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A woman aged mid 70s with mild learning difficulty presented with eye symptoms, headache and shoulder pains. She was found to have bilateral posterior scleritis secondary to giant cell arteritis. Posterior scleritis is a potentially blinding condition, with ambiguous symptoms and signs, therefore diagnosis is commonly delayed. This patient was seen in General Practice as well as in Accident and Emergency, where diagnoses of acute angle closure glaucoma as well as iritis were rightfully considered. Her symptoms persisted for 11 days before ophthalmic opinion was sought. Thorough assessment of a painful red eye as well as knowledge of these red-flag signs may allow early treatment and a better outcome for patients. The Royal College of Ophthalmologists has published guidance on how to assess and manage visual loss in people with learning difficulties, including recognising ‘symptoms’ such as hesitancy on steps, eye rubbing and loss of interest in activities.

Temporal arteritis is usually caused by giant cell arteritis (GCA). However, inflammation of the temporal artery can also occur secondary to autoimmune diseases or infections.We present a remarkable case of a man in his 70s with biopsy proven temporal arteritis, who was later diagnosed with meningovascular neurosyphilis. The presentation of an acute onset monocular vision loss with inflammation of the temporal artery on biopsy appeared a GCA, misleading the physicians, as it turned out to be a manifestation of neurosyphilis.

Giant cell arteritis (GCA) typically presents with headache, scalp tenderness or visual disturbance. Other symptoms include orofacial pain, constitutional symptoms and ischaemic stroke. An 81-year-old woman with a background of type-2 diabetes and hypertension presented with headache, oral pain and right visual loss. Examination showed hypertension, nodular temporal arteries, reduced visual acuity and suspected oral candida. Inflammatory markers were raised and she was diagnosed with GCA and commenced on corticosteroids. During treatment she developed tongue ulceration, then acute vertigo and incoordination with nystagmus and ataxia. Neuroimaging confirmed bilateral, cerebellar ischaemic strokes and temporal artery biopsy was consistent with GCA. With corticosteroids and secondary prevention of stroke measures she is now functionally independent. Oral pain is an uncommon symptom of GCA and delays in recognition may lead to catastrophic consequences. Clinicians should be aware of uncommon presentations and to optimise additional ischaemic stroke risk-factors.

A 50-year-old woman was referred to rheumatology for new onset polyarthralgia and headache. She had a history of metastatic lung adenocarcinoma and was started on treatment with the programmed death 1 receptor (PD-1) antagonist pembrolizumab 2 months prior. Examination revealed left temporal artery tenderness and hand synovitis. Investigations revealed enlarged temporal artery on ultrasound imaging. On steroid treatment, she had resolution of symptoms, but due to significant steroid side effects required methotrexate and her PD-1 antagonist therapy was continued in consultation with her oncologist. Her malignant disease has remained stable, and she has improved functional status.

A 74-year-old woman presented with fever, jaw claudication and bilateral temporal pain lasting for 1 week. On examination, the bilateral temporal arteries (TAs) were nodularly swollen, tender and hypopulsatile ([figure 1A][1]). A blood test showed elevated white cell count (9.33×109/L), high

A 75-year-old Caucasian woman presented with sudden-onset multifocal scotomas in her right eye's central vision for 1 day. There were subtle white intraretinal foveal lesions that correlated with patchy inner retinal hyperreflectivity on optical coherence tomography, suggestive of paracentral acute middle maculopathy. Initial cerebrovascular work-up was negative. Review of systems was positive for lethargy and jaw claudication. The sedimentation rate and c-reactive protein were elevated, but platelet count was normal. The patient was started on 60 mg oral prednisone daily and underwent bilateral temporal artery that confirmed the diagnosis of giant cell arteritis.

Glucocorticoids are associated with serious side effects related to dosing and time of use. Unfortunately, there is no standard method for determining glucocorticoid exposure, especially in patients undergoing long-term treatment.The aim of this work was to create a free and easy-to-use web application to calculate, in a systematic way, the total cumulative dose of corticosteroids.The total cumulative dose is calculated as the sum of all periods of treatment with different doses of corticosteroids, and is expressed as the equivalent dose of prednisone in mg. Glucocorticoid doses during periods in which the available information is missing or incomplete are estimated by systematic assumptions.A simulation exercise using standard patterns of steroid use in polymyalgia rheumatica, and giant cell arteritis showed that even when the period of no information reached 50% of the time, the accuracy of the calculator had a mean absolute percentage error (MAPE)7%.This tool simplifies and standardizes the glucocorticoids cumulative dose calculation, thereby minimizing bias in the assessment of glucocorticoid cumulative dose.

Giant cell arteritis (GCA) is the most common vasculitis affecting people older than 50 years. The last decades have shed new light on the clinical paradigm of this condition, expanding its spectrum beyond cranial vessel inflammation. GCA can be now considered a multifaceted vasculitic syndrome encompassing inflammation of cranial and extra-cranial arteries and girdles, isolated or combined. Such heterogeneity often leads to diagnostic delays and increases the likelihood of acute and chronic GCA-related damage. On the other hand, the approach to suspected GCA patients has been revolutionized by the introduction of vascular ultrasound which allows a rapid, cost-effective, and non-invasive GCA diagnosis. Likewise, the use of tocilizumab is now part of the therapeutic algorithm of GCA and ensures a satisfactory disease control even in steroid-refractory patients. Nonetheless, some aspects of GCA still need to be clarified, including the clinical correlation of different histological patterns, and the prevention of long-term vascular complications. This narrative review depicts the diagnostic and therapeutic aspects of GCA most relevant in clinical practice, with a focus on clinical updates and novelties introduced over the last decade.

© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ., Objective: To develop and validate updated classification criteria for giant cell arteritis (GCA). Methods: Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in six phases: (1) identification of candidate items, (2) prospective collection of candidate items present at the time of diagnosis, (3) expert panel review of cases, (4) data-driven reduction of candidate items, (5) derivation of a points-based risk classification score in a development data set and (6) validation in an independent data set. Results: The development data set consisted of 518 cases of GCA and 536 comparators. The validation data set consisted of 238 cases of GCA and 213 comparators. Age ≥50 years at diagnosis was an absolute requirement for classification. The final criteria items and weights were as follows: positive temporal artery biopsy or temporal artery halo sign on ultrasound (+5); erythrocyte sedimentation rate ≥50 mm/hour or C reactive protein ≥10 mg/L (+3); sudden visual loss (+3); morning stiffness in shoulders or neck, jaw or tongue claudication, new temporal headache, scalp tenderness, temporal artery abnormality on vascular examination, bilateral axillary involvement on imaging and fluorodeoxyglucose-positron emission tomography activity throughout the aorta (+2 each). A patient could be classified as having GCA with a cumulative score of ≥6 points. When these criteria were tested in the validation data set, the model area under the curve was 0.91 (95% CI 0.88 to 0.94) with a sensitivity of 87.0% (95% CI 82.0% to 91.0%) and specificity of 94.8% (95% CI 91.0% to 97.4%). Conclusion: The 2022 American College of Rheumatology/EULAR GCA classification criteria are now validated for use in clinical research.

Multimodality cardiovascular imaging is an essential component of the clinical management of patients with large-vessel vasculitis (LVV), a chronic, relapsing and remitting inflammatory disease of the aorta and its major branches. Imaging is needed to confirm the initial diagnosis, to survey the extent and severity of arterial involvement, to screen for cardiovascular complications and for subsequent long-term disease monitoring. Indeed, diagnosing LVV can be challenging due to the non-specific nature of the presenting symptoms, which often evoke a broad differential. Identification of disease flares and persistent residual arteritis following conventional treatments for LVV present additional clinical challenges. However, by identifying and tracking arterial inflammation and injury, multimodality imaging can help direct the use of disease-modifying treatments that suppress inflammation and prevent or slow disease progression. Each of the non-invasive imaging modalities can provide unique and complementary information, contributing to different aspects of the overall clinical assessment. This article provides a focused review of the many roles of multimodality imaging in LVV.

Scalp necrosis is a rare complication of giant cell arteritis (GCA); however, it is a predictor of severe disease. In this case study, a patient presented with GCA complicated by polymyalgia rheumatica with scalp necrosis. An 86-year-old woman was admitted to the hospital for pulsating headache, scalp pain, jaw claudication, and generalised pain. Bilateral temporal arteries were found to be distended and pulseless, and scalp necrosis was observed in the parietal region. Simultaneous high-resolution contrast-enhanced magnetic resonance imaging (MRI) sequences of the head, shoulder, and hip showed staining around the bilateral shallow temporal arteries, shoulder, and hip joints, which was confirmed as GCA with polymyalgia rheumatica using other examination findings. After treatment with early induction remission therapy, scalp necrosis healed, but jaw claudication persisted. Six months after the start of treatment, scalp necrosis was cured to full hair growth. Despite remission induction therapy combined with tocilizumab, the patient had persistent jaw claudication for several months. At that time, a high-resolution contrast-enhanced MRI re-examination was useful in assessing disease activity. GCA with scalp necrosis may cause prolonged jaw claudication reflecting the progression of ischaemic lesions, whereas the disease activity can be accurately assessed by combining MRI studies.

Giant cell arteritis (GCA) is a chronic, inflammatory condition, primarily affecting the medium and larger arteries. The purpose of this narrative review is to describe GCA in the context of headache and facial pain, based on a case and the available current literature. Understanding the etiology, pathophysiology, the associated conditions, and the differential diagnoses is important in managing GCA.In a patient presenting with unilateral facial/head pain with disturbances of vision, GCA should be considered in the differential diagnosis. There is an association of GCA with several comorbid conditions, and infections including coronavirus-19 (COVID-19) infection. Management of GCA primarily depends upon the identification of the affected artery and prompt treatment. Permanent visual loss and other serious complications are associated with GCA. GCA is characterized by robust inflammation of large- and medium-sized arteries and marked elevation of systemic mediators of inflammation. An interdisciplinary approach of management involving the pertinent specialties is strongly recommended.

This is a case report of ‘familial giant cell arteritis’ in three siblings from northwest India. This is the first case report of ‘familial giant cell arteritis’ in a non-Caucasian family.

We describe the case of a patient who presented with symptoms of persistent headaches, left-sided facial pain and blurry vision of the left eye. The patient had recovered from a herpes zoster infection of the V1 division of the trigeminal nerve 1 month prior. Serum inflammatory markers were elevated, raising concern for temporal arteritis. Empiric high-dose prednisone was initiated. Bilateral temporal artery biopsies were performed but did not show evidence of vasculitis or multinucleated giant cells. Instead, extracellular material deposits were present within the vessel walls. Congo red staining was diagnostic for amyloidosis. Liquid chromatography and mass spectrometry identified the amyloid fibrils to be transthyretin-type (ATTR) consistent with age-related amyloidosis. Temporal artery involvement of amyloidosis is rare but when present is most often due to light chain amyloidosis. Based on our review of the literature, only a few cases of temporal artery ATTR amyloidosis have been reported.

The vasculitides encompass a group of inflammatory conditions affecting the blood vessels with severe consequences including tissue ischemia, structural abnormalities, such as aneurysms/dissections, and end organ damage. The different forms are commonly classified based on the size of the blood vessel involved as large-vessel, medium-vessel, and small-vessel vasculitis. The American College of Rheumatology/Vasculitis Foundation recently published guidelines on the management of several forms of primary systemic vasculitides. In this review, the recommendations for giant cell arteritis, Takayasu arteritis, polyarteritis nodosa, granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis are discussed. We highlight the key recommendations, aspects where they diverge from other published guidelines, controversies, and areas of uncertainty.

This prospective population-based study estimated the incidence of giant cell arteritis (GCA) in northeastern Paris. GCA cases diagnosed between 2015 and 2017 were obtained from local hospital and community-based physicians and the national health insurance system database. Criteria for inclusion were living in the study area at that time and fulfilling the 1990 American College of Rheumatology classification criteria and/or its expanded version. Cranial and large-vessel GCA cases were defined by the presence or absence of cranial signs and/or symptoms, respectively. Annual incidence was calculated by dividing the number of incident cases by the size of the study population ≥ 50 years old. Completeness of case ascertainment was assessed by a three-source capture-recapture analysis. Among the 62 included cases, 42 (68%) were women, mean (± SD) age 77.3 ± 9.1 years. The annual incidence of GCA in northeastern Paris and completeness of case ascertainment were estimated at 7.6 (95% CI 5.9-9.8) per 100,000 inhabitants ≥ 50 years old and 66% (95% CI 52-92%), respectively. Incidence increased with age, peaked at age 80-89 years, and was almost twice as high in women versus men. Large-vessel GCA cases, mean (± SD) age 68.6 ± 11.5 years, accounted for 8% of all GCA cases. In this study, GCA epidemiology was mainly driven by cases with cranial GCA signs or symptoms and incidence results were consistent with recent European and past French studies.

Objectives GCA is a large vessel vasculitis in which metabolically active immune cells play an important role. GCA diagnosis is based on CRP/ESR and temporal artery biopsies (TABs), in combination with 18F-fluorodeoxyglucose ([18F]FDG)-PET/CT relying on enhanced glucose uptake by glycolytic macrophages. Here, we studied circulating Pyruvate Kinase M2 (PKM2), a glycolytic enzyme, as a possible systemic marker of vessel wall inflammation in GCA. Methods Immunohistochemical detection of PKM2 was performed on inflamed (n = 12) and non-inflamed (n = 4) TABs from GCA patients and non-GCA (n = 9) patients. Dimeric PKM2 levels were assessed in plasma of GCA patients (n = 44), age-matched healthy controls (n = 41), metastatic melanoma patients (n = 7) and infection controls (n = 11). CRP, ESR and macrophage markers calprotectin and YKL-40 were correlated with plasma PKM2 levels. To detect the cellular source of plasma PKM2 in tissue, double IF staining was performed on inflamed GCA TABs. [18F]FDG-PET scans of 23 GCA patients were analysed and maximum standard uptake values and target to background ratios were calculated. Results PKM2 is abundantly expressed in TABs of GCA patients. Dimeric PKM2 plasma levels were elevated in GCA and correlated with CRP, ESR, calprotectin and YKL-40 levels. Elevated plasma PKM2 levels were downmodulated by glucocorticoid treatment. PKM2 was detected in both macrophages and T cells at the site of vascular inflammation. Circulating PKM2 levels correlated with average target to background ratios PET scores. Conclusion Elevated plasma PKM2 levels reflect active vessel inflammation in GCA and may assist in disease diagnosis and in disease monitoring.

The objective of this study was to describe the perioperative findings during temporal artery biopsy (TAB) and the characteristics associated with a diagnosis of giant cell arteritis (GCA). Perioperative findings were prospectively described by a single operator blinded to the clinical and laboratory characteristics of the patients on 40 consecutive TABs, of which 21 were positive (53%) for GCA. Patients with a TAB positive for GCA (TAB+) more frequently had abnormalities on palpation of the temporal artery than negative TAB (TAB-) patients (mainly pulse abolition (p=0.007), indurated artery (p=0.002), and painful artery (p=0.021)). The appearance of a big artery (p0.001), a thickened artery (p0.001), and an indurated artery at incision (p0.001) was significantly associated with a positive TAB. A multivariate model identified a big artery, no local bleeding, and pain during artery traction as being associated with TAB positivity (sensitivity 71.4% and specificity 89.5%). The appearance of the temporal artery during TAB is important in predicting the positivity of the biopsy. Whether this should influence the optimal length of the TAB warrants a prospective study.

This study analyzed the characteristics, management, and outcomes of patients with poly- myalgia rheumatica (PMR) hospitalized with acute myocardial infarction (AMI), including sensitivity analysis for presence of giant cell arteritis (GCA). Using the National Inpatient Sample (January 2004 to September 2015) and International Classification of Diseases, Ninth Revision, all AMI hospitalizations were stratified into main groups: PMR and no-PMR ; and subsequently, PMR, PMR with GCA, and GCA and no-PMR. Outcomes were all- cause mortality, major adverse cardiovascular/cerebrovascular events (MACCEs), major bleeding, and ischemic stroke as well as coronary angiography (CA) and percutaneous coronary intervention (PCI). Multivariable logistic regression was used to determine adjusted odds ratios with 95% confidence interval (95% CI). A total of 7, 622, 043 AMI hospitalizations were identified, including 22, 597 patients with PMR (0.3%) and 5, 405 patients with GCA (0.1%). Patients with PMR had higher rates of mortality (5.8% vs 5.4%, p = 0.013), MACCEs (10.2% vs 9.2%, p

We herein report a patient with giant cell arteritis (GCA) who developed pneumatosis intestinalis (PI) while she was in a clinically sustained remission phase. A 79-year-old woman with GCA involving the thoracic aorta and its first branches to the posterior tibial arteries had been treated with high-dose prednisolone. Nine weeks after initiating treatment and while in clinically sustained remission with a normal CRP level, PI and pneumoperitoneum were incidentally found during scheduled positron emission tomography-computed tomography, which also revealed slight residual inflammation of GCA. This is a very rare case of PI complicated by GCA, and we discuss the possible relationships.

This article describes the various forms of inflammatory lesions of the aorta and large arteries, including chronic periaortitis, as well as the diagnostic methods are considered. Large vessel vasculitis represent the most common entities, however, there is also an association with other rheumatological or inflammatory diseases, drug-induced or paraneoplastic entities. Instrumental imaging modalities play an important role in the diagnosis.Рассматриваются различные формы воспалительного поражения аорты и крупных артерий, включая хронический периаортит, а также методы их диагностики. К наиболее распространенным относятся васкулиты крупных сосудов, вместе с тем поражение аорты и крупных артерий возможно при различных других ревматических и воспалительных заболеваниях, вследствие лекарственной или паранеопластической реакции. Решающее значение для уточнения фазы болезни и определения тактики лечения принадлежит визуализации с помощью инструментальных методов обследования.

Giant cell arteritis (GCA) is the commonest of the large-vessel vasculitides. Aortic inflammation in patients with GCA was first described over 80 years ago, but it has only been possible to study this systematically following the development of more sophisticated imaging techniques such as computed tomography angiography, magnetic resonance angiography and positron emission tomography. Both NICE and the European League Against Rheumatism (EULAR) recognise that aortic dissection may complicate GCA but stop short of recommending routine imaging. We report a case that highlights a possible need for large-vessel imaging at the time of diagnosis and during follow-up to enable earlier recognition of aortitis and associated complications including dissection.

Reports of mortality risks among individuals with giant cell arteritis (GCA) have been mixed. Our aim was to evaluate all-cause mortality among individuals with GCA relative to the general population over time.We performed a population-based study in Ontario, Canada using health administrative data. We studied a cohort of 22,677 GCA patients ages ≥50 years that was identified using a validated case definition (with 81% positive predictive value, 100% specificity). General population comparators were residents ages ≥50 years without GCA. Deaths were ascertained from vital statistics. Annual crude, age- and sex-standardized, and age- and sex-specific all-cause mortality rates were determined for individuals with and without GCA between 2000 and 2018. Standardized mortality ratios (SMRs) were estimated.Age- and sex-standardized mortality rates were significantly higher for GCA patients than comparators, and trending to increase over time with 50.0 deaths per 1,000 GCA patients in 2000 (95% confidence interval [95% CI] 34.0-71.1) and 57.6 deaths per 1,000 GCA patients in 2018 (95% CI 50.8-65.2), whereas mortality rates in the general population significantly declined over time. The annual SMRs for GCA patients generally increased over time, with the lowest SMR occurring in 2002 (1.22 [95% CI 1.03-1.40]) and the highest in 2018 (1.92 [95% CI 1.81-2.03]). GCA mortality rates were more elevated for male patients than female patients.Over a 19-year period, mortality rates were increased among GCA patients relative to the general population, and more premature deaths were occurring in younger age groups. The relative excess mortality for GCA patients did not improve over time.

Giant cell arteritis (GCA) is the most frequent vasculitis affecting adults aged 50 years. Cardiac involvement in GCA is considered rare, and only a few cases of pericarditis have been reported. The aim of this study was to determine the characteristics and prognosis of GCA patients suffering from pericardial involvement at diagnosis.We conducted a single-centre, retrospective chart review of patients with GCA in internal medicine departments (from 2000 to 2020). Patients were identified through a centralized hospital database. We retrospectively collected demographic, clinicobiological, histological, imaging, treatment and outcome data. Patients with pericardial effusion, defined as an effusion visible on the CT-scan performed at GCA diagnosis were compared to those without pericardial involvement.Among the 250 patients with GCA, 23 patients (9.2%) had pericardial effusion on CT-scan. The comparison between the groups revealed similar distribution of age, gender, cranial symptoms and ocular ischaemic complications. Patients with pericardial effusion had a higher frequency of weight loss. They also had lower haemoglobin levels and higher platelet levels (p = 0.006 and p = 0.002, respectively), and they more frequently had positive temporal artery biopsy. There were no differences concerning the treatment, relapses, follow-up duration or deaths.This case series sheds light on GCA as a cause of unexplained pericardial effusion or symptomatic pericarditis among adults aged 50 years and elevated inflammatory biological markers. Fortunately, pericardial involvement is a benign GCA manifestation. In that context, the search for constitutional symptoms, cranial symptoms and associated signs of polymyalgia rheumatica is crucial for rapidly guiding GCA diagnosis.

Background The purpose of this study was to learn about the clinical practice of specialists who care for patients with giant cell arteritis, to verify whether they follow the diagnosis and treatment recommendations for this disease, and to identify areas for improvement. Methods A cross-sectional survey on clinical practice in 2019. The survey was completed by 167 physicians (64% rheumatologists, 27% internal medicine specialists, and 9% other specialists) who attended a course on updating giant cell arteritis treatment. We compared the clinical practice collected in the study with the latest recommendations approved by the European League Against Rheumatism (EULAR). Results The physicians surveyed cared for a median of 10 patients (interquartile range 6–30) with giant cell arteritis during their practice. As a diagnostic method, respondents used temporal artery biopsy (84%), temporal artery ultrasound (61%) or other imaging techniques (37%). As first-line therapy, respondents used high-dose glucocorticoids (at least 40 mg of prednisone, or equivalent, per day) (84%), glucocorticoids with methotrexate (7%) and glucocorticoids with tocilizumab (5%). The most frequent drugs used for relapse were methotrexate (37%) and tocilizumab (58%). Conclusion Our results indicate that the medical specialists surveyed follow the recent EULAR recommendations for giant cell arteritis diagnosis and therapy.

Despite appropriate use of corticosteroids, an important minority of patients with giant cell arteritis (GCA) develop progressive vision loss during the initial stages of the disease or during corticosteroid tapering. Tocilizumab is the only clearly effective adjunctive treatment to corticosteroids in the management of GCA, but questions regarding its efficacy specifically in the neuro-ophthalmic population and its role in mitigating vision loss have not been broached until recently.The authors queried Pubmed using the search terms "GCA" and "tocilizumab" in order to identify English-language publications either explicitly designed to evaluate the influence of tocilizumab on the ophthalmic manifestations of GCA or those which reported, but were not primarily focused on, ophthalmic outcomes.Recent retrospective analyses of populations similar to those encountered in neuro-ophthalmic practice suggest that tocilizumab is effective in decreasing the frequency of GCA relapse, the proportion of flares involving visual manifestations of GCA, and the likelihood of permanent vision loss. Data regarding the utility of tocilizumab to curtail vision loss at the time of diagnosis are limited to case reports.Compared with conventional corticosteroid monotherapy, treatment of GCA with both corticosteroids and tocilizumab may decrease the likelihood of permanent vision loss. Further prospective, collaborative investigation between rheumatologists and neuro-ophthalmologists is required to clarify the ophthalmic and socioeconomic impact of tocilizumab on the treatment of GCA.

To evaluate the diagnosis performances of halo and compression signs alone and combined, assessed by a high frequency 22-MHz probe, and test their agreement in giant cell arteritis (GCA).In this cross-sectional study on patients suspected with GCA, halo sign was defined as hypo or iso-echogenic circumferential aspect of the vessel wall in transverse or longitudinal view; and compression sign was defined as visibility of the vessel wall upon transducer-imposed compression of the artery. Agreement of the two signs was tested using the Cohen's kappa statistic.A total of 80 patients (50% women) were included with a mean age of 74.4 years. Twenty participants (25%) were ultimately treated for GCA. Halo and compression signs have respective prevalences of 35% and 48%, with respective sensitivity and specificity of 80% and 80% for the halo sign; and 85% and 65% for the compression sign. The kappa coefficient for the global agreement of the two signs was 0.67 (95% confident interval: 0.54-0.85). Combination of the two signs give a sensitivity of 80% and a specificity of 81.7%.Halo and compression signs assessed by a high frequency probe, show a good level of agreement for the diagnosis of GCA and improve ultrasound specificity when combined together.

Giant cell arteritis (GCA) is the most common vasculitis in adults and is associated with significant morbidity and mortality. Temporal artery biopsy (TAB) remains the gold standard for diagnosis in the United States; however, practices vary in the length of artery obtained and whether bilateral simultaneous biopsies are obtained.Retrospective chart review of all TABs performed at the Johns Hopkins Wilmer Eye Institute between July 1, 2007, and September 30, 2017.Five hundred eighty-six patients underwent TAB to evaluate for GCA. Of 404 unilateral biopsies, 68 (16.8%) were positive. Of 182 patients with bilateral biopsies, 25 (13.7%) had biopsies that were positive and 5 patients (2.7%) had biopsies that were discordant, meaning only 1 side was positive. There was no significant difference in the average postfixation length of positive and negative TAB specimens (positive mean length 1.38 ± 0.61 cm, negative mean length 1.39 ± 0.62 cm, P = 0.9).There is no significant association between greater length of biopsy and a positive TAB result in our data. Although the rate of positive results was not higher in the bilateral group compared with the unilateral group, 2.7% of bilateral biopsies were discordant, similar to previously published rates. Overall, this suggests that initial bilateral biopsy may increase diagnostic yield, albeit by a small amount.

Polymyalgia rheumatica (PMR) is an inflammatory rheumatic disease characterized by stiffness and aching mainly in the shoulders, neck and hip girdles. The underlying pathogenesis of PMR involves myeloid lineage activation with a high expression of pattern recognition receptors. In addition, vaccination against severe acute respiratory syndrome coronavirus 2 with mRNA-1273 functions as both an immunogen and intrinsic adjuvant. It leads to the activation of innate immunity, resulting in antibody production. We herein report the first case of PMR-like syndrome seven days after mRNA-1273 vaccination. Reassuringly, the symptoms, such as pain of the neck, shoulder girdle and pelvic girdle, as well as elevated inflammatory markers were resolved within a month without glucocorticoid or immunosuppressant administration.

ObjectivesGranulocyte-macrophage colony-stimulating factor (GM-CSF) is implicated in pathogenesis of giant cell arteritis. We evaluated the efficacy of the GM-CSF receptor antagonist mavrilimumab in maintaining disease remission.MethodsThis phase 2, double-blind, placebo-controlled trial enrolled patients with biopsy-confirmed or imaging-confirmed giant cell arteritis in 50 centres (North America, Europe, Australia). Active disease within 6 weeks of baseline was required for inclusion. Patients in glucocorticoid-induced remission were randomly assigned (3:2 ratio) to mavrilimumab 150 mg or placebo injected subcutaneously every 2 weeks. Both groups received a 26-week prednisone taper. The primary outcome was time to adjudicated flare by week 26. A prespecified secondary efficacy outcome was sustained remission at week 26 by Kaplan-Meier estimation. Safety was also assessed.ResultsOf 42 mavrilimumab recipients, flare occurred in 19% (n=8). Of 28 placebo recipients, flare occurred in 46% (n=13). Median time to flare (primary outcome) was 25.1 weeks in the placebo group, but the median was not reached in the mavrilimumab group (HR 0.38; 95% CI 0.15 to 0.92; p=0.026). Sustained remission at week 26 was 83% for mavrilimumab and 50% for placebo recipients (p=0.0038). Adverse events occurred in 78.6% (n=33) of mavrilimumab and 89.3% (n=25) of placebo recipients. No deaths or vision loss occurred in either group.ConclusionsMavrilimumab plus 26 weeks of prednisone was superior to placebo plus 26 weeks of prednisone for time to flare by week 26 and sustained remission in patients with giant cell arteritis. Longer treatment is needed to determine response durability and quantify the glucocorticoid-sparing potential of mavrilimumab.Trial registration numberClinicalTrials.gov number: NCT03827018, Europe (EUdraCT number: 2018-001003-36), and Australia (CT-2018-CTN-01 865-1).

Two clinical subsets of giant cell arteritis have been identified with different histological and CT findings. However, PET/CT findings have not been compared with temporal artery biopsy (TAB).The aims of this study were to describe clinical and histological findings in patients with giant cell arteritis according to the presence or absence of aortitis in PET/CT at the disease diagnosis, and to identify independent factors related to aortic involvement.Patients were included and followed prospectively. Clinical symptoms and TAB findings were recorded. PET/CT was performed in the first 10 days of steroid therapy. Aortitis was defined if a grade 3 uptake on visual analysis was present on arterial wall. Clinical and histological variables were compared according to the presence or absence of aortitis on PET/CT. Multivariate analysis was performed to identify independent factors related to the presence of aortitis.Twenty-seven patients (median age, 77.6 years) were included. PET/CT was performed with a median delay of 5.0 days. Aortitis was observed in 8 patients. Patients with aortitis were younger (69.9 vs 83.7 years, P = 0.04) and had less frequently ischemic manifestations (25.0% vs 84.2%, P = 0.006) than patients without aortitis. Giant multinucleated cells were more frequent on TAB from patients with aortitis (71.4% vs 16.7%), and its presence was an independent risk factor for the occurrence of aortic involvement on PET/CT (odds ratio, 12.2; P = 0.046).Our study shows that giant cells on TAB are associated with the presence of aortitis on PET/CT. Patients with aortic involvement are younger and show less frequently ischemic manifestations.

BackgroundPolymyalgia rheumatica is the second most common inflammatory rheumatic disease of people >50 years. Glucocorticoid therapy is highly effective, but many patients require treatment for several years. Effective glucocorticoid sparing agents are still needed.MethodsIn this double-blind, multi-centre phase 2/3 clinical trial, we randomly assigned 36 patients with new onset polymyalgia rheumatica from three centres to receive subcutaneous tocilizumab (162 mg per week) or placebo for 16 weeks (1:1 ratio). All patients received oral prednisone, tapered from 20 mg to 0 mg over 11 weeks.The primary endpoint was the proportion of patients in glucocorticoid-free remission at week 16; key secondary endpoints, including time to first relapse and cumulative glucocorticoid dose at weeks 16 and 24, were evaluated.ResultsFrom 20 November 2017 to 28 October 2019 39 patients were screened for eligibility; 19 patients received tocilizumab and 17 placebo. Glucocorticoid-free remission at week 16 was achieved in 12 out of 19 patients on tocilizumab (63.2%) and 2 out of 17 patients receiving placebo (11.8%, p=0.002), corresponding to an OR of 12.9 (95 % CI: 2.2 to 73.6) in favour of tocilizumab. Mean (±SD) time to first relapse was 130±13 and 82±11 days (p=0.007), respectively, and the median (IQR) cumulative glucocorticoid dose was 727 (721–842) mg and 935 (861–1244) mg (p=0.003), respectively. Serious adverse events were observed in five placebo patients and one tocilizumab patient.ConclusionIn patients with new onset polymyalgia rheumatica undergoing rapid glucocorticoid tapering, tocilizumab was superior to placebo regarding sustained glucocorticoid-free remission, time to relapse and cumulative glucocorticoid dose.Trial registration numberNCT03263715

Background/purposePreclinical vascular inflammation models have demonstrated effective suppression of arterial wall lesional T cells through inhibition of Janus kinase 3 and JAK1. However, JAK inhibition in patients with giant cell arteritis (GCA) has not been prospectively investigated.MethodsWe performed a prospective, open-label, pilot study of baricitinib (4 mg/day) with a tiered glucocorticoid (GC) entry and accelerated taper in patients with relapsing GCA.Results15 patients were enrolled (11, 73% female) with a mean age at entry of 72.4 (SD 7.2) years, median duration of GCA of 9 (IQR 7–21) months and median of 1 (1–2) prior relapse. Four (27%) patients entered the study on prednisone 30 mg/day, 6 (40%) at 20 mg/day and 5 (33%) at 10 mg/day. Fourteen patients completed 52 weeks of baricitinib. At week 52, 14/15 (93%) patients had ≥1 adverse event (AE) with the most frequent events, including infection not requiring antibiotics (n=8), infection requiring antibiotics (n=5), nausea (n=6), leg swelling (n=2), fatigue (n=2) and diarrhoea (n=1). One subject required baricitinib discontinuation due to AE. One serious adverse event was recorded. Only 1 of 14 (7%) patients relapsed during the study. The remaining 13 patients achieved steroid discontinuation and remained in disease remission during the 52-week study duration.ConclusionIn this proof-of-concept study, baricitinib at 4 mg/day was well tolerated and discontinuation of GC was allowed in most patients with relapsing GCA. Larger randomised clinical trials are needed to determine the utility of JAK inhibition in GCA.Trial registration numberNCT03026504.

Giant cell arteritis (GCA) is the most common type of systemic vasculitis in the elderly. Untreated, it can lead to irreversible blindness. Its diagnosis relies on a temporal artery biopsy (TAB). However, a proportion of patients have small vessel vasculitis (SVV) on biopsy; the prognosis of which remains unclear. The aim of this study is to compare the clinical presentation and long-term outcomes of those with SVV with negative and positive biopsies to determine whether long-term corticosteroid therapy can be avoided in these patients.Post hoc analysis of patients with suspected GCA who underwent TAB and fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) scan as part of a prospective GCA and PET cohort. Patients were divided in to 3 groups based on TAB result: positive (inflammation in the main artery wall), negative (no inflammation), and SVV (isolated vasa vasorum or periadventitial SVV). Clinical, serological, and PET/CT data of patients with SVV were compared with those with positive and those with negative biopsies.For the 58 eligible patients recruited between May 2016 and December 2017, 11 had SVV, 12 had positive, and 35 had negative biopsies. Patients with SVV had similar clinical, serological, and PET/CT findings to those with negative biopsies. Compared with those with positive biopsies, patients with SVV had lower erythrocyte sedimentation rate (25 vs 78 mm/hour; P = 0.02), platelet count (296 vs 385 ×109/L; P = 0.03), and a lower median total vascular score on PET/CT scan (1.0 vs 13.5; P = 0.01). Median prednisone dose was lower (4.8 vs 11.7 mg; P = 0.015) and fewer were on steroid-sparing agents (20% vs 67%; P = 0.043) at 6 months. The percentage of patients with a clinical diagnosis of GCA was similar between those with SVV (3/11, 27.3%) and those with negative biopsies (5/35, 14.3%; P = 0.374).Patients with SVV on TAB had similar clinical features, PET/CT findings, and 6-month outcomes to those with negative biopsies. Small vessel vasculitis can be treated as equivalent to a negative biopsy when being considered for diagnosis and treatment of GCA.

Purpose The purpose of this study was to identify which combination of imaging modalities should be used to obtain the best diagnostic performance for the non-invasive diagnosis of giant cell arteritis (GCA). Materials and methods This IRB-approved prospective single-center study enrolled participants presenting with a suspected diagnosis of GCA from December 2014 to October 2017. Participants underwent high-resolution 3T magnetic resonance imaging (MRI), temporal and extra-cranial arteries ultrasound and retinal angiography (RA), prior to temporal artery biopsy (TAB). Diagnostic accuracy of each imaging modality alone, then a combination of several imaging modalities, was evaluated. Several algorithms were constructed to test optimal combinations using McNemar test. Results Forty-five participants (24 women, 21 men) with mean age of 75.4 ± 16 (SD) years (range: 59–94 years) were enrolled; of these 43/45 (96%) had ophthalmological symptoms. Diagnosis of GCA was confirmed in 25/45 (56%) patients. Sensitivity and specificity of MRI, ultrasound and RA alone were 100% (25/25; 95% CI: 86–100) and 86% (19/22; 95% CI: 65–97), 88% (22/25; 95% CI: 69–97) and 84% (16/19; 95% CI: 60–97), 94% (15/16; 95% CI: 70–100) and 74% (14/19; 95% CI: 49–91), respectively. Sensitivity, specificity, positive predictive and negative predictive values ranged from 95 to 100% (95% CI: 77–100), 67 to 100% (95% CI: 38–100), 81 to 100% (95% CI: 61–100) and 91 to 100% (95% CI: 59–100) when combining several imaging tests, respectively. The diagnostic algorithm with the overall best diagnostic performance was the one starting with MRI, followed either by ultrasound or RA, yielding 100% sensitivity (22/22; 95% CI: 85–100%) 100% (15/15; 95% CI: 78–100) and 100% accuracy (37/37; 95% CI: 91–100). Conclusion The use of MRI as the first imaging examination followed by either ultrasound or RA reaches high degrees of performance for the diagnosis of GCA and is recommended in daily practice.

We aimed to evaluate joint and vessel uptake in patients with polymyalgia rheumatica (PMR) by FDG-PET and correlate it with clinical findings.Consecutive PMR patients, without clinical signs of giant cell arteritis, underwent a standardised clinical examination and FDG-PET/CT. Controls were consecutive subjects undergoing FDG-PET for the suspicion of neoplasm not confirmed by the examination. Uptake was evaluated by a qualitative visual score, using the liver uptake as reference and by the semi-quantitative mean standardised uptake value (SUV) and target-to-background ratio (TBR) methods.Eighty-four patients and 84 controls (55 women, median age 73 years, range 50-92 years in both groups) were studied. Sixteen patients were taking glucocorticoids (GC). PMR patients showed a higher articular uptake than controls. GC-treated patients showed uptake lower than GC-naïve patients, but still higher than controls. PMR patients showed a higher vascular uptake than controls in all districts except in the carotid arteries, when evaluated by the visual score. Conversely, the semi-quantitative approach yielded no significant differences. Forty-two patients (50%) showed PET evidence of large-vessel vasculitis (LVV), defined as uptake ≥ than that of the liver, and 11.9% showed LVV with vascular uptake higher than that of the liver. The correlation between clinical findings and uptake was scarce. Neither clinical nor laboratory findings could predict the presence of LVV.Patients with PMR show a typical joint pattern at FDG-PET. There are no clinical or laboratory predictors of LVV. Imaging appears to be the only tool to assess LVV in these patients.

Giant cell arteritis (GCA) is a vasculitis often revealed by visual signs. Diagnosis is challenging and urgent. Retinal angiography (RA) and MRI allow effective diagnosis. We compared those and proposed an imaging-based approach to diagnose GCA in ophthalmological practice.We conducted a retrospective study based on the data collected from patients suspected to have GCA on ophthalmological findings. Fluorescein (FA) and indocyanine green (ICG) RAs and MRI were performed and compared with final diagnosis.Among the 41 patients included, 25 were diagnosed with GCA. Sensitivities and specificities of FA and ICG were not different. MRI showed a higher sensitivity and specificity. The approach consisting in performing RA followed by MRI provided a better accuracy.Our study shows that RA can be supplemented by MRI in a specialized center to provide the most accurate diagnosis in GCA revealed by visual signs.