LVV) appeared more likely, which was confirmed by the subsequent imaging diagnostics. This rare case of LVV without an increase in one of the inflammatory parameters CRP or ESR highlights the importance of the medical history and targeted diagnostic procedures.

Temporal arteritis presenting as third nerve palsy - a case report and review of literature.

Giant Cell Arteritis (GCA), also known as Temporal Arteritis, is a type of large vessel vasculitis primarily affecting the elderly population. It typically manifests with headaches, visual impairment, and jaw claudication. Although third nerve palsy as the primary presentation of GCA is rare, it has been reported in previous instances. In this report, we describe the case of a patient presenting with pupil-sparing third nerve palsy, ultimately diagnosed with GCA, and successfully managed with steroids and tocilizumab. A lady in her 80s with past medical history of well-controlled hypertension, bladder cancer in remission, a twenty-pack year smoking history, cervical and lumbar spine stenosis, and recent immunizations presented with acute onset of right-sided pupil-sparing third nerve palsy. Labs were pertinent for an elevated ESR and CRP. Brain imaging was without acute abnormalities. A temporal artery biopsy established evidence of healed arteritis and a diagnosis of GCA was made. The patient was treated with pulse-dose steroids followed by an oral steroid taper and tocilizumab. At one month follow-up, there was partial resolution in her ophthalmoplegia. We underscore the importance of considering temporal arteritis as a potential cause of third nerve palsy in the elderly before attributing it solely to microvascular ischemia, particularly in patients with constitutional features. Additionally, in our comprehensive literature review, we aim to consolidate the existing data from similar presentations, shedding light on the clinical manifestation and disease trajectory.

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.

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)

Validation of a patient-reported outcome measure for giant cell arteritis.

OBJECTIVES: GCA is systemic vasculitis manifesting as cranial, ocular or large vessel vasculitis. A prior qualitative study developed 40 candidate items to assess the impact of GCA on health-related quality of life (HRQoL). This study aimed to determine final scale structure and measurement properties of the GCA patient reported outcome (GCA-PRO) measure. METHODS: Cross-sectional study included UK patients with clinician-confirmed GCA. They completed 40 candidate items for the GCA-PRO at times 1 and 2 (3 days apart), EQ-5D-5L, ICECAP-A, CAT-PROM5 and self-report of disease activity. Rasch and exploratory factor analyses informed item reduction and established structural validity, reliability and unidimensionality of the final GCA-PRO. Evidence of validity was also established with hypothesis testing (GCA-PRO vs other PRO scores, and between participants with 'active disease' vs those 'in remission') and test-retest reliability. RESULTS: The study population consisted of 428 patients: mean (s.d.) age 74.2 (7.2), 285 (67%) female; 327 (76%) cranial GCA, 114 (26.6%) large vessel vasculitis and 142 (33.2%) ocular involvement. Rasch analysis eliminated 10 candidate GCA items and informed restructuring of response categories into four-point Likert scales. Factor analysis confirmed four domains: acute symptoms (eight items), activities of daily living (seven items), psychological (seven items) and participation (eight items). The overall scale had adequate Rasch model fit (χ2 = 25.219, degrees of freedom = 24, P = 0.394). Convergent validity with EQ5D-5L, ICECAP-A and Cat-PROM5 was confirmed through hypothesis testing. Internal consistency and test-retest reliability were excellent. CONCLUSION: The final GCA-PRO is a 30-item, four-domain scale with robust evidence of validity and reliability in measuring HRQoL in people with GCA.

Executive summary on the optimization of the multidisciplinary and integrated approach to polymyalgia rheumatica and giant cell arteritis in Madrid region.

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.

Jaw claudication and jaw stiffness in giant cell arteritis: secondary analysis of a qualitative research dataset.

Objective: Jaw symptoms can be a vital clue to the diagnosis of GCA. Guidelines recommend more intensive treatment if jaw claudication is present. We sought to explore how patients with GCA described their jaw symptoms. Methods: We carried out a secondary, qualitative analysis of interview data from 36 participants from the UK (n = 25) and Australia (n = 11), originally collected in order to develop a patient-reported outcome measure for GCA. In all cases, GCA had been confirmed by biopsy/imaging. Interview transcripts were organized within QSR NVivo 12 software and analysed using template analysis. Themes were refined through discussion among the research team, including a patient partner. Results: Twenty of 36 participants reported jaw symptoms associated with GCA. The median age of these 20 participants was 76.5 years; 60% were female. Five themes were identified: physical sensations; impact on function; impact on diet; symptom response with CSs; and attribution to other causes. Physical sensations included ache, cramp, stiffness and 'lockjaw'. Functional impacts included difficulty in eating/chewing, cleaning teeth, speaking or opening the mouth. Dietary impacts included switching to softer food. Response to CSs was not always immediate. Jaw symptoms were initially mis-attributed by some participants to arthritis, age or viral illnesses; or by health-care professionals to a dental cavity, ear infection or teeth-grinding. Conclusion: Jaw symptoms in GCA are diverse and can lead to diagnostic confusion with primary temporomandibular joint disorder, potentially contributing to delay in GCA diagnosis. Further research is needed to determine the relationship of jaw stiffness to jaw claudication.

Giant cell arteritis: incidence and phenotypic distribution in Western Norway 2013-2020.

Objectives: There is an increasing awareness of the spectrum of phenotypes in giant cell arteritis (GCA). However, there is sparse evidence concerning the phenotypic distribution which may be influenced by both genetic background and the environment. We established a cohort of all GCA-patients in the Bergen Health Area (Western Norway), to describe the phenotypic distribution and whether phenotypes differ with regards to incidence and clinical features. Methods: This is a retrospective cohort study including all GCA-patients in the Bergen Health Area from 2013-2020. Data were collected by reviewing patient records, and patients considered clinically likely GCA were included if they fulfilled at least one set of classification criteria. Temporal artery biopsy (TAB) and imaging results were used to classify the patients according to phenotype. The phenotype "cranial GCA" was used for patients with a positive TAB or halo sign on temporal artery ultrasound. "Non-cranial GCA" was used for patients with positive findings on FDG-PET/CT, MRI-, or CT angiography, or wall thickening indicative of vasculitis on ultrasound of axillary arteries. Patients with features of both these phenotypes were labeled "mixed." Patients that could not be classified due to negative or absent examination results were labeled "unclassifiable". Results: 257 patients were included. The overall incidence of GCA was 20.7 per 100,000 persons aged 50 years or older. Overall, the cranial phenotype was dominant, although more than half of the patients under 60 years of age had the non-cranial phenotype. The diagnostic delay was twice as long for patients of non-cranial and mixed phenotype compared to those of cranial phenotype. Headache was the most common clinical feature (78% of patients). Characteristic clinic features occurred less frequently in patients of non-cranial phenotype compared to cranial phenotype. Conclusion: The overall incidence for GCA was comparable to earlier reports from this region. The cranial phenotype dominated although the non-cranial phenotype was more common in patients under 60 years of age. The diagnostic delay was longer in patients with the non-cranial versus cranial phenotype, indicating a need for examination of non-cranial arteries when suspecting GCA.

Giant-cell arteritis related strokes: scoping review of mechanisms and rethinking treatment strategy?

Stroke is a rare and severe complication of giant cell arteritis (GCA). Although early diagnosis and treatment initiation are essential, the mechanism of stroke is often related to vasculitis complicated by arterial stenosis and occlusion. Its recurrence is often attributed to early steroid resistance or late GCA relapse, so immunosuppressive treatment is often reinforced. However, many questions concerning the mechanisms of stroke remain elusive, and no review to date has examined the whole data set concerning GCA-related stroke. We therefore undertook this scoping review. GCA-related stroke does not necessarily display general signs and inflammatory parameters are sometimes normal, so clinicians should observe caution. Ischemic lesions often show patterns predating watershed areas and are associated with stenosis or thrombosis of the respective arteries, which are often bilateral. Lesions predominate in the siphon in the internal carotid arteries, whereas all the vertebral arteries may be involved with a predominance in the V3-V4 segments. Ultrasonography of the cervical arteries may reveal edema of the intima (halo sign), which is highly sensitive and specific of GCA, and precedes stenosis. The brain arteries are spared although very proximal arteritis may rarely occur, if the patient has microstructural anatomical variants. Temporal artery biopsy reveals the combination of mechanisms leading to slit-like stenosis, which involves granulomatous inflammation and intimal hyperplasia. The lumen is sometimes occluded by thrombi (<15%), suggesting that embolic lesions may also occur, although imaging studies have not provided strong evidence for this. Moreover, persistence of intimal hyperplasia might explain persisting arterial stenosis, which may account for delayed stroke occurring in watershed areas. Other possible mechanisms of stroke are also discussed. Overall, GCA-related stroke mainly involves hemodynamic mechanisms. Besides early diagnosis and treatment initiation, future studies could seek to establish specific preventive or curative treatments using angioplasty or targeting intimal proliferation.

Eosinophilic Granulomatosis with Polyangiitis presenting as Vasculitis in the Temporal Artery.

A 59-year-old man presented to the Emergency Department with vision disturbance, presenting concurrently bronchial asthma and pansinusitis, with complete blood count showing marked eosinophilia (32,420/mL at 79% of white blood cells). Clinical, laboratory, and imaging investigations were unremarkable except for persisting eosinophilia. A histological examination of a biopsied temporal artery showed vasculitis in the temporal artery and concomitant granulomatous inflammation, with lymphocytes, eosinophils, and multinucleated giant cells. Based on the biopsy and a positive anti-myeloperoxidase antibody (anti-MPO/p-ANCA) result, a diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) was made. The patient was initially treated with methylprednisolone pulses and recovered vision. We consider the present case as EGPA manifesting as temporal arteritis with vision loss and treated it as such, with the patient making a full recovery without further symptomatology occurrences. The current case underlines how ANCA-associated vasculitides can rarely manifest in the form of temporal arteritis.

Increased vertebral canal diameter measured by ultrasonography as a sign of vasculitis in patients with giant cell arteritis.

Introduction: The diagnosis of giant cell arteritis (GCA) by ultrasonography including large vessels, apart from the temporal artery increases the sensibility of the study and informs about the risk of specific complications. However, there is less information about the study of these arteries, whose affection carries higher proportion of severe complications. Objectives: To describe and analyze the value of the diameter of the cervical vertebral canal of the vertebral artery (VA) as a sign of vertebral vasculitis (VV) related to GCA and estimate the risk of stroke complications. Materials and methods: Observational study of a population that includes patients with GCA with and without VA vasculitis as well as healthy subjects. We evaluated whether there were differences in VA diameter in the groups and, if so, we estimated the diagnostic capacity of the variable that best defines VA diameter using a ROC curve. Cut-off points with their associated reliability chosen thereafter. Results: There were 347 subjects included:107 with GCA of whom 37 had vertebral vasculitis, 240 healthy controls. In patients with GCA and VV, the VA diameter was increased (No GCA 3.4 mm, GCA without VV 3.6 mm, GCA with VV 5.2 mm p < 0.01). According to the ROC curves, the variable defining vertebral diameter with best diagnostic accuracy is the sum of both sides (area under the curve of 0.98). With a cut-off point of 8.45 mm, the reliability values are: sensitivity 94.1%, specificity 94.5%, PPV 82.1% and NPV 98.4%. With a cut-off point of 9.95 mm, the sensitivity is 52.9% and the specificity is 100%. Likewise, VA diameter is independently associated with the presence of stroke in the vertebrobasilar territory (OR 1.6, range 1.2-2.2). Conclusion: The VA diameter, measured as the sum of both sides, is an objectively measurable sign with very high reliability for detect vertebral vasculitis in patients with GCA. It is proposed here as a novel echographic sign, which can aid the detection of the involvement of an artery where the complications are especially serious.

Factors predicting death and cancer in patients with giant cell arteritis in Western Norway 1972-2012: a retrospective observational cohort study.

Objectives: Evidence as to whether or not giant cell arteritis (GCA) confers added risk of cancer or death is conflicting. Our aim was to identify factors predicting death or cancer in a large Norwegian GCA-cohort. Methods: This is a retrospective observational cohort study including patients diagnosed with GCA in Western Norway during 1972-2012. Patients were identified through computerized hospital records using the International Classification of Diseases coding. Medical records were reviewed and data about registered deaths and cancer occurrences were extracted from the Norwegian Cause of Death Registry and the Cancer Registry of Norway. We investigated predicting factors using Cox proportional hazards regression. Results: We identified 881 cases with a validated diagnosis of GCA (60% biopsy-verified). 490 patients (56%) died during the study period. Among 767 patients with no registered cancer prior to GCA diagnosis, 120 (16%) were diagnosed with cancer during the study period. Traditional risk factors were the main predictors of death; age at time of GCA-diagnosis [hazard ratio (HR) 2.81], smoking (HR 1.61), hypertension (HR 1.48) and previous cardiovascular disease (HR 1.26). Hemoglobin (Hb) level was also associated with risk of death with increasing Hb-levels at time of GCA-diagnosis indicating decreased risk of death (HR 0.91). Other GCA-related factors were not predictive of death. We did not identify any predictors of cancer risk. Conclusion: In our cohort of GCA-patients, the risk of death was predominantly predicted by age and traditional risk factors. We found no significant associations with regards to the risk of incident cancer.

Giant Cell Arteritis Masquerading As Migraine: A Case Report.

Giant cell arteritis, or temporal arteritis, is a chronic granulomatous vasculitis that affects large- and medium-sized arteries. An elderly male of 61 years presenting with chronic headaches for the past one year had been misdiagnosed as having migraine because of the similarity in symptoms. General examination revealed the presence of bilateral large, tortuous temporal arteries without any scalp tenderness, diminished arterial pulsations, or skin changes over the dilated arteries. A temporal artery biopsy revealed giant cell arteritis and was treated with steroids. This case report highlights the importance of considering secondary headaches, especially giant cell arteritis, in the differential diagnosis of new-onset headaches or worsening headaches in the elderly.