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AJNR. American Journal of Neuroradiology Jun 2024Giant cell arteritis (GCA) is the most common primary large vessel systemic vasculitis in the western world. Even though the involvement of scalp and intracranial... (Review)
Review
Giant cell arteritis (GCA) is the most common primary large vessel systemic vasculitis in the western world. Even though the involvement of scalp and intracranial vessels has received much attention in the neuroradiology literature, GCA, being a systemic vasculitis can involve multiple other larger vessels including aorta and its major head and neck branches. Herein, the authors present a pictorial review of the various cranial, extracranial and orbital manifestations of GCA. An increased awareness of this entity may help with timely and accurate diagnosis, helping expedite therapy and preventing serious complications.ABBREVIATIONS: ACR= American College of Rheumatology, AION= Anterior Ischemic Optic Neuropathy, EULAR= European League Against Rheumatism, GCA= Giant Cell Arteritis, LV-GCA= Large vessel GCA, PMR= Polymyalgia Rheumatica, US= Ultrasound, VWI= Vessel Wall Imaging.
PubMed: 38906672
DOI: 10.3174/ajnr.A8388 -
Eye (London, England) Jun 2024Treatment of giant cell arteritis (GCA) aims initially to prevent acute visual loss, and subsequently to optimise long-term quality of life. Initial prevention of acute... (Review)
Review
Treatment of giant cell arteritis (GCA) aims initially to prevent acute visual loss, and subsequently to optimise long-term quality of life. Initial prevention of acute visual loss in GCA is well-standardised with high-dose glucocorticoid therapy but in the longer term optimising quality of life requires tailoring of treatment to the individual. The licensing of the IL-6 receptor inhibitor tocilizumab combined with advances in vascular imaging have resulted in many changes to diagnostic and therapeutic practice. Firstly, GCA is a systemic disease that may involve multiple vascular territories and present in diverse ways. Broadening of the "spectrum" of what is called GCA has been crystallised in the 2022 GCA classification criteria. Secondly, the vascular inflammation of GCA frequently co-exists with the extracapsular musculoskeletal inflammation of the related disease, polymyalgia rheumatica (PMR). Thirdly, GCA care must often be delivered across multiple specialities and healthcare organisations requiring effective interprofessional communication. Fourthly, both GCA and PMR may follow a chronic or multiphasic disease course; long-term management must be tailored to the individual patient's needs. In this article we focus on some areas of current rheumatology practice that ophthalmologists need to be aware of, including comprehensive assessment of extra-ocular symptoms, physical signs and laboratory markers; advanced imaging techniques; and implications for multi-speciality collaboration.
PubMed: 38898105
DOI: 10.1038/s41433-024-03153-7 -
Rheumatology (Oxford, England) Jun 2024Immune checkpoint inhibitor (ICI) associated inflammatory arthritis (ICI-IA) occurs in 4-6% of ICI-treated patients based on one observational study. We identified cases...
OBJECTIVES
Immune checkpoint inhibitor (ICI) associated inflammatory arthritis (ICI-IA) occurs in 4-6% of ICI-treated patients based on one observational study. We identified cases of ICI-IA using administrative claims to study its incidence and characteristics at the population level.
METHODS
We used the Medicare 5% sample to identify patients initiating ICIs. Cancer patients were identified by having ≥ 2 ICD-9/10-CM diagnosis codes from an oncologist for lung cancer, melanoma, or renal/urothelial cancer. ICI-IA was defined as having two Medicare claims ≥ 30 days apart with combinations of ICD-9/10-CM diagnosis codes that favored specificity. ICI-IA was identified in patients with a musculoskeletal diagnosis after ICI initiation, who had i.) no inflammatory arthritis or inflammatory rheumatic disease before ICI initiation ever, and ii) no musculoskeletal complaint in the one year prior to ICI. We examined DMARD utilization and visits to rheumatology in patients with ICI-IA. Landmark analysis and a time varying Cox proportional hazards model for overall survival was constructed.
RESULTS
The incidence of ICI-IA was 7.2 (6.1-8.4) per 100 patient years. Patients with ICI-IA were mean (SD) age 73.5(7.0) years, 48% women, 91% white. Median(IQR) time from ICI initiation to first ICI-IA diagnosis was 124(56, 252) days. Only 24(16%) received care from a rheumatologist, and 24(16%) were prescribed a DMARD (46% by a rheumatologist). The HR for mortality in patients with ICI-IA was 0.86 (95% CI 0.59-1.26, p= 0.45).
CONCLUSIONS
The incidence of ICI-IA identified in claims data is similar to that reported in observational studies, however, few patients are treated with a DMARD or see a rheumatologist. There was no difference in overall survival between ICI-treated patients with and without ICI-IA.
PubMed: 38889288
DOI: 10.1093/rheumatology/keae343 -
Expert Opinion on Investigational Drugs Jun 2024Polymyalgia rheumatica (PMR) is an inflammatory rheumatic disorder characterized by pain and stiffness in the shoulder and pelvic girdles, constitutional symptoms, and...
INTRODUCTION
Polymyalgia rheumatica (PMR) is an inflammatory rheumatic disorder characterized by pain and stiffness in the shoulder and pelvic girdles, constitutional symptoms, and elevated acute-phase reactants. Glucocorticoids (GCs) remain the first-choice treatment for PMR, but relapses are common. Identification of steroid-sparing agents is therefore of utmost importance.
AREAS COVERED
The efficacy of conventional immunosuppressive drugs is controversial. The use of interleukin (IL)-6 receptor inhibitors proved to be effective and safe in treating PMR patients. Currently, there are 12 ongoing clinical trials exploring potential treatments such as leflunomide, low-dose IL-2, rituximab, abatacept, secukinumab, Janus kinase inhibitors, and selective inhibitors like SPI-62 and ABBV 154.
EXPERT OPINION
The high efficacy of IL-6 R receptor inhibitors as well as the numerous drug trials currently recruiting suggest that several therapeutic options will be available in the near future. Accurate diagnosis and early stratification of PMR patients according to the giant cell arteritis-PMR Spectrum Disease 'GPSD' and potential risk factors for relapsing disease or GC-related adverse events are crucial to identify patients who would benefit most from GC-sparing agents. The development of internationally accepted definitions for remission and relapse is urgently needed. Early referral strategies to specialist settings would improve disease stratification and personalized treatment.
PubMed: 38879822
DOI: 10.1080/13543784.2024.2366847 -
Revista Clinica Espanola Jun 2024Systematic review of current evidence to analyze the prevalence of extracranial large vessel vasculitis (LVV) using F-FDG PET/CT in patients with polymyalgia rheumatica...
OBJECTIVE
Systematic review of current evidence to analyze the prevalence of extracranial large vessel vasculitis (LVV) using F-FDG PET/CT in patients with polymyalgia rheumatica (PMR) or giant cell arteritis (GCA).
MATERIALS AND METHODS
PubMed and EMBASE were searched and the results were screened by two reviewers. Study quality was assessed using a modified version of the Newcastle-Ottawa scale. Heterogeneity between studies was assessed using the I statistic and the Q test. Further subgroup analyses were performed by disease type, study quality, and F-FDG PET/CT uptake criteria. Publication bias was assessed by funnel plot and Egger's test.
RESULTS
268 publications were identified, of which 17 met the selection criteria and were included in the meta-analysis. The overall pooled prevalence of extracranial LVV by F-FDG PET/CT was 54.5% [95% CI: 42.6%-66.1%]. In patients with GCA the prevalence was significantly higher than in patients with PMR (60.1% vs. 41.8%, P = 0.006). Likewise, studies with a lower risk of bias reported a higher prevalence of extracranial LVV (61.1% vs. 46.9%; P = 0.010). No publication bias was observed.
CONCLUSIONS
The F-FDG PET/CT test may be useful in the detection of extracranial LVV, both in patients with PMR or GCA. Such involvement is more frequent in patients with GCA, and may vary depending on the quality of the studies.
PubMed: 38852739
DOI: 10.1016/j.rceng.2024.06.005 -
Therapeutic Advances in Musculoskeletal... 2024Rheumatologists are increasingly utilizing ultrasound for suspected giant cell arteritis (GCA) or Takayasu arteritis (TAK). This enables direct confirmation of a... (Review)
Review
Rheumatologists are increasingly utilizing ultrasound for suspected giant cell arteritis (GCA) or Takayasu arteritis (TAK). This enables direct confirmation of a suspected diagnosis within the examination room without further referrals. Rheumatologists can ask additional questions and explain findings to their patients while performing ultrasound, preferably in fast-track clinics to prevent vision loss. Vascular ultrasound for suspected vasculitis was recently integrated into rheumatology training in Germany. New European Alliance of Associations for Rheumatology recommendations prioritize ultrasound as the first imaging tool for suspected GCA and recommend it as an imaging option for suspected TAK alongside magnetic resonance imaging, positron emission tomography and computed tomography. Ultrasound is integral to the new classification criteria for GCA and TAK. Diagnosis is based on consistent clinical and ultrasound findings. Inconclusive cases require histology or additional imaging tests. Robust evidence establishes high sensitivities and specificities for ultrasound. Reliability is good among experts. Ultrasound reveals a characteristic non-compressible 'halo sign' indicating intima-media thickening (IMT) and, in acute disease, artery wall oedema. Ultrasound can further identify stenoses, occlusions and aneurysms, and IMT can be measured. In suspected GCA, ultrasound should include at least the temporal and axillary arteries bilaterally. Nearly all other arteries are accessible except the descending thoracic aorta. TAK mostly involves the common carotid and subclavian arteries. Ultrasound detects subclinical GCA in over 20% of polymyalgia rheumatica (PMR) patients without GCA symptoms. Patients with silent GCA should be treated as GCA because they experience more relapses and require higher glucocorticoid doses than PMR patients without GCA. Scores based on intima-thickness (IMT) of temporal and axillary arteries aid follow-up of GCA, particularly in trials. The IMT decreases more rapidly in temporal than in axillary arteries. Ascending aorta ultrasound helps monitor patients with extracranial GCA for the development of aneurysms. Experienced sonologists can easily identify pitfalls, which will be addressed in this article.
PubMed: 38846756
DOI: 10.1177/1759720X241251742 -
Frontiers in Medicine 2024Giant cell arteritis (GCA) is characterized by inflammation of large and medium vessels. First-line therapy for the treatment of GCA are glucocorticoids, which are...
BACKGROUND
Giant cell arteritis (GCA) is characterized by inflammation of large and medium vessels. First-line therapy for the treatment of GCA are glucocorticoids, which are effective while potential adverse effects should be considered, especially during long-term use. The aim was to investigate the incidence of glucocorticoids' adverse effects and potential predictors for them.
MATERIALS AND METHODS
138 GCA patients were retrospectively evaluated for newly developed glucocorticoid adverse effects in 2020. Potential predictors, defined as initial glucocorticoid pulse therapy, relapse of GCA and concomitant polymyalgia rheumatica as well as parameters of inflammation and endothelial dysfunction, including pulse-wave velocity and intima-media-thickness, were measured in 2012.
RESULTS
Potential new glucocorticoid adverse effects per patient was 1 (25th-75th 0-3) of which chronic kidney disease progression (29%), bone fractures (23.2%), cataracts (18.1%), dementia, and arterial hypertension (each at 12.3%) were most commonly recorded. Significant associations were found between occurrence of any relapse and new diabetes mellitus and between initial glucocorticoid pulse therapy and new dementia (all with < 0.05). In multivariate regression analysis, any relapse was a predictor for developing diabetes mellitus (OR 9.23 [95% CI 1.33-64.05], = 0.025). However, no correlations were observed between endothelial dysfunction or inflammatory parameters and development of new glucocorticoid adverse effects.
CONCLUSION
GCA relapses may be associated for development of diabetes mellitus potentially by increasing glucocorticoid doses. Parameters of inflammation and endothelial dysfunction are not suited predictors for glucocorticoid adverse effects.
PubMed: 38841591
DOI: 10.3389/fmed.2024.1382946 -
BMJ Case Reports Jun 2024We report a case of a woman in her early 80s with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis presented as myalgia mimicking polymyalgia rheumatica...
We report a case of a woman in her early 80s with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis presented as myalgia mimicking polymyalgia rheumatica (PMR). She had positive results for the Neer and Hawkins-Kennedy impingement tests, and a normal serum creatine kinase (CK) concentration. At first, we suspected PMR; however, the patient did not strictly meet the classification criteria. Electromyography revealed an abnormal myogenic pattern, and muscle MRI revealed intramuscular and fascial hyperintensity. Moreover, chest CT revealed interstitial lung disease, and test results for ANCAs were positive. We diagnosed the patient with ANCA-associated vasculitis based on the criteria and treated her with corticosteroids and rituximab. Thus, ANCA-associated vasculitis can cause muscle involvement without elevation of the CK concentration and mimic PMR.
Topics: Humans; Polymyalgia Rheumatica; Female; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Diagnosis, Differential; Aged, 80 and over; Rituximab; Magnetic Resonance Imaging; Electromyography; Myalgia
PubMed: 38839420
DOI: 10.1136/bcr-2023-257828 -
Zeitschrift Fur Rheumatologie Jun 2024A German expert committee recommends defining fast-track clinics (FTC) for the acute diagnosis of giant cell arteritis (GCA) as follows: easy and prompt reachability at...
A German expert committee recommends defining fast-track clinics (FTC) for the acute diagnosis of giant cell arteritis (GCA) as follows: easy and prompt reachability at least on weekdays, scheduling appointments ideally within 24 h, examination by a specialist with GCA expertise, ≥ 2 experts per FTC, ≥ 50 patients with suspected GCA per year, sonologists with ≥ 300 (≥ 50) temporal and axillary artery examinations, adherence to standard operating procedures, availability of an ≥ 18 (≥ 15) MHz and a lower frequency linear ultrasound probe, and collaboration with partners for neurology and ophthalmology consultations, magnetic resonance imaging (MRI), positron emission tomography-computed tomography (PET-CT, possibly CT), and for temporal artery biopsy.
PubMed: 38832967
DOI: 10.1007/s00393-024-01532-9 -
QJM : Monthly Journal of the... May 2024Giant cell arteritis (GCA) is one of the most common large vessel (LVV) vasculitis and is associated with a high risk of relapse and cardiovascular complications....
OBJECTIVE
Giant cell arteritis (GCA) is one of the most common large vessel (LVV) vasculitis and is associated with a high risk of relapse and cardiovascular complications. Improving risk stratification remains a significant issue in this patient population. We aimed to perform a cluster analysis among GCA to identify clusters and evaluate their prognostic value.
METHODS
In a multicenter cohort study, we performed hierarchical cluster analysis on the factor analysis of mixed data coordinates results with 283 GCA patients' characteristics to generate clusters and assess incidence of relapse, cardiovascular events and death.
RESULTS
Three clusters were identified: "Vascular relapsing profile" (23.0%), "Typical GCA profile" (47.7%), and "Ophthalmologic elderly profile" (29.3%). The "Vascular relapsing profile" cluster included younger patients with more frequent relapses and cardiovascular events, particularly thoracic aortic aneurysms. The "Typical GCA profile" was the largest, with classic cranial manifestations and frequently associated polymyalgia rheumatica. The "Ophthalmologic elderly profile" had the oldest patients with more visual loss and the highest mortality rate.
CONCLUSIONS
Our findings underline the varied prognostic landscape within GCA, emphasizing the poor cardiovascular prognosis of younger patients with LV involvement and the higher mortality among elderly patients. This reinforces the need for further research regarding the screening of aortic abnormalities and whether those patients might benefit from intensive treatment with biotherapy and cardiovascular risk factors management.
PubMed: 38806178
DOI: 10.1093/qjmed/hcae105