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Background: Takayasu arteritis (TAK) and giant cell arteritis (GCA) are two major large vessel vasculitis, with varied epidemiology by geographical location, age, and race. However, the epidemiological data in Chinese population is rarely reported. This study estimated the epidemiology of TAK and GCA in Shanghainese individuals residing in China over a 10‐year period. Methods: TAK data for individuals over 16 years and GCA data for individuals over 50 years were retrieved from 38 comprehensive hospitals in Shanghai, China through the electronic medical record systems between January 1, 2011, and December 31, 2020. A systematic literature review was performed to determine the global distribution of TAK and GCA by searching PubMed, Ovid‐Medline, Excerpta Medica Database (EMBASE), and Web of Science. Results: In 173 identified TAK cases (67% females; mean age, 46 ± 15 years), the period prevalence was 11.72 cases per million, and the mean annual incidence was 1.33 cases per million. The highest prevalence (17.74 cases per million) and incidence (1.71 cases per million) were observed in the 16‐ to 34‐year‐old age group. In 92 identified GCA cases (56% females; age, >50 years), the period prevalence was 2.73 cases per 100 000 persons, and the mean annual incidence was 1.91 cases per 100 000 persons. Meta‐analysis of the incidence study of TAK and GCA showed that the pooled incidence rate of TAK and GCA was 1.29 per million and 15.48 per 100 000 person‐years, respectively. Subgroup analysis showed that the incidence of TAK was significantly higher in Asia than in other regions, while the incidence of GCA was higher in Europe, especially North Europe. Conclusion: The epidemiological patterns of TAK and GCA were comprehensively mapped globally and locally, in Shanghai, China. [ABSTRACT FROM AUTHOR]

This article discusses a case of giant cell arteritis (GCA) with genital system involvement. GCA is a chronic inflammatory condition that primarily affects individuals over the age of 50. While GCA commonly affects the temporal arteries, it can also involve other arteries, including those in the lung, breast, and gynecological system. The article presents a case of a 61-year-old female patient who was diagnosed with GCA based on the analysis of ovarian and uterine pathology specimens. The patient's symptoms and acute phase reactants returned to normal with treatment, and her progress has been monitored for 3 years. The article emphasizes the importance of considering GCA in patients with systemic inflammatory symptoms and genital system problems. [Extracted from the article]

This document explores the use of ultrasound (US) in rheumatology practice, specifically focusing on a new model called Point of Care US (POCUS) in Rheumatology (POCRUS). The authors argue that learning US examination skills can improve diagnostic accuracy and clinical confidence in rheumatology. They propose a probability-directed approach to US, using standardized assessments based on international classification criteria for rheumatic and musculoskeletal diseases. The document acknowledges the support of various organizations in organizing and reporting the findings of an online course on POCRUS. [Extracted from the article]

This article reports on a rare case of a patient with minimal change nephrotic syndrome, juvenile temporal arteritis (JTA), and eosinophilia. JTA is a rare disease characterized by localized vasculitis involving the temporal artery, and it is more commonly seen in younger male patients. Kimura's disease, another rare disease, often involves the internal organs, including the kidney, but the simultaneous development of JTA and nephrotic syndrome has not been reported before. The patient in this case study was a middle-aged woman, which is atypical for JTA. The authors suggest that further investigation is needed to determine if JTA is a manifestation of Kimura's disease. [Extracted from the article]

This article discusses a rare case of squamous cell carcinoma (SCC) of the scalp that presented with symptoms similar to giant cell arteritis (GCA). GCA is a systemic vasculitis that primarily affects medium to large vessels and is commonly seen in older individuals. The patient in this case study had a history of SCC and presented with a temporal headache and jaw pain, which are typical symptoms of GCA. However, a biopsy revealed SCC invading the perivascular soft tissue, ruling out GCA. The article highlights the importance of considering malignancy, such as SCC, as a differential diagnosis in patients with suspected GCA who do not respond to steroids. [Extracted from the article]

Transient perivascular inflammation of the carotid artery (TIPIC) is a rare vascular disease characterized by inflammation of the carotid artery, leading to thickening of the carotid wall and unilateral carotidynia. This case report presents a 48-year-old woman who was diagnosed with TIPIC syndrome in a rheumatology outpatient clinic. The patient experienced right neck pain and numbness in the right hand, accompanied by local tenderness and increased arterial pulse. Laboratory tests showed no abnormalities, and imaging studies revealed inflammatory processes in the carotid artery. TIPIC syndrome is a self-limiting condition with a good prognosis and can be treated with nonsteroidal anti-inflammatory drugs. It is important for healthcare providers to be aware of this syndrome to prevent unnecessary treatment and to differentiate it from systemic vasculitis. [Extracted from the article]

This article discusses granulomatous mastitis (GM), a condition primarily affecting premenopausal women. It explores the use of systemic immunosuppression as a treatment for refractory GM and finds that drugs like methotrexate, hydroxychloroquine, and mycophenolate can induce remission and improve symptoms. However, the evidence for these treatments is limited, and more research is needed. The study emphasizes the importance of considering individual patient circumstances when deciding on treatment options. [Extracted from the article]

This article summarizes a study published in the International Journal of Rheumatic Diseases that explores the genetic association between iron status and two rheumatic diseases: polymyalgia rheumatica (PMR) and giant cell arteritis (GCA). The study used a method called Mendelian randomization (MR) to analyze genetic variants related to iron status and their connection to PMR and GCA. The findings indicate that genetically determined iron status is linked to a decreased risk of PMR but not GCA. The study suggests that iron supplementation may be a potential strategy for preventing or treating PMR, but further research is needed to confirm these findings. [Extracted from the article]

The article discusses the treatment of dermatomyositis (DM) with anti-melanoma differentiation-associated gene 5 (MDA5) antibody disease, specifically focusing on rapidly progressive interstitial lung disease (RP-ILD). RP-ILD is a serious complication of MDA5-DM, with a high mortality rate. The article highlights the importance of early and aggressive therapy for high-risk MDA5 positive patients to prevent RP-ILD. The use of combination therapy, including immunosuppression and steroids, is recommended. Medications such as mycophenolate, tacrolimus, and tofacitinib have shown promise in treating MDA5-ILD. The article emphasizes the need for prompt diagnosis, monitoring of antibody levels and immune activation markers, and timely decision-making regarding therapy. [Extracted from the article]

What the genes tell us in the VEXAS syndrome? Tocilizumab for treatment of cutaneous and systemic manifestations of vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome without myelodysplastic syndrome. 7 Huang H, Zhang W, Cai W. VEXAS syndrome in myelodysplastic syndrome with autoimmune disorder. VEXAS syndrome in a female patient with constitutional 45,X (turner syndrome). [Extracted from the article]

Aim: To explore demographic characteristics, biopsy length, and blood biomarker performance in an Australian cohort of patients who have undergone temporal artery biopsy (TAB) for giant cell arteritis (GCA). Methods: We extracted data on biopsies performed for GCA between January 2016 and December 2020 at public hospitals in Perth. Sensitivity, specificity, and area under the curve (AUC) were calculated for blood results. We evaluated the proportion of biopsies with post‐fixation length less than 15 mm and explored several length associations. Results: We retrospectively reviewed biopsies of 360 patients (65.8% female, mean age 72.1 years). Biopsy‐positive patients were older (6.0 years, P < 0.01), and had higher C‐reactive protein (CRP) (44.5 mg/L, P < 0.01), erythrocyte sedimentation rate (ESR) (18.9 mm/h, P < 0.01), and platelets (86.8 × 103/μL, P < 0.01) compared with biopsy‐negative patients. CRP and platelets had the highest AUCs at 0.76 and 0.71, respectively. Sensitivities for CRP and ESR were 96.2% and 91.5%, respectively. Specificities were comparatively low at 41.3% for CRP and 37.4% for ESR. The proportion of biopsies with sub‐optimal length was 55.9% and this varied significantly by site (P < 0.01). Smaller sites performed worse, with a sub‐optimal biopsy rate of 87% amongst the three smallest sites. Conclusion: ESR and CRP are helpful preliminary investigations, especially in identifying low‐risk patients, but their specificity is limited. Smaller centers had a higher proportion of biopsies with sub‐optimal length. Considering the importance of biopsy length for TAB diagnostic value, reviewing biopsy data may assist services in developing improvement strategies. [ABSTRACT FROM AUTHOR]

Giant cell arteritis (GCA) is a chronic vasculitic disorder predominantly affecting medium to large sized arteries, prevalent in the 50 plus age group. This case illustrates an atypical presentation of this disease in the form of bilateral sensorineural hearing loss (SNHL). Apart from the presence of constitutional and vertiginous symptoms, there were essentially no classical features of GCA. Differentials were broad including infection, malignancy and medication toxicity as well as brain, eye and ear syndromes such as Cogan's syndrome, all of which were eventually excluded. Her diagnosis was ultimately confirmed on positron emission tomography, which highlights the diagnostic importance of this modality. She was managed with corticosteroids then tocilizumab and is making a gradual recovery. Literature review demonstrates that SNHL is more prevalent than previously suggested in GCA, although this does not have widespread recognition. Mechanisms of SNHL in GCA include vascular occlusion, immunological mechanisms including cross reactivity with viral antigens and direct viral infection. SNHL does appear to improve with corticosteroids. This case emphasizes the importance of considering GCA as an important differential in SNHL. [ABSTRACT FROM AUTHOR]

More than 95% of the patients with GCA present with elevated levels of acute phase reactants (erythrocyte sedimentation rate and/or C-reactive protein [CRP]) at disease onset.2 The treatment of active GCA is based primarily upon glucocorticoids. The Cochrane Review indicated that, among patients with newly diagnosed or relapsing GCA, tocilizumab therapy leads to an increase in the proportion of patients achieving sustained remission at 12 months or more. Therefore, glucocorticoids are recommended at minimum possible doses and duration of treatment in older populations.7 In this regard, tocilizumab might be beneficial in terms of lowering the cumulative dose of glucocorticoids, as well as preventing prolonged use of these agents in patients with GCA. How beneficial and safe is tocilizumab for patients with giant cell arteritis?. [Extracted from the article]

Objectives: To evaluate the effectiveness of leflunomide as a steroid‐sparing agent among Indian patients with giant cell arteritis (GCA) and to assess the changes of "halo sign" within affected arteries, detected ultrasonographically, after remission. Methods: In this prospective observational study, patients fulfilling American College of Rheumatology criteria for GCA and having halo sign in temporal artery ultrasound were treated with leflunomide and predefined tapering dose of prednisolone. Ultrasounds of temporal and axillary arteries were done at baseline and after remission were achieved. Results: Twenty‐two GCA patients were followed up for a median duration of 24 months (interquartile range, IQR: 18‐33). All patients showed clinical improvement and steroids could be stopped in 17 out of 22 patients. Median time to achieve remission (symptom‐free with normal inflammatory markers) was 3 (95% confidence interval [CI]: 2.4‐3.6) months. Median time to achieve reduction to prednisolone dose <5 mg/d was 9 months (95% CI: 7‐11). Prednisolone dose could be reduced in all patients while on leflunomide, suggesting steroid‐sparing effect and a steroid‐free remission could be achieved after a median of 14 months (95% CI: 9.4‐18.6). Ultrasonographically, all patients showed improvement of halo signs, after 8 weeks (IQR 7.25‐12). Seven patients experienced a clinical relapse after 12 months (IQR: 5‐21) of initial remission. The predictors of relapse were duration of symptoms before initiation of immunosuppression therapy and delayed achievement remission by strict criteria. Conclusion: This study showed efficacy and safety of leflunomide as a steroid‐sparing agent in Indian GCA patients. [ABSTRACT FROM AUTHOR]

Aim: To assess the diagnostic accuracy of temporal artery ultrasound compared with temporal artery biopsy and clinical diagnosis in patients with suspected giant cell arteritis (GCA) over 10 years in an Australian center. Method: Patients presenting to Westmead Hospital with possible GCA from March 2011 to December 2020 were retrospectively identified. The following parameters were obtained from the medical record: clinical presentation, inflammatory markers, temporal artery ultrasound findings, and temporal artery biopsy report. Data were assembled in a 2 × 2 table; sensitivity and specificity of temporal artery ultrasound compared with temporal artery biopsy and clinical diagnosis were calculated. Results: Over the 10‐year study period, 65 temporal artery ultrasounds were performed in 63 patients (n = 65; 61.9% female) with a mean ± standard deviation age of 69.6 ± 12.3 years. Thirteen out of 65 (20%) temporal artery ultrasounds had findings suggestive of GCA. Twenty patients (31.7%) had a clinical diagnosis of GCA irrespective of sonographic or biopsy findings. Compared with temporal artery biopsy, temporal artery ultrasound had a sensitivity of 71.4% and specificity of 93.3%. Compared with clinical diagnosis made by the treating rheumatologist, temporal artery ultrasound had a sensitivity of 55% and specificity of 95.3%. Conclusion: Temporal artery ultrasound is a useful non‐invasive investigation in the assessment of suspected GCA. If positive in the setting of a suggestive clinical presentation, a temporal artery ultrasound probably avoids the need for a temporal artery biopsy. Temporal artery ultrasound could be more widely used in the clinical management of GCA. [ABSTRACT FROM AUTHOR]

This article presents a case report of a young female patient with widespread arterial stenoses that initially appeared to be Takayasu arteritis (TAK), a type of large vessel vasculitis. However, further investigation revealed that the patient had elevated levels of lipoprotein (a) (Lp(a)), a type of low-density lipoprotein associated with accelerated atherosclerosis. The patient also had a family history of atherosclerotic cardiovascular disease. The article emphasizes the importance of separately testing for Lp(a) in young patients with atherosclerotic cardiovascular disease, as it can help avoid misdiagnosis and guide appropriate treatment. [Extracted from the article]

Background: Takayasu arteritis (TAK) is a rare large vessel vasculitis, and epidemiological data on TAK are lacking in China. Thus, we designed this study to estimate the TAK prevalence and incidence in residential Shanghai, China. Methods: Data on diagnosed TAK cases aged over 16 years were retrieved from 22 tertiary hospitals in Shanghai through hospital electronic medical record systems between January 1, 2015 and December 31, 2017 to estimate the prevalence and incidence. A systematic literature review based on searches in PubMed, Ovid‐Medline, Excerpta Medica Database (EMBASE), Web of Science, and China National Knowledge Infrastructure (CNKI) was performed to summarize TAK distribution across the world. Results: In total 102 TAK patients, with 64% female, were identified. The point prevalence (2015‐2017) was 7.01 (95% CI 5.65‐8.37) cases per million, and the mean annual incidence was 2.33 (1.97‐3.21) cases per million. The average age of TAK patients was 44 ± 16 years, with the highest prevalence (11.59 [9.23‐19.50] cases per million) and incidence (3.55 [0.72 3.74] cases per million) in the 16 to 34 years population. Seventeen reports were included in the system review, showing that the epidemiology of TAK varied greatly across the world. The incidence and prevalence were both relatively higher in Asian countries, with the prevalence ranging 3.3‐40 cases per million and annual incidence ranging 0.34‐2.4 cases per million. Conclusions: The prevalence and incidence of TAK in Shanghai was at moderate to high levels among the previous reports. The disease burden varied globally among racial populations. [ABSTRACT FROM AUTHOR]

Vasculitis may rarely be seen in the course of myeloproliferative neoplasms (MPN). In vasculitis associated with hematological diseases, mostly small‐ and medium‐vessel involvement is expected, aortitis is very rare. It is not exactly known whether large‐vessel vasculitis associated with MPN is a paraneoplastic phenomenon or coincidental. We aimed to present an uncommon case diagnosed with chronic myeloid leukemia and Takayasu arteritis concurrently. [ABSTRACT FROM AUTHOR]

Aim: Diagnosing and monitoring vascular activity in giant cell arteritis (GCA) is difficult due to the paucity of specific serological biomarkers. We assessed the utility of 8 novel biomarkers in an inception cohort of newly suspected GCA patients. Method: Consecutive patients were enrolled between May 2016 and December 2017. Serum was collected within 72 hours of commencing corticosteroids and at 6 months. It was analyzed for levels of intra‐cellular adhesion molecule 1, vascular endothelial growth factor (VEGF), pentraxin 3, von Willebrand factor and procalcitonin (5‐plex R&D Systems multiplex assay) and interleukin (IL)6, IL12 and interferon‐γ (high‐sensitivity 3‐plex ProcartaPlex multiplex assay). A GCA specific positron emission tomography / computed tomography (PET/CT) scan was performed at enrolment with uptake in each vascular territory graded and summed to derive a total vascular score (TVS). Results: For the 63 patients enrolled, 12 (19%) had a final diagnosis of biopsy‐positive GCA and a further 9 had a clinical diagnosis of biopsy‐negative GCA. None of the 8 biomarkers was significantly higher in GCA patients compared with those with alternative diagnoses, or demonstrated a positive correlation with the PET/CT TVS. This was in contrast to the C‐reactive protein (CRP) and erythrocyte sedimentation rate (ESR) which were higher in the biopsy‐positive GCA cohort (P <.04) and showed weak positive correlations with the TVS (correlation coefficient 0.34, P <.01). Procalcitonin did not distinguish between GCA and infection. Concentrations of CRP, ESR, VEGF and pentraxin 3 decreased between diagnosis and 6 months in GCA patients. Conclusion: This study did not identify new serological biomarkers to assist in diagnosing or assessing the vasculitis burden in GCA. [ABSTRACT FROM AUTHOR]

Objectives: Bisphosphonates may cause autoimmune reactions via a cytokine‐mediated acute phase response. A case of giant cell arteritis (GCA) after zoledronate injection was recently reported. We aimed to evaluate this association by reviewing the incidence of GCA after bisphosphonate administration. Methods: This was a retrospective study using the medical claims data of elderly patients in the 20% Medicare random sample from 2008‐2014 who had received zoledronate or ibandronate. Patients who had a diagnosis claim of GCA within the past year before receiving either bisphosphonate were excluded. The development of GCA was assessed in 2 ways: GCA diagnosis claim within 28 days of bisphosphonate injection and another claim within 90 days of initial claim; and temporal artery biopsy claim within 28 days of bisphosphonate injection and GCA diagnosis claim within 90 days of biopsy. Due to the Centers for Medicare & Medicaid Services reporting requirements we excluded numbers less than 11 from analysis. Results: The incidence of GCA was 0.010% and 0.013% after zoledronate and ibandronate injection respectively. In the zoledronate group incidence was highest in patients aged 75‐85 years (0.011%), in Whites (0.011%), in the northeast census region (0.013%) and higher in females (0.011% vs 0.009%). All GCA cases noted in the ibandronate group involved White females. We are unable to report incidences by age and region due to the paucity of data. Conclusion: The incidence of GCA after bisphosphonate injection was not increased compared to the generally reported incidence in the USA. [ABSTRACT FROM AUTHOR]

Aim: Few, separate, small retrospective studies in giant cell arteritis (GCA) reported that patients: (a) with diabetes mellitus had less positive temporal artery biopsies (TAB); (b) on angiotensin‐converting enzyme inhibitors (ACE‐I) or angiotensin II receptor blockers (ARB) experienced fewer relapses; and (c) on statins experienced the same frequency of clinical complications and relapses as non‐exposed patients. This retrospective chart review study simultaneously investigated the impact of these 3 factors on a cohort of patients followed in 2 large Canadian centers (Hamilton and Toronto, ON). Methods: One hundred and thirty‐seven patients diagnosed with GCA between 1993 and 2015 were included in the study. Presenting symptoms, TAB results, disease complications and outcomes (relapses, duration of glucocorticoid use) were compared between exposed (diabetes/ACE/ARB/statin) and non‐exposed patients, with adjustment for main potential confounding variables. Results: Temporal artery biopsies was less often positive in patients with pre‐existing diabetes (relative risk 0.24; 95% CI: 0.069‐0.81). Patients who developed diabetes after diagnosis had a lower relapse‐free survival (adjusted hazards ratio [HR] 0.28; 95% CI: 0.095‐0.84). Patients taking ARBs prior to diagnosis were more likely to successfully discontinue glucocorticoids without a flare in the following 3 months (adjusted HR 2.46; 95% CI: 1.2‐5.3). Clinical complications and relapse rates did not differ between patients on statin therapy or not. Conclusion: Diabetic patients with GCA were less likely to have a positive TAB, and more likely to relapse. ARB therapy prior to diagnosis showed an association with success at discontinuing glucocorticoids. Statin therapy did not alter the clinical presentation or course of GCA. [ABSTRACT FROM AUTHOR]

Aim: Positron emission tomography/computed tomography (PET/CT) can detect cranial and large vessel inflammation in giant cell arteritis (GCA). We aimed to determine the change and significance of vascular activity at diagnosis and 6 months. Method: Newly diagnosed GCA patients underwent time‐of‐flight fluorine‐18‐fluoro‐2‐deoxyglucose PET/CT from vertex to diaphragm within 72 hours of commencing corticosteroids and were followed for 12 months. A 6 months scan was performed in patients with inflammatory features on biopsy or CT aortitis. Vascular uptake was visually graded by 2 blinded readers across 18 artery segments from 0 (no increased uptake) to 3 (very marked uptake). Scores were summed to give a total vascular score (TVS). Results: We enrolled 21 GCA patients and 15 underwent the serial scan. Twelve (57%) patients experienced a relapse and 5 of these had ischemic features of vision disturbance, jaw or limb claudication. The median TVS fell from 14 (interquartile range [IQR] 4‐24) at baseline to 5 (IQR 0‐10) at 6 months (P <.01) with reduction in both cranial and large artery scores. While the overall relapse rate was similar between patients with a high (≥10) and low baseline TVS, patients with high scores were numerically more likely to experience an ischemic relapse (33% vs 11%, P =.34). Five out of 15 patients had persistent uptake in at least 1 vessel on the serial PET/CT but none experienced a subsequent relapse. Conclusion: Vascular activity decreased in cranial and large arteries between diagnosis and 6 months. Persistent activity did not predict subsequent relapse. [ABSTRACT FROM AUTHOR]

Objectives: To measure serial interleukin (IL)‐6 levels in newly diagnosed patients with giant cell arteritis (GCA), treated in a randomized controlled trial of modified‐release prednisone (MR) vs immediate‐release prednisolone (IR) used in a tapering regimen conforming to British Society for Rheumatology GCA guidelines. Methods: Patients (n = 12) were randomized into 2 treatment arms (7 MR, 5 IR) and followed over 26 weeks. We measured IL‐6 with additional markers. Results: A significantly higher overall mean IL‐6 level (P < .05) was seen in IR (mean = 12.15, standard error [SE] = 1.90) compared with MR (mean = 4.39, SE = 1.84). Mean collagen type 1 cross‐linked C‐telopeptide (CTX) concentration was significantly higher (P <.05) in both groups at week 4 (mean = 0.29, SE = 0.04) compared with week 26 (mean = 0.13, SE = 0.02). MR patients had adrenocorticotropic hormone (ACTH) suppression compared with IR (P < .05) throughout without differences in cortisol levels (P = .34). No significant differences were seen between arms in other markers. Conclusion: Our study suggests that elevated levels of IL‐6 in new GCA are better suppressed by MR prednisone compared with IR prednisolone. CTX was significantly reduced in both treatment arms indicating early metabolic effect of glucocorticoids on bone. ACTH suppression with MR prednisone may reflect a greater impact on the hypothalamic‐pituitary‐adrenal axis although cortisol was not affected. MR prednisone warrants further investigation in GCA. [ABSTRACT FROM AUTHOR]

Aim: To investigate muscle stiffness changes in patients treated for giant cell arteritis (GCA) with high‐dose oral glucocorticoids. Methods: Using ultrasound elastography, shear wave velocity (SWV) was measured in the quadriceps, hamstrings and biceps brachii muscles of 14 patients with GCA (4 male, mean age ± SD, 68.2 ± 4.3 years) within the first 2 weeks of initiating glucocorticoid treatment (baseline) and repeated after 3 and 6 months treatment. Muscle strength and performance tests were performed at each visit. Baseline measures were compared with those from 14 healthy controls. Linear mixed models were used to test for change in patient measures over time. Results: At baseline, muscle SWV in patients was not significantly different from controls. With glucocorticoid treatment, there was a reduction in SWV in the leg but not the arm muscles. SWV decreased by a mean of 14% (range 8.3%‐17.3%; P = .001) after 3 months and 18% (range 10.2%‐25.3%; P < .001) after 6‐months in the quadriceps and hamstrings during the resting position. The baseline, 3 and 6 months mean SWV (±SD) for the vastus lateralis were 1.62 ± 0.16 m/s, 1.40 ± 0.10 m/s and 1.31 ± 0.06 m/s, respectively (P < .001). In the patient group as a whole, there was no significant change in muscle strength. However, there were moderate correlations (r = .54‐.69) between exhibiting weaker muscle strength at follow‐up visits and a greater reduction in SWV. Conclusion: Glucocorticoid therapy in patients with GCA was associated with a significant reduction in proximal leg muscle stiffness during the first 6 months. Future research should study a larger sample of patients for a longer duration to investigate if diminished muscle stiffness precedes signs of glucocorticoid‐induced myopathy. [ABSTRACT FROM AUTHOR]

Objective: To identify risk factors associated with disease relapse in giant cell arteritis (GCA). Methods: We reviewed data from 30 consecutive, newly diagnosed patients with GCA. The patients were divided according to relapse or non‐relapse status, and their baseline characteristics were compared. Results: Among the 30 patients, 8 relapsed at a median of 28 weeks from GCA diagnosis. Patients with relapse were male‐dominant (male: 88% vs female: 41%, P = 0.02) and showed a higher body mass index (BMI, 23 kg/m2 vs 19 kg/m2, P < 0.01) than non‐relapse patients. Patients with BMI ≥ 21 kg/m2 showed a significantly higher relapse rate than those with BMI < 21 kg/m2 during the 100‐week follow‐up (46% vs 0%, log‐rank test, P < 0.01). Conclusion: Higher BMI may be associated with relapse in patients with GCA. [ABSTRACT FROM AUTHOR]

There are six steps of evolution to validate and implement a new imaging technology: technical capacity, diagnostic and staging impact, management impact, treatment outcomes and cost-effectiveness. No studies have investigated whether LVV disease activity can be monitored by FDG PET/CT in patients on glucocorticoid-sparing drugs such as tumor necrosis factor (TNF- ) blocking agents for TA or interleukin-6 receptor blockade (tocilizumab) for GCA.[23] Nodal parameters of FDG PET/CT performed during radiotherapy for locally advanced mucosal primary head and neck squamous cell carcinoma can predict treatment outcomes: SUVmean and response rate are useful imaging biomarkers. Effect of glucocorticoid treatment on computed tomography angiography detected large-vessel inflammation in giant-cell arteritis. [Extracted from the article]

Aims: To investigate the utility of fluorine‐18‐labelled deoxyglucose positron emission tomography (FDG‐PET) in routine clinical practice to diagnose and monitor disease activity and treatment response in large vessel vasculitis in a South Australian cohort. Methods: We performed a retrospective clinical audit of adult patients who received a FDG‐PET at a tertiary referral center between August 2010 and August 2015, where the term "vasculitis" appeared in either the request or report. Results: A total of 45 patients met the inclusion criteria. Nine patients (20%) had a positive FDG‐PET for large vessel vasculitis. FDG‐PET was positive in 3/6 (50%) patients who met the American College of Rheumatology (ACR) criteria for giant cell arteritis or Takayasu's arteritis (TA) on retrospective review. A positive FDG‐PET for large vessel inflammation assisted the primary clinician in making the diagnosis of unclassified large vessel vasculitis in six patients. Four of the seven patients who had more than one scan for large vessel vasculitis demonstrated normalized FDG uptake on subsequent scans after a period on glucocorticoid treatment. The remaining three patients persisted in having increased FDG uptake on FDG‐PET imaging while on active treatment. Conclusion: Fluorine‐18‐labelled deoxyglucose positron emission tomography has a role in the diagnosis of large vessel vasculitis, particularly in patients with a high suspicion of active large vessel vasculitis who do not meet the ACR criteria. FDG‐PET may have a role in monitoring disease activity in selected patients with large vessel vasculitis especially in identifying occult sites of large vessel inflammation or to titrate prednisolone therapy. [ABSTRACT FROM AUTHOR]

Aim: To determine the diagnostic yield of 18F‐fluorodeoxyglucose positron emission tomography (PET) in disease activity assessment of large vessel vasculitides (LVV). Methods: Patients with LVV who had undergone PET (between 2004 and June 2010) or PET co‐registered with computed tomography (PET/CT; since June 2010) were identified. Clinical disease activity was assessed using established scoring systems. PET images were reviewed by two blinded nuclear medicine physicians. Uptake of the aortic wall was compared to the liver uptake utilizing a visual 4‐point score, with a vessel wall uptake similar or higher than liver uptake considered as active disease. Various target‐to‐background ratios were calculated. Receiver operator characteristics analysis was applied to determine the diagnostic accuracy of PET for detecting clinically active disease. Interobserver agreement of visual readings was measured with Cohen´s kappa. Results: Eighty examinations in 62 patients were analyzed, with a mean time between diagnosis and PET of 106 ± 171 weeks. Fifty‐seven cases were finally classified as clinically active and 23 cases as clinically inactive. With a cut‐off value of 1.3, the aorta‐to‐liver ratio yielded a sensitivity and specificity of 84.2% and 82.6% (area under the curve 0.9). Overall, sensitivity and specificity of visual analysis were 68.4% and 91.3%, but sensitivity decreased to 54% in patients treated for more than 3 months. Interobserver agreement of visual rating was excellent (κ: 0.93). Conclusion: Positron emission tomography is specific and reliable in disease activity assessment of LVV, but lacks sensitivity for detecting active disease in patients under long‐term immunosuppressive treatment. [ABSTRACT FROM AUTHOR]

We present a case of an elderly, immunosuppressed patient with rheumatoid arthritis who was not appropriately vaccinated, and subsequently developed herpes zoster ophthalmicus, which initially presented similar to giant cell arteritis. Evidence‐based vaccinations are integral in decreasing the incidence of preventable diseases and promoting optimal health at the individual and population level. Although the patient ultimately did not suffer any long‐term adverse sequelae, this case highlights the importance of vaccination in the rheumatology setting, and to consider both inflammatory and infectious causes of headache and vision changes in the elderly. [ABSTRACT FROM AUTHOR]

Takayasu arteritis (TA), despite being classified as a large vessel vasculitis, has distinct genetic, pathological and clinical features as compared to giant cell arteritis. It is a rare disease seen more commonly in Asian countries. The challenge lies in assessing the degree of inflammation in a narrowed vessel and immunosuppressive therapy improves inflammatory features but is unable to open up an occluded vessel. It may have a positive effect on retarding further occlusion. Like antineutrophil cytoplasmic antibody‐associated vasculitis, TA needs a collaborative effort to do randomized controlled therapy to provide benefit to patients. [ABSTRACT FROM AUTHOR]

Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are forms of large‐vessel vasculitides that affect the aorta and its branches. There is ongoing debate about whether they are within a spectrum of the same disease or different diseases. Shared commonalities include clinical features, evidence of systemic inflammation, granulomatous inflammation on biopsy, role of T‐helper (Th)‐1 and Th17 in the pathogenesis, and, abnormalities of the aorta and its branches on imaging. However, there are also several differences in the geographic distribution, genetics, inflammatory cells and responses to treatment. This review highlights the similarities and differences in the epidemiology, pathogenesis, clinical manifestations, imaging findings and treatment responses in these conditions. Current data supports that they are two distinct conditions despite the numerous similarities. [ABSTRACT FROM AUTHOR]

We discuss recent and prospective research in small and large vessel vasculitis. Large cohorts of Takayasu arteritis (TA) have been recently published from across the world, clarifying our understanding of this uncommon disease. Novel open‐ended approaches like large‐scale genotyping, proteomics and metabolomics have helped gain novel insights into TA, giant cell arteritis (GCA) and anti‐neutrophil cytoplasmic antibody‐associated vasculitis (AAV). Recent advances in the imaging of TA and GCA offer promise for earlier diagnosis and better monitoring of response to therapy. Although two randomized controlled trials of abatacept and tocilizumab failed to meet their primary end‐points, successful large‐scale studies of abatacept and tocilizumab in GCA hold promise for better disease control. While cyclophosphamide has revolutionized the management of AAV, increasing use of rituximab as an alternative induction regimen, as well as use of novel approaches involving reduced or no corticosteroid use for AAV and alternative agents such as avacopan (a complement 5a receptor antagonist) hold promise for lesser toxic induction regimens in the future. Increasingly, the risk of cardiovascular events and comorbidities such as osteoporosis are being recognized as factors affecting long‐term prospects of patients with vasculitis. There is a shift in emphasis to utilize patient‐reported outcomes to more accurately gauge the impact of vasculitides and their treatment. [ABSTRACT FROM AUTHOR]

The classification of the systemic vasculitides has been controversial for several decades. The Chapel Hill consensus Conference definitions originally developed in 1994, but revised and extended in 2012 are now widely accepted. The American College of Rheumatology (ACR) criteria were first published in 1990, are now generally accepted to be out of date and new criteria are needed. More recently the classical division of the ANCA vasculitides using clinical phenotype has come under scrutiny with evidence from epidemiological, genetic and outcome studies that perhaps these conditions should be classified on the basis of ANCA specificity into PR3‐ANCA positive and MPO‐ANCA positive groups. The traditional distinction between giant cell arteritis and Takayasu arteritis has been questioned and some recent studies of GCA have included patients with only extra‐cranial disease. The Diagnostic and Classification Criteria of Vasculitis study (DCVAS) will provide new validated classification criteria for the systemic vasculitides. [ABSTRACT FROM AUTHOR]

The classification of the systemic vasculitides has been controversial for several decades. The Chapel Hill consensus Conference definitions originally developed in 1994, but revised and extended in 2012 are now widely accepted. The American College of Rheumatology (ACR) criteria were first published in 1990, are now generally accepted to be out of date and new criteria are needed. More recently the classical division of the ANCA vasculitides using clinical phenotype has come under scrutiny with evidence from epidemiological, genetic and outcome studies that perhaps these conditions should be classified on the basis of ANCA specificity into PR3‐ANCA positive and MPO‐ANCA positive groups. The traditional distinction between giant cell arteritis and Takayasu arteritis has been questioned and some recent studies of GCA have included patients with only extra‐cranial disease. The Diagnostic and Classification Criteria of Vasculitis study (DCVAS) will provide new validated classification criteria for the systemic vasculitides. [ABSTRACT FROM AUTHOR]

Giant cell arteritis is the commonest primary vasculitis of the elderly. However, the prevalence does vary widely between populations with highest incidence amongst Northern Europeans and lowest amongst East Asians. Preliminary studies suggest that clinical manifestations may differ between different populations. Newer diagnostic approaches including ultrasound, MR angiography and PET imaging are under review. While there have been recent advances in the diagnosis of GCA particularly with regard to imaging, there is an urgent need for improvements in methods of diagnosis, treatment and requirement for screening. Glucocorticoid treatment remain the backbone of therapy. However, glucocorticoid therapy is associated with significant adverse effects. Conventional and novel immunosuppressive agents have only demonstrated modest effects in a subgroup of steroid refractory GCA due to the different arms of the immune system at play. However, recently a study of IL‐6 blockade demonstrated benefit in GCA. Newer approaches such as fast‐track pathways can also result in improvements in consequences of GCA including blindness. [ABSTRACT FROM AUTHOR]

Dear Editor, Paraneoplastic vasculitis is a rare complication which can be associated with myeloproliferative disorders (MPD). Skin biopsy showing leukocytoclastic vasculitis and sural nerve biopsy showing lymphocytic vasculitis (hematoxylin and eosin staining) gl DISCUSSION Vasculitis may behave as a paraneoplastic syndrome associated with various hematological malignancies. [Extracted from the article]

Abstract: Background: The hypothesis that patients with polymyalgia rheumatica (PMR) or giant cell arteritis (GCA) have a high risk for future cardiovascular diseases has not been adequately tested. The aim of this study is to evaluate this hypothesis in Japan, where the prevalence and severity of PMR and GCA are the lowest. Methods: A propensity score matched cohort study was conducted at St. Luke's International Hospital, Tokyo, Japan, from 2003 to 2016. We included all patients who were diagnosed as PMR or GCA cases and matched comparators with a proportion of 1 : 2. Our primary outcome was newly diagnosed cardiovascular disease. The propensity score was calculated using logistic regression with forward stepwise selection in 30 variables. Kaplan–Meier curves were drawn and the log‐rank test and Cox proportional hazard model were performed for survival analyses. Two types of sensitivity analyses were conducted to confirm the results. Results: Among 2461 potential patients, the propensity score identified 504 (168 cases and 336 comparators) patients. During follow up (median 839.5 days), 110 (21.8%) developed cardiovascular diseases. The Kaplan–Meier curves between those with and without PMR or GCA were not significantly different (P = 0.85). The Cox proportional hazard model calculated the hazard ratio (HR) of those with PMR or GCA compared to those without as 0.96 (95% CI: 0.64–1.46). The results from sensitivity analyses were consistent (HR 0.70–1.06). Conclusion: Patients with PMR or GCA may not have a higher risk of future cardiovascular diseases among the Japanese population. The sensitivity analyses and sample size calculation supported the results. [ABSTRACT FROM AUTHOR]

Abstract: Objective: A feasibility study to assess efficacy and safety of modified release (MR) prednisone (Lodotra™) compared to immediate release (IR) prednisolone in patients with newly diagnosed giant cell arteritis (GCA). Methods: Twelve patients with new diagnosis of GCA were initially treated with high‐dose prednisolone (40–60 mg) daily for 4 weeks and then randomized to two open arms to continue tapering steroid treatment with either standard IR prednisolone or MR prednisone. Patients were reviewed every 2 weeks either face to face or by telephone, for a total of 26 weeks. Disease activity, steroid‐related side effects, sleep disturbance, fatigue scores and blood tests were systematically monitored. The primary endpoint (efficacy) was defined as the proportion of patients achieving persistent clinical disease control (without features of active disease and remaining flare free at 26 weeks) in each arm. Results: At 26 weeks, 6/7 patients taking MR prednisone were in persistent control, compared with 4/5 receiving IR prednisone. One patient in each group suffered a disease flare necessitating an increased steroid dose. There were no statistically significant differences between the groups in terms of reduction in inflammatory markers, Health Assessment Questionnaire, visual analogue scale, fatigue and improvement in EuroQol 5D scores. Conclusion: This trial shows that MR prednisone appears to be a safe and effective treatment for GCA with a similar outcome profile to standard IR prednisolone. [ABSTRACT FROM AUTHOR]

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 I2 of 89%. Conclusion Our study demonstrated a statistically significant increased risk of PAD among patients with GCA. [ABSTRACT FROM AUTHOR]

The author discusses development of research regarding therapies for treatment of large vessel vasculitis (LVV). Topics discussed include illustration of stages involved in disease progression, observation of high-dose glucocorticoids (GC) as mainstay in LVV treatment, utilization of Methotrexate as adjunctive therapy leads to reduction in GC exposure, involvement of Leflunomide as steroid-sparing for treatment of giant cell arteritis (GCA) and elucidation of Abatacept as anti-T cell therapy.

Aim Although Takayasu arteritis ( TAK) and giant cell arteritis ( GCA) have been considered as distinct disease entities, similarities of these diseases have been recently reported. However, little data is available regarding this issue in Japanese patients with TAK and GCA. In addition, the classification criteria for TAK established in 1990 by the American College of Rheumatology ( ACR) have been criticized due to the age restriction for disease onset (≤ 40 years). Thus, we aimed to compare the clinical characteristics of Japanese patients with TAK and those with GCA and to clarify whether clinical differences existed between patients with early-onset (≤ 40 years) and late-onset (> 40 years) TAK. Methods We enrolled 86 patients with TAK and nine with GCA who visited our department from 1990 to 2014. The diagnoses of TAK and GCA were based on the criteria of the Japanese Circulation Society and the ACR, respectively. Results Mean ages at onset for TAK and GCA were 36.4 and 71.0 years, respectively. Patients with TAK had significantly higher incidences of aortic regurgitation and carotid and subclavian arterial involvement, lower frequencies of polymyalgia rheumatica, and better prognoses than those with GCA. In contrast, the clinical characteristics, distribution of arterial lesions, treatments administered, and prognoses of patients with early- and late-onset TAK were comparable. Conclusions These results suggested that TAK and GCA differed substantially, and that the age restriction (≤ 40 years) may not be necessary for the diagnosis of TAK. [ABSTRACT FROM AUTHOR]

The article discusses a study focusing on fluorine-18 fluoro-2-deoxyglucose (18F-FDG) uptake in giant cell arteritis (GCA) patients. Topics discussed include the use of positron emission tomography/computed tomography (PET/CT) in the study; the branches of external carotid artery (ECA); and the detection of the arteritis of the superficial temporal and occipital vessels.

Primary systemic vasculitis is a group of heterogeneous disorders, characterized by inflammation of blood vessels causing end organ damage from ischemia, aneurysm formation or dissection. Delay in the early diagnosis owing to non-specific symptoms, lack of definitive serological tests, limited availability of biopsy and standard imaging tests pose significant challenge in the management of these diseases. 18F-fluorodeoxyglucose ( FDG) positron emission tomography ( PET) scanning is being increasingly used in the management of systemic vasculitis, especially the large vessel vasculitides: giant cell arteritis ( GCA) and Takayasu arteritis ( TAK). FDG- PET involves detection of positron rays emitted by FDG, a fluorinated glucose analogue which is avidly taken up by metabolically active inflammatory cells in the walls of involved blood vessels. 18F- FDG- PET scan, especially when combined with computed tomography or magnetic resonance imaging ( MRI) can give information about active inflammation as well as structural damage associated with vasculitis. In patients with GCA, FDG- PET has acceptable sensitivity and specificity for the early diagnosis in non-cranial GCA, cranial GCA with negative biopsy, assessment of immediate response to treatment, predicting prognosis, but has limited value in serial follow-up and prediction of relapses. In TAK, FDG- PET can be useful for early diagnosis and probably for serial assessment of disease activity. FDG- PET has a limited role in medium and small vessel vasculitis. [ABSTRACT FROM AUTHOR]