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Temporal artery biopsy (TAB) is frequently used to guide treatment for suspected temporal arteritis. Our purpose was to determine the influence on subsequent temporal arteritis treatment, particularly the initiation, termination, or continuation of corticosteroids after a histologically negative TAB. This is a retrospective analysis from a single regional referral center on all patients undergoing TAB March 2003 through November 2010. Demographic, clinical, and surgical informations were recorded including changes in treatment based on biopsy results. In all, 237 patients had complete documentation for review; the average age was 71 years (range 34 to 94) and 56% were women. Thirty-six patients had 42 positive biopsies; 26 biopsies were bilateral. Positive biopsy results were defined as having marked intimal thickening, transmural inflammation, and “giant cells.” Neither length of biopsy specimen nor preoperative steroid use affected pathologic diagnosis (2.41 vs 2.38 cm, P = .46, and 52% vs 50%, P = .8, respectively). Symptoms included new-onset headache (75%), preauricular tenderness and jaw claudication (32%), erythrocyte sedimentation rate greater than 50 mm/h (60%), and a score of 3 or more using the American College of Rheumatology criteria (56%). Among the 56% of patients who met the criteria for the clinical diagnosis of temporal arteritis, 22% demonstrated a positive biopsy. Biopsy results had no significant impact on subsequent treatment in 69% of patients who met clinical diagnostic criteria (P = .7); in the remaining 31%, biopsy results altered subsequent treatment with either corticosteroid initiation or discontinuation. The pathologic results of the TAB did not significantly affect treatment in most patients.

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.

Introduction Giant cell arteritis (GCA) is a common large vessel vasculitis in those over age 50 years. This meta-analysis examined the geographical and temporal distribution of the incidence, prevalence, and mortality of GCA. Methods A systematic review was conducted using EMBASE, Scopus, and PubMed from their inceptions until 2019. Studies were included if they reported at least 50 or more GCA patients and defined the location and time frame. Articles on mortality were included and standardized mortality ratio (SMR) was extracted where possible. Mean pooled prevalence, incidence, and SMR were calculated using a random effects model. Linear regression was used to explore correlations between latitude and incidence, prevalence, and mortality. Results Of the 3569 citations identified, 107 were included. The pooled incidence of GCA was 10.00 [9.22, 10.78] cases per 100,000 people over 50 years old. This incidence was highest in Scandinavia 21.57 [18.90, 24.23], followed by North and South America 10.89 [8.78, 13.00], Europe 7.26 [6.05, 8.47], and Oceania 7.85 [− 1.48, 17.19]. Pooled prevalence was 51.74 [42.04, 61.43] cases per 100,000 people over age 50. Annual mortality was 20.44 [17.84, 23.03] deaths/1000. Mortality generally decreased over the years of publication ( p  = 0.0008). Latitude correlated significantly with incidence ( p  = 0.0011), but not with prevalence, or mortality. Conclusions GCA incidence varies nearly 3-fold between regions and is highest in Scandinavia but not significantly. Mortality may be improving over time.

Key Points Giant cell arteritis (GCA) is best understood as an inflammatory vascular syndrome with features of cranial and/or large-vessel vasculitis, systemic inflammation and polymyalgia rheumatica (PMR), which frequently overlap GCA and PMR are among the most common inflammatory rheumatic diseases in the elderly; the prevalence of these diseases is expected to increase due to ageing of the population The role and value of imaging in GCA and PMR is evolving quickly The pathophysiology of GCA is characterized by phases of initiation, transmural inflammation and chronic vessel wall injury and repair, each of which might be novel drug targets Glucocorticoids are the standard-of-care treatment for GCA and PMR, although methotrexate is used in individual cases and anti-IL-6 therapy is now approved for the treatment of GCA The selection of patients for biologic DMARD therapy, defining the best treatment strategies and the development of reliable outcome parameters are challenges in the future management of GCA and PMR Rapid progress in the fields of giant cell arteritis and polymyalgia rheumatica has resulted in the introduction of imaging techniques into routine clinical practice and in promising reports on the efficacy of biologic agents for treatment. Further research should further advance our understanding of the epidemiology, pathogenesis, imaging and treatment of these diseases. The fields of giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) have advanced rapidly, resulting in a new understanding of these diseases. Fast-track strategies and improved awareness programmes that prevent irreversible sight loss through early diagnosis and treatment are a notable advance. Ultrasonography and other imaging techniques have been introduced into routine clinical practice and there have been promising reports on the efficacy of biologic agents, particularly IL-6 antagonists such as tocilizumab, in treating these conditions. Along with these developments, which should improve outcomes in patients with GCA and PMR, new questions and unmet needs have emerged; future research should address which pathogenetic mechanisms contribute to the different phases and clinical phenotypes of GCA, what role imaging has in the early diagnosis and monitoring of GCA and PMR, and in which patients and phases of these diseases novel biologic drugs should be used. This article discusses the implications of recent developments in our understanding of GCA and PMR, as well as the unmet needs concerning epidemiology, pathogenesis, imaging and treatment of these diseases.

Temporal arteritis (TA) is a chronic, systemic vasculitis most often presenting with severe headaches localized in the temporal region, low-grade fever, anorexia, weight loss and generalized malaise. Besides these typical characteristics, a number of vague and non-specific oral and/or ocular symptoms may also be present. A search using Medline (1955-2006) was performed for unusual oral and ocular/orbital presentations of TA. A variety of oral and ocular/orbital manifestations associated with TA have been reported. These can mislead physicians, causing a delay in establishing a diagnosis and initiating treatment. Increased awareness is necessary for the prompt recognition of this potentially devastating disease. Particularly, dentists and ophthalmologists should include TA in their differential diagnosis, as they may be the first to deal with these patients.

Part of the Oxford Rheumatology Library series, Polymyalgia Rheumatica and Giant Cell Arteritis provides quick and practically relevant information on several aspects of the diseases, particularly on diagnosis and management, with the ultimate aim of improving the patient's care.

Objectives To study the epidemiology and mortality in patients with biopsy-proven giant cell arteritis (GCA) in southern Sweden. Methods The study area was the County of Skåne. Patients with a positive temporal artery biopsy between 1997 and 2010 were identified using a regional register and a structured review of all histopathology reports. Standardised mortality ratios (SMR) were calculated using data for the Swedish population as the reference. Results There were 840 patients with biopsy-proven GCA (626 women). The annual incidence rate per 100 000 inhabitants aged ≥50 years was 14.1 (95% CI 13.1 to 15.0); 7.7 (6.7 to 8.7) for men and 19.6 (18.1 to 21.1) for women, without seasonal variations. The incidence increased with age, with estimates of 2.0, 11.8, and 31.3 per 100 000 in the age groups 50–60, 61–70, 71–80 years, respectively (p<0.001). The age-standardised and sex-standardised incidence rate decreased from 15.9/100 000 in 1997–2001 to 13.3/100 000 in 2007–2010 (p=0.026). Two hundred and seventy-nine patients (207 women) died during the observation period. Mortality was significantly increased over the first 2 years after GCA diagnosis (SMR 1.52 (95% CI 1.20 to 1.85)), but not with longer follow-up. The estimated excess mortality was greater in women and in patients aged ≤70 years at diagnosis. Conclusions In this large population-based study of biopsy-proven GCA from southern Sweden, the incidence of GCA may have decreased over time. Short-term mortality was increased, in particular among those diagnosed at ≤70 years of age, but long-term survival was not impaired.

Giant cell arteritis is relatively rare in Indian subjects. A retrospective chart review of cases of giant cell arteritis (GCA) in patients with anterior ischemic optic neuropathy (AION) for last five years (2015 to 2020) in a tertiary care Institution was done. GCA cases were diagnosed on the basis of clinical criteria of new onset of headache along with marked sudden visual loss supplemented by raised erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), and confirmed on temporal artery biopsy. Occult GCA was defined as patients presenting with predominantly ophthalmic symptoms without systemic involvement. All the clinical details of GCA cases have been described in detail. Out of 30 cases of AION, GCA was the diagnosis in 4 cases (13.33%). Among the 4 cases of GCA, 3 cases (75%) were of occult in nature. Three cases underwent superficial temporal artery biopsy which was confirmatory for GCA. GCA was rare in Indian scenario. Earlier age of presentation, male preponderance, and higher number of occult GCA with predominantly ophthalmic involvement were the findings in our study.

Background: Recent studies indicate increased risks of malignant lymphomas among individuals treated with corticosteroids, but have not taken into account the underlying reasons for steroid use, so the increased risks might be attributable to the underlying disease or concomitant treatments other than steroids. Polymyalgia rheumatica (PMR) and temporal arteritis (giant cell arteritis, GCA) are common inflammatory conditions treated with steroids as single immunosuppressive therapy, but data on lymphoma risk in GCA/PMR are limited. Objective: To assess the risk of lymphoma associated with steroid treatment of GCA/PMR. Methods: The association between GCA/PMR and malignant lymphomas (overall, and separately for non-Hodgkin lymphoma, Hodgkin lymphoma, and chronic lymphatic leukaemia) was examined in a nationwide, population based, case–control study of 42 676 lymphoma cases and 78 487 matched population controls, using prospectively recorded data on lymphomas from the Swedish cancer register 1964–2000 and data on pre-lymphoma hospital admissions for GCA/PMR from the Swedish inpatient register 1964–2000. Odds ratios (OR) associated with a pre-lymphoma hospital admission for GCA/PMR were calculated using conditional logistic regression. Results: 153 lymphoma cases and 345 population controls had a history of GCA/PMR, resulting in an overall OR for malignant lymphomas of 0.81 (95% confidence interval, 0.67 to 0.98). The OR varied little with lymphoma type, sex, age, and calendar period. The OR for GCA was 0.67 (0.48 to 0.98) and for PMR, 0.83 (0.67 to 1.04). Conclusions: Treated GCA is not associated with increased lymphoma risks, which suggests that even at considerable cumulative doses, steroids may not appreciably increase lymphoma risk.

Background Relapses frequently occur in giant cell arteritis (GCA), and long-term glucocorticoid therapy is required. The identification of associated factors with poor treatment outcomes is important to decide the treatment algorithm of GCA. Methods We enrolled 139 newly diagnosed GCA patients treated with glucocorticoids between 2007 and 2014 in a retrospective, multi-center registry. Patients were diagnosed with temporal artery biopsy, 1990 American College of Rheumatology classification criteria, or large vessel lesions (LVLs) detected by imaging based on the modified classification criteria. Poor treatment outcomes (non-achievement of clinical remission by week 24 or relapse during 52 weeks) were evaluated. Clinical remission was defined as the absence of clinical signs and symptoms in cranial and large vessel areas, polymyalgia rheumatica (PMR), and elevation of C-reactive protein (CRP) levels. A patient was determined to have a relapse if he/she had either one of the signs and symptoms that newly appeared or worsened after achieving clinical remission. Re-elevation of CRP without clinical manifestations was considered as a relapse if other causes such as infection were excluded and the treatment was intensified. Associated factors with poor treatment outcomes were analyzed by using the Cox proportional hazard model. Results Cranial lesions, PMR, and LVLs were detected in 77.7%, 41.7%, and 52.5% of the enrolled patients, respectively. Treatment outcomes were evaluated in 119 newly diagnosed patients who were observed for 24 weeks or longer. The mean initial dose of prednisolone was 0.76 mg/kg/day, and 29.4% received any concomitant immunosuppressive drugs at baseline. Overall, 41 (34.5%) of the 119 patients had poor treatment outcomes; 13 did not achieve clinical remission by week 24, and 28 had a relapse after achieving clinical remission. Cumulative rates of the events of poor treatment outcomes in patients with and without LVLs were 47.5% and 17.7%, respectively. A multivariable model showed the presence of LVLs at baseline was significantly associated with poor treatment outcomes (adjusted hazard ratio [HR] 3.54, 95% CI 1.52–8.24, p  = 0.003). Cranial lesions and PMR did not increase the risk of poor treatment outcomes. Conclusion The initial treatment intensity in the treatment algorithm of GCA could be determined based upon the presence or absence of LVLs detected by imaging at baseline.