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Journal of Internal Medicine Nov 2022Polymyalgia rheumatica (PMR) is the most common inflammatory rheumatic disease affecting people older than 50 years and is 2-3 times more common in women. The most... (Review)
Review
Polymyalgia rheumatica (PMR) is the most common inflammatory rheumatic disease affecting people older than 50 years and is 2-3 times more common in women. The most common symptoms are pain and morning stiffness in the shoulder and pelvic girdle and the onset may be acute or develop over a few days to weeks. General symptoms such as fatigue, fever and weight loss may occur, likely driven by systemic IL-6 signalling. The pathology includes synovial and periarticular inflammation and muscular vasculopathy. A new observation is that PMR may appear as a side effect of cancer treatment with checkpoint inhibitors. The diagnosis of PMR relies mainly on symptoms and signs combined with laboratory markers of inflammation. Imaging modalities including ultrasound, magnetic resonance imaging and positron emission tomography with computed tomography are promising new tools in the investigation of suspected PMR. However, they are still limited by availability, high cost and unclear performance in the diagnostic workup. Glucocorticoid (GC) therapy is effective in PMR, with most patients responding promptly to 15-25 mg prednisolone per day. There are challenges in the management of patients with PMR as relapses do occur and patients with PMR may need to stay on GC for extended periods. This is associated with high rates of GC-related comorbidities, such as diabetes and osteoporosis, and there are limited data on the use of disease-modifying antirheumatic drugs and biologics as GC sparing agents. Finally, PMR is associated with giant cell arteritis that may complicate the disease course and require more intense and prolonged treatment.
Topics: Antirheumatic Agents; Biological Products; Biomarkers; Female; Giant Cell Arteritis; Glucocorticoids; Humans; Inflammation; Interleukin-6; Polymyalgia Rheumatica; Prednisolone
PubMed: 35612524
DOI: 10.1111/joim.13525 -
Clinical & Experimental Optometry Sep 2020Temporal arteritis (TA), or giant cell arteritis, is a systemic autoimmune vasculitis affecting patients over 50 years of age. It can cause rapid, irreversible... (Review)
Review
Temporal arteritis (TA), or giant cell arteritis, is a systemic autoimmune vasculitis affecting patients over 50 years of age. It can cause rapid, irreversible bilateral vision loss in older adults and is therefore considered an ophthalmological emergency. Many of the symptoms and signs of TA can be vague, non-specific and gradual in onset, often leading to a delayed or inaccurate diagnosis. As such, it is important for a wide variety of primary optometrists and health practitioners to maintain a robust understanding of the clinical presentation, key investigations and time-sensitive management of this disease, as early initiation of treatment for TA can be vision- and life-saving.
Topics: Biopsy; Diagnosis, Differential; Disease Management; Giant Cell Arteritis; Humans; Positron Emission Tomography Computed Tomography; Temporal Arteries
PubMed: 31663193
DOI: 10.1111/cxo.12975 -
Eye (London, England) Jun 2020The landscape of the investigation and management of giant cell arteritis (GCA) is advancing. In this review we will outline the recent advances by searching the current... (Review)
Review
The landscape of the investigation and management of giant cell arteritis (GCA) is advancing. In this review we will outline the recent advances by searching the current English literature for relevant articles using key words of giant cell arteritis, temporal arteritis, Horton's disease, investigation, and treatment. Delay in diagnosis, diagnostic uncertainty and glucocorticoid (GC) morbidity are among the highest concerns of clinicians and patients in this disease area. The positive news is that fast track pathways, imaging techniques and new therapies are emerging for routine management of GCA. Future directions for intervention in the treatment paradigm will be discussed.
Topics: Giant Cell Arteritis; Glucocorticoids; Humans
PubMed: 31582795
DOI: 10.1038/s41433-019-0608-7 -
JAMA Internal Medicine Oct 2020Current clinical guidelines recommend selecting diagnostic tests for giant cell arteritis (GCA) based on pretest probability that the disease is present, but how pretest... (Meta-Analysis)
Meta-Analysis
IMPORTANCE
Current clinical guidelines recommend selecting diagnostic tests for giant cell arteritis (GCA) based on pretest probability that the disease is present, but how pretest probability should be estimated remains unclear.
OBJECTIVE
To evaluate the diagnostic accuracy of symptoms, physical signs, and laboratory tests for suspected GCA.
DATA SOURCES
PubMed, EMBASE, and the Cochrane Database of Systematic Reviews were searched from November 1940 through April 5, 2020.
STUDY SELECTION
Trials and observational studies describing patients with suspected GCA, using an appropriate reference standard for GCA (temporal artery biopsy, imaging test, or clinical diagnosis), and with available data for at least 1 symptom, physical sign, or laboratory test.
DATA EXTRACTION AND SYNTHESIS
Screening, full text review, quality assessment, and data extraction by 2 investigators. Diagnostic test meta-analysis used a bivariate model.
MAIN OUTCOME(S) AND MEASURES
Diagnostic accuracy parameters, including positive and negative likelihood ratios (LRs).
RESULTS
In 68 unique studies (14 037 unique patients with suspected GCA; of 7798 patients with sex reported, 5193 were women [66.6%]), findings associated with a diagnosis of GCA included limb claudication (positive LR, 6.01; 95% CI, 1.38-26.16), jaw claudication (positive LR, 4.90; 95% CI, 3.74-6.41), temporal artery thickening (positive LR, 4.70; 95% CI, 2.65-8.33), temporal artery loss of pulse (positive LR, 3.25; 95% CI, 2.49-4.23), platelet count of greater than 400 × 103/μL (positive LR, 3.75; 95% CI, 2.12-6.64), temporal tenderness (positive LR, 3.14; 95% CI, 1.14-8.65), and erythrocyte sedimentation rate greater than 100 mm/h (positive LR, 3.11; 95% CI, 1.43-6.78). Findings that were associated with absence of GCA included the absence of erythrocyte sedimentation rate of greater than 40 mm/h (negative LR, 0.18; 95% CI, 0.08-0.44), absence of C-reactive protein level of 2.5 mg/dL or more (negative LR, 0.38; 95% CI, 0.25-0.59), and absence of age over 70 years (negative LR, 0.48; 95% CI, 0.27-0.86).
CONCLUSIONS AND RELEVANCE
This study identifies the clinical and laboratory features that are most informative for a diagnosis of GCA, although no single feature was strong enough to confirm or refute the diagnosis if taken alone. Combinations of these symptoms might help direct further investigation, such as vascular imaging, temporal artery biopsy, or seeking evaluation for alternative diagnoses.
Topics: Biopsy; Blood Sedimentation; Clinical Laboratory Techniques; Giant Cell Arteritis; Humans; Physical Examination; Positron-Emission Tomography; Temporal Arteries; Ultrasonography
PubMed: 32804186
DOI: 10.1001/jamainternmed.2020.3050 -
Health Technology Assessment... Nov 2016Giant cell arteritis (GCA) is a relatively common form of primary systemic vasculitis, which, if left untreated, can lead to permanent sight loss. We compared ultrasound... (Clinical Trial)
Clinical Trial
The Role of Ultrasound Compared to Biopsy of Temporal Arteries in the Diagnosis and Treatment of Giant Cell Arteritis (TABUL): a diagnostic accuracy and cost-effectiveness study.
BACKGROUND
Giant cell arteritis (GCA) is a relatively common form of primary systemic vasculitis, which, if left untreated, can lead to permanent sight loss. We compared ultrasound as an alternative diagnostic test with temporal artery biopsy, which may be negative in 9-61% of true cases.
OBJECTIVE
To compare the clinical effectiveness and cost-effectiveness of ultrasound with biopsy in diagnosing patients with suspected GCA.
DESIGN
Prospective multicentre cohort study.
SETTING
Secondary care.
PARTICIPANTS
A total of 381 patients referred with newly suspected GCA.
MAIN OUTCOME MEASURES
Sensitivity, specificity and cost-effectiveness of ultrasound compared with biopsy or ultrasound combined with biopsy for diagnosing GCA and interobserver reliability in interpreting scan or biopsy findings.
RESULTS
We developed and implemented an ultrasound training programme for diagnosing suspected GCA. We recruited 430 patients with suspected GCA. We analysed 381 patients who underwent both ultrasound and biopsy within 10 days of starting treatment for suspected GCA and who attended a follow-up assessment (median age 71.1 years; 72% female). The sensitivity of biopsy was 39% [95% confidence interval (CI) 33% to 46%], which was significantly lower than previously reported and inferior to ultrasound (54%, 95% CI 48% to 60%); the specificity of biopsy (100%, 95% CI 97% to 100%) was superior to ultrasound (81%, 95% CI 73% to 88%). If we scanned all suspected patients and performed biopsies only on negative cases, sensitivity increased to 65% and specificity was maintained at 81%, reducing the need for biopsies by 43%. Strategies combining clinical judgement (clinician's assessment at 2 weeks) with the tests showed sensitivity and specificity of 91% and 81%, respectively, for biopsy and 93% and 77%, respectively, for ultrasound; cost-effectiveness (incremental net monetary benefit) was £485 per patient in favour of ultrasound with both cost savings and a small health gain. Inter-rater analysis revealed moderate agreement among sonographers (intraclass correlation coefficient 0.61, 95% CI 0.48 to 0.75), similar to pathologists (0.62, 95% CI 0.49 to 0.76).
LIMITATIONS
There is no independent gold standard diagnosis for GCA. The reference diagnosis used to determine accuracy was based on classification criteria for GCA that include clinical features at presentation and biopsy results.
CONCLUSION
We have demonstrated the feasibility of providing training in ultrasound for the diagnosis of GCA. Our results indicate better sensitivity but poorer specificity of ultrasound compared with biopsy and suggest some scope for reducing the role of biopsy. The moderate interobserver agreement for both ultrasound and biopsy indicates scope for improving assessment and reporting of test results and challenges the assumption that a positive biopsy always represents GCA.
FUTURE WORK
Further research should address the issue of an independent reference diagnosis, standards for interpreting and reporting test results and the evaluation of ultrasound training, and should also explore the acceptability of these new diagnostic strategies in GCA.
FUNDING
The National Institute for Health Research Health Technology Assessment programme.
Topics: Aged; Biopsy; Cost-Benefit Analysis; Female; Giant Cell Arteritis; Glucocorticoids; Humans; Male; Middle Aged; Prospective Studies; Reproducibility of Results; Sensitivity and Specificity; Temporal Arteries; Ultrasonography
PubMed: 27925577
DOI: 10.3310/hta20900 -
Journal of the Chinese Medical... Jul 2005Temporal arteritis, a chronic inflammatory vasculitis involving medium- and large-sized arteries, has rarely been reported in Asia. However, we report 2 cases, in which...
Temporal arteritis, a chronic inflammatory vasculitis involving medium- and large-sized arteries, has rarely been reported in Asia. However, we report 2 cases, in which the patients initially presented with headache. Physical examination disclosed engorged, hard and palpable vessels in the temporal areas. Temporal-artery biopsy revealed 2 different types of arteritis: the multinucleated giant cell type and the panarteritis type without multinucleated giant cells. One patient was positive for immunoglobulin G anticardiolipin antibody. The pathologic findings of the different subsets of temporal arteritis, and the relationship between anticardiolipin antibody and the extent of vascular complications of temporal arteritis, are discussed.
Topics: Aged; Antibodies, Anticardiolipin; Biopsy; Giant Cell Arteritis; Humans; Male; Temporal Arteries
PubMed: 16038374
DOI: 10.1016/S1726-4901(09)70170-4 -
Current Opinion in Infectious Diseases Jun 2016Giant cell arteritis (GCA) is a serious disease and the most common cause of vasculitis in the elderly. Here, studies describing the recent discovery of varicella zoster... (Review)
Review
PURPOSE OF REVIEW
Giant cell arteritis (GCA) is a serious disease and the most common cause of vasculitis in the elderly. Here, studies describing the recent discovery of varicella zoster virus (VZV) in the temporal arteries of patients with GCA are reviewed.
RECENT FINDINGS
GCA is characterized by severe headache/head pain and scalp tenderness. Many patients also have a history of vision loss, jaw claudication, polymyalgia rheumatica, fever, night sweats, weight loss, and fatigue. The erythrocyte sedimentation rate and C-reactive protein are usually elevated. Diagnosis is confirmed by temporal artery biopsy, which reveals vessel wall damage and inflammation, with multinucleated giant cells and/or epithelioid macrophages. Skip lesions are common. Importantly, temporal artery biopsies are pathologically negative in many clinically suspect cases. The present review highlights recent virological findings in temporal arteries from patients with pathologically verified GCA and in temporal arteries from patients who manifest clinical and laboratory features of GCA but whose temporal artery biopsies are pathologically negative for GCA. Virological analysis revealed that VZV is present in most GCA-positive and GCA-negative temporal artery biopsies, particularly in skip areas that correlate with adjacent GCA disease.
SUMMARY
The presence of VZV in GCA-positive and GCA-negative temporal arteries reflects the possible role of VZV in triggering the immunopathology of GCA and indicates that both groups of patients should be treated with antivirals in addition to corticosteroids. Whether oral antiviral agents and steroids are as effective as intravenous acyclovir and steroids, and the dosage and duration of treatment, remain to be determined.
Topics: Aged; Aged, 80 and over; Female; Giant Cell Arteritis; Herpesviridae Infections; Herpesvirus 3, Human; Humans; Male; Middle Aged; Temporal Arteries
PubMed: 26871403
DOI: 10.1097/QCO.0000000000000258 -
Nihon Rinsho Men'eki Gakkai Kaishi =... 2013Giant cell arteritis (GCA) is a granulomatous arteritis and it occurs older (more than 50 years) individuals. As GCA frequently involves temporal artery, this disease... (Review)
Review
Giant cell arteritis (GCA) is a granulomatous arteritis and it occurs older (more than 50 years) individuals. As GCA frequently involves temporal artery, this disease had been called as temporal arteritis. However, except for the temporal artery, GCA affects branches of the carotid arteries, as well as aorta and its major branches preferentially. Thus, this arteritis is collectively called as GCA from the characteristic histological findings. We systematically introduce the clinical pictures of GCA in the first half of this article. Because affected arteries of GCA are different in each patient, some patients do not present with classical clinical features, such as headache and tenderness of temporal artery. Recently, there are several variant forms of GCA have been recognized. Certain subtypes demonstrate organ dysfunction, such as visual loss or peripheral neuropathy with minimal or absent classical and systemic manifestations. This form of GCA is referred to as occult GCA. On the other hand, other form of GCA presents with a systemic inflammatory syndrome in the absence of focal ischemic symptoms. This is referred to as silent or masked GCA. In the latter half of this article, we introduce two patients presenting with such atypical presentations. One could be diagnosed as occult GCA, and the other was diagnosed as silent GCA with large vessel type. GCA is a heterogenous disease with more than a single clinical picture. This article can provide considerations of the wide spectrum of presentation of this characteristic systemic arteritis.
Topics: Aged; Cyclophosphamide; Diagnostic Imaging; Drug Therapy, Combination; Female; Giant Cell Arteritis; Humans; Immunosuppressive Agents; Japan; Male; Methotrexate; Methylprednisolone; Middle Aged; Prednisolone; Prognosis; Pulse Therapy, Drug; Temporal Arteries
PubMed: 24390106
DOI: 10.2177/jsci.36.459 -
Frontiers in Immunology 2021Giant cell arteritis (GCA) is a granulomatous systemic vasculitis of large- and medium-sized arteries that affects the elderly. In recent years, advances in diagnostic... (Review)
Review
Giant cell arteritis (GCA) is a granulomatous systemic vasculitis of large- and medium-sized arteries that affects the elderly. In recent years, advances in diagnostic imaging have revealed a greater degree of large vessel involvement than previously recognized, distinguishing classical cranial- from large vessel (LV)- GCA. GCA often co-occurs with the poorly understood inflammatory arthritis/bursitis condition polymyalgia rheumatica (PMR) and has overlapping features with other non-infectious granulomatous vasculitides that affect the aorta, namely Takayasu Arteritis (TAK) and the more recently described clinically isolated aortitis (CIA). Here, we review the literature focused on the immunopathology of GCA on the background of the three settings in which comparisons are informative: LV and cranial variants of GCA; PMR and GCA; the three granulomatous vasculitides (GCA, TAK, and CIA). We discuss overlapping and unique features between these conditions across clinical presentation, epidemiology, imaging, and conventional histology. We propose a model of GCA where abnormally activated circulating cells, especially monocytes and CD4 T cells, enter arteries after an unknown stimulus and cooperate to destroy it and review the evidence for how this mechanistically occurs in active disease and improves with treatment.
Topics: Animals; Aorta; CD4-Positive T-Lymphocytes; Giant Cell Arteritis; Humans; Inflammation Mediators; Monocytes; Takayasu Arteritis; Temporal Arteries
PubMed: 33717128
DOI: 10.3389/fimmu.2021.623716 -
The Netherlands Journal of Medicine Jun 2016A systematic literature search was performed to summarise current knowledge on extracranial giant cell arteritis (GCA), i.e. large-artery involvement in patients with or... (Review)
Review
A systematic literature search was performed to summarise current knowledge on extracranial giant cell arteritis (GCA), i.e. large-artery involvement in patients with or without clinically apparent temporal arteritis (cranial GCA). Extracranial GCA is increasingly recognised, both in patients with cranial GCA and with solitary extracranial GCA, due to increased awareness among physicians and development of modern imaging modalities. The literature on the pathogenesis and histopathology of extracranial GCA is scarce. It is considered to be similar to cranial GCA. Patients with solitary extracranial GCA often present with non-specific signs and symptoms, although vascular manifestations, mostly secondary to stenosis, may occur. Due to the non-specific clinical presentation and low sensitivity of temporal artery biopsies, extracranial GCA is usually diagnosed by imaging. 18F-FDG-PET, MRI, CT angiography and ultrasound are used for this purpose. At present, the optimal diagnostic strategy is undetermined. The choice for a particular modality can be guided by the clinical scenario that raises suspicion of extracranial GCA, in addition to local availability and expertise. Extracranial complications in GCA consist of aortic aneurysm or dissection (mainly the ascending aorta), aortic arch syndrome, arm claudication and posterior stroke (although this is technically a cranial complication, it often results from stenosis of the vertebrobasilar arteries). Mortality is generally not increased in patients with GCA. Treatment of patients with solitary extracranial and those with extracranial and cranial GCA has been debated in the recent literature. In general, the same strategy is applied as in patients with temporal arteritis, although criteria regarding who to treat are unclear. Surgical procedures may be indicated, in which case optimal medical treatment prior to surgery is important.
Topics: Aortic Dissection; Aortic Aneurysm; Aortic Arch Syndromes; Aortitis; Axillary Artery; Biopsy; Carotid Artery Diseases; Computed Tomography Angiography; Femoral Artery; Fluorodeoxyglucose F18; Giant Cell Arteritis; Glucocorticoids; Humans; Iliac Artery; Immunosuppressive Agents; Magnetic Resonance Angiography; Mesenteric Arteries; Positron Emission Tomography Computed Tomography; Prognosis; Radiopharmaceuticals; Renal Artery; Subclavian Artery; Temporal Arteries; Vascular Surgical Procedures
PubMed: 27323671
DOI: No ID Found