BACKGROUND: Temporal artery (TA) biopsy is the current standard for diagnosing temporal arteritis, but has limited sensitivity. Colour duplex ultrasonography is a newer, non-invasive method of diagnosing temporal arteritis. METHODS: A systematic review was performed of trials comparing TA biopsy with duplex ultrasonography. Duplex results (halo sign, stenosis or vessel occlusion) were compared with either TA biospy findings or the American College of Rheumatology research criteria for diagnosing temporal arteritis. Trials were identified from MEDLINE, Embase and the Cochrane Library trials register. The performance of duplex ultrasonography was assessed with weighted independent sensitivity and specificity values, and summary receiver operating characteristic curve analysis. RESULTS: There were 17 eligible studies containing 998 patients. When the halo sign on duplex imaging was compared with TA biopsy, the sensitivity was 75 (95 per cent confidence interval 67 to 82) per cent and the specificity was 83 (78 to 88) per cent. There was no heterogeneity across the eligible studies. CONCLUSION: Duplex ultrasonography was relatively accurate for diagnosing temporal arteritis. It should become the first-line investigation, with biopsy reserved for patients with a negative scan.

Assessment of the pattern and extent of arterial involvement and measurement of current disease activity are essential for the management of Takayasu arteritis (TA). Since there is no completed, placebo-controlled, randomized clinical trial, the level of evidence for management of TA is low, generally reflecting the results of open studies, case series and expert opinion. The most commonly used agents include corticosteroids and conventional immunosuppressive agents such as MTX, AZA, MMF and LEF. In patients who remain resistant and/or intolerant to these agents, biologic drugs including TNF inhibitors, rituximab and tocilizumab seem to be promising. Antiplatelet treatment may also lower the frequency of ischaemic events in TA. In the presence of short-segment, critical arterial stenosis, balloon angioplasty or stent graft replacement may be useful. On the other hand, long-segment stenosis with extensive periarterial fibrosis or occlusion requires surgical bypass of the affected segment, which is clearly associated with superior results compared with endovascular intervention. As a general rule, both endovascular intervention and surgical procedures should be avoided during the active phase of the disease. Earlier diagnosis, better assessment of disease activity and future clinical trials will obviously improve the management of TA.

BACKGROUND: Takayasu arteritis (TA) is a rare, systemic, inflammatory vasculitis of granulomatous nature, and still of unknown etiology. It mainly involves the aorta and its major branches and is more commonly seen in women of childbearing age and Asians. TA leads to stenosis, occlusion, or aneurysmal degeneration of large arteries, and its pathogenesis seems to be mainly due to an abnormal cell-mediated immunity, although other molecular and genetic abnormalities may contribute. The diagnosis and treatments lie on clinical and arteriographic findings. Because of its fluctuating course, both clinical scores and biomarkers are currently evaluated. The aim of this review is to report a comprehensive and methodologically robust state of the art about Takayasu arteritis, including the latest data and evidences in the definition, epidemiology, pathogenesis and etiology, clinical manifestations and classification, diagnosis, assessment of disease activity and progression, biomarkers, and treatment. METHODS: We searched all publications addressing definition, epidemiology, pathogenesis, etiology, classification, diagnosis, biomarkers, and treatment of TA. Randomized trials, cohort studies, and reviews were contemplated to give a breadth of clinical data. PubMed and Scopus were searched from August 2010 to November 2015. RESULTS: Of the 3,056 records found, 267 matched our inclusion criteria. After reading the full-text articles, we decided to exclude 169 articles because of the following reasons: (1) no innovative or important content; (2) no multivariable analysis; (3) insufficient data; (4) no clear potential biases or strategies to solve them; (5) no clear end-points; and (6) inconsistent or arbitrary conclusions. The final set included 98 articles. CONCLUSIONS: This review presents the last updates in all fields of Takayasu arteritis. Still today, large areas of TA pathogenesis and disease-activity assessment need to be further investigated to better treat patients with TA.

BACKGROUND: Takayasu's arteritis (TA) is a chronic inflammatory disease that involves the aorta and its major branches; however, only limited data are available on TA in Taiwan. This study presents the clinical features, angiographic findings, and response to treatment of patients with TA at a single institute in Taiwan. METHODS: A search of the hospital database for ICD9 code 446.7 (Takayasu's disease) between 1990 and 2010 was performed. Seven cases fulfilled the 1990 American College of Rheumatology diagnostic criteria for Takayasu's disease. Angiographic classification was made according to the guidelines of the 1994 International TA Conference in Tokyo. RESULTS: All of our cases were female, and the median age at diagnosis was 27.5 years (range 14-36 years). Four patients had an angiographic classification of type I (57.1%), two were classified as type V (28.6%), and one was classified as type III (14.3%). The most common symptoms/signs were dizziness and vascular bruits. Two patients underwent bypass surgery, four endovascular stenting, and one hybrid bypass with stenting. After a mean follow-up period of 50.3 ± 68.2 months (range 12.3-199.6 months), both the procedure success and survival rates were 100%. There were four restenosis cases (57.1%), one in the surgical bypass group without symptoms (33.3%), and three in the endovascular group (60%), five restenosis in 14 stents (35.7%). Also, these three patients received secondary endovascular procedure for percutaneous transluminal angioplasty or restenting. CONCLUSION: There have not been any case series reports about treatments of Takayasu's disease in Taiwan to date, based on a search of the PubMed/MEDLINE and Cochrane Library databases. Although endovascular treatment is becoming more prevalent, the restenosis rate is still high, and long-term follow-up and further strategy for restenosis management are the main challenges.

BACKGROUND: Giant cell arteritis (GCA) is a common inflammatory condition that affects medium and large-sized arteries and can cause sudden, permanent blindness. At present there is no alternative to early treatment with high-dose corticosteroids as the recommended standard management. Corticosteroid-induced side effects can develop and further disease-related ischaemic complications can still occur. Alternative and adjunctive therapies are sought. Aspirin has been shown to have effects on the immune-mediated inflammation in GCA, hence it may reduce damage caused in the arterial wall. OBJECTIVES: To assess the safety and effectiveness of low-dose aspirin, as an adjunctive, in the treatment of giant cell arteritis (GCA). SEARCH METHODS: We searched CENTRAL (which contains the Cochrane Eyes and Vision Group Trials Register) (2013, Issue 12), Ovid MEDLINE, Ovid MEDLINE In-Process and Other Non-Indexed Citations, Ovid MEDLINE Daily, Ovid OLDMEDLINE (January 1946 to January 2014), EMBASE (January 1980 to January 2014), Latin American and Caribbean Health Sciences Literature Database (LILACS) (January 1982 to January 2014), the metaRegister of Controlled Trials (mRCT) (www.controlled-trials.com), ClinicalTrials.gov (www.clinicaltrials.gov), the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP) (www.who.int/ictrp/search/en) and the US Food and Drugs Administration (FDA) web site (www.fda.gov). We did not use any date or language restrictions in the electronic searches for trials. We last searched the electronic databases on 24 January 2014. SELECTION CRITERIA: We planned to include only randomised controlled trials (RCTs) comparing outcomes of GCA with and without concurrent adjunctive use of low-dose aspirin. DATA COLLECTION AND ANALYSIS: Two authors independently assessed the search results for trials identified by the electronic searches. No trials met our inclusion criteria, therefore we undertook no assessment of risk of bias or meta-analysis. MAIN RESULTS: We found no RCTs that met the inclusion criteria. AUTHORS' CONCLUSIONS: There is currently no evidence from RCTs to determine the safety and efficacy of low-dose aspirin as an adjunctive treatment in GCA. Clinicians who are considering the use of low-dose aspirin as an adjunctive treatment in GCA must also recognise the established haemorraghic risks associated with aspirin, especially in the context of concurrent treatment with corticosteroids. There is a clear need for effectiveness trials to guide the management of this life-threatening condition.

OBJECTIVE: Different lines of evidence have highlighted the role of IL-17A in the inflammatory process occurring in giant cell arteritis (GCA). The aim of the present study was to assess whether the IL17A locus influences GCA susceptibility and its clinical subphenotypes. METHODS: We carried out a large meta-analysis including a total of 1266 biopsy-proven GCA patients and 3779 healthy controls from four European populations (Spain, Italy, Germany and Norway). Five IL17A polymorphisms (rs4711998, rs8193036, rs3819024, rs2275913 and rs7747909) were selected by tagging and genotyped using TaqMan assays. Allelic combination and dependency tests were also performed. RESULTS: In the pooled analysis, two of the five analysed polymorphisms showed evidence of association with GCA (rs2275913: PMH=1.85E-03, OR=1.17 (1.06-1.29); rs7747909: PMH=8.49E-03, OR=1.15 (1.04-1.27)). A clear trend of association was also found for the rs4711998 variant (PMH=0.059, OR=1.11 (1.00-1.23)). An independent effect of rs2275913 and rs4711998 was evident by conditional regression analysis. In addition, the haplotype harbouring the risk alleles better explained the observed association than the polymorphisms independently (likelihood p value <10(-05)). CONCLUSIONS: Polymorphisms within the IL17A locus show a novel association with GCA. This finding supports the relevant role of the Th17 cells in this vasculitis pathophysiology.

OBJECTIVE: The interleukin-6 pathway is up-regulated in giant cell arteritis (GCA), Takayasu arteritis (TA), and polymyalgia rheumatica (PMR). We retrospectively assessed the outcomes of 10 patients with relapsing/refractory GCA, TA, or PMR treated with tocilizumab (TCZ). METHODS: Patients with GCA (n = 7), TA (n = 2), and PMR (n = 1) received TCZ. Seven subjects had failed at least 1 second-line agent. The outcomes evaluated were symptoms of disease activity, inflammatory markers, ability to taper glucocorticoids, and cross-sectional imaging when indicated clinically. RESULTS: The mean followup time of this cohort since diagnosis was 27 months (range 16-60 months). The patients were treated with TCZ for a mean period of 7.8 months (range 4-12 months). Before TCZ therapy, the patients experienced an average of 2.4 flares/year. All patients entered and maintained clinical remission during TCZ therapy. The mean daily prednisone dosages before and after TCZ initiation were 20.8 mg/day (range 7-34.3 mg/day) and 4.1 mg/day (range 0-10.7 mg/day), respectively (P = 0.0001). The mean erythrocyte sedimentation rate declined from 41.5 mm/hour (range 11-68 mm/hour) to 7 mm/hour (range 2.2-11.3 mm/hour; P = 0.0001). The adverse effects of TCZ included mild neutropenia (n = 4) and transaminitis (n = 4). One patient flared 2 months after TCZ discontinuation. An autopsy on 1 patient who died from a postoperative myocardial infarction following elective surgery revealed persistent vasculitis of large and medium-sized arteries. CONCLUSION: TCZ therapy led to clinical and serologic improvement in patients with refractory/relapsing GCA, TA, or PMR. The demonstration of persistent large-vessel vasculitis at autopsy of 1 patient who had shown a substantial response requires close scrutiny in larger studies.

IMPORTANCE: Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are related inflammatory disorders occurring in persons aged 50 years and older. Diagnostic and therapeutic approaches are heterogeneous in clinical practice. OBJECTIVE: To summarize current evidence regarding optimal methods for diagnosing and treating PMR and GCA. EVIDENCE REVIEW: MEDLINE, EMBASE, and Cochrane databases were searched from their inception dates to March 30, 2016. Screening by 2 authors resulted in 6626 abstracts, of which 50 articles met the inclusion criteria. Study quality was assessed using the Quality Assessment of Diagnostic Accuracy Studies (QUADAS-2) tool or American College of Cardiology Foundation/American Heart Association methodology. FINDINGS: Twenty randomized clinical trials for therapy (n = 1016 participants) and 30 imaging studies for diagnosis and/or assessing response to therapy (n = 2080 participants) were included. The diagnosis of PMR is based on clinical features such as new-onset bilateral shoulder pain, including subdeltoid bursitis, muscle or joint stiffness, and functional impairment. Headache and visual disturbances including loss of vision are characteristic of GCA. Constitutional symptoms and elevated inflammatory markers (>90%) are common in both diseases. Ultrasound imaging enables detection of bilateral subdeltoid bursitis in 69% of PMR patients. In GCA, temporal artery biopsy remains the standard for definitive diagnosis. Ultrasound and magnetic resonance imaging (MRI) of large vessels revealing inflammation-induced wall thickening support the diagnosis of GCA (specificity 78%-100% for ultrasound and 73%-97% for MRI). Glucocorticoids remain the primary treatment, but the optimal initial dose and tapering treatment regimens are unknown. According to consensus-based recommendations, initial therapy for PMR is prednisone, 12.5 to 25 mg/day or equivalent, and 40 to 60 mg/day for GCA, followed by individualized tapering regimens in both diseases. Adjunctive methotrexate may reduce cumulative glucocorticoid dosage by 20% to 44% and relapses by 36% to 54% in both PMR and GCA. Use of tocilizumab as additional treatment with prednisone showed a 2- to 4-fold increase in remission rates of GCA in a randomized clinical trial (N = 30). CONCLUSIONS AND RELEVANCE: Diagnosis of PMR/GCA is made by clinical features and elevated inflammatory markers. In PMR, ultrasound imaging may improve diagnostic accuracy. In GCA, temporal artery biopsy may not be required in patients with typical disease features accompanied by characteristic ultrasound or MRI findings. Consensus-based recommendations suggest glucocorticoids as the most effective therapy for PMR/GCA. Methotrexate may be added to glucocorticoids in patients at risk for relapse and in those with glucocorticoid-related adverse effects or need for prolonged glucocorticoid therapy.

CRD SUMMARY: This review concluded that duplex ultrasonography was relatively accurate for diagnosing temporal arteritis and should become the first-line investigation, with biopsy reserved for patients with negative scans. These conclusions should be interpreted with caution due to the possibility of missing studies, limitations with the quality assessment and analysis and a lack of data on the clinical significance of the findings.

AIM: To investigate the association between giant cell arteritis (GCA) and risk of peripheral arterial disease (PAD). METHODS: We conducted a systematic review and meta-analysis of observational cohort studies that reported relative risks, hazard ratios or standardized incidence ratios with 95% confidence comparing PAD risk in patients with GCA versus non-GCA subjects. Pooled risk ratios and 95% confidence intervals were calculated using a random-effect, generic inverse variance of DerSimonian and Laird. RESULTS: Four studies with 9789 patients with GCA and 236 728 controls were identified and included in our data analysis. The pooled risk ratio of PAD among patients with GCA compared with controls was 1.88 (95% CI 1.04-3.41). The statistical heterogeneity was high with an I(2) of 89%. CONCLUSION: Our study demonstrated a statistically significant increased risk of PAD among patients with GCA.