Chetan Bhikhubhai Mukhtyar

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• Doctor of Medicine
• Consultant at Norfolk and Norwich University Hospitals NHS Foundation Trust

Objective: Ultrasonography has been proposed as the initial diagnostic modality in suspected giant cell arteritis. Proposed quality standards advocate for a certified sonographer. Currently, there are no formal training programmes, and single educational events do not suffice as certification. We developed a preceptorship programme for diagnostic u...

Objective Ultrasonography is crucial for diagnosing giant cell arteritis (GCA); however, training opportunities are rare. This study tested the reliability of ultrasonography findings and measurements of the intima-media thickness (IMT) among ultrasonography experts by using phantoms of the axillary (AA) and temporal arteries (TA) created with high...

Objective To report the annual and age-specific incidence of ANCA associated vasculitis and polyarteritis nodosa in the adult population of Norfolk County, UK. Methods Individuals newly diagnosed with ANCA associated vasculitis or polyarteritis nodosa between 1st Jan 2011 and 31st December 2020, residing in the postal areas of NR1-NR30 were includ...

Background/Purpose: Ultrasonography has been validated as a diagnostic tool for giant cell arteritis (GCA). Due to the urgent need for glucocorticoid treatment in acute GCA and the fact that ultrasound detected inflammation normalizes rapidly upon start of treatment, it is challenging to identify patients with pathological vessels for educational p...

Polymyalgia rheumatica (PMR) is characterised by stiffness and pain in the shoulders, hips, and neck and presents most commonly in the eighth decade. It can coexist with giant cell arteritis and the two diseases may share some pathophysiological mechanisms. This narrative review considers present-day ideas about PMR in a historical context, from th...

Objectives To establish the sensitivity and negative predictive value of a multimodal pathway incorporating ultrasonography, 18-fluorodeoxyglucose labelled positron emission tomography computed tomography and temporal artery biopsy for the diagnosis of giant cell arteritis. Methods 1000 consecutive referrals for a new diagnosis of giant cell arter...

Background Ultrasonography (US) for diagnosis of large vessel vasculitis (LVV) is a recommended first line diagnostic procedure. We have been running an US led diagnostic pathway in our centre since January 2017. This pathway is also used to diagnose relapsing disease including in patients who are present with proximal shoulder girdle pain and stif...

Background ANCA associated vasculitis (AAV) and Polyarteritis Nodosa (PAN) are primary systemic vasculitides with the potential to cause organ or life-threatening disease. Our centre serves as the secondary care provider for the county of Norfolk, UK. The annual incidence of Granulomatosis with Polyangiitis (GPA) and microscopic polyangiitis (MPA)...

https://scientific.sparx-ip.net/archiveeular/?c=a&view=3&item=2024POS1335

Objectives To analyse the new evidence (2018–2022) for the management of systemic lupus erythematosus (SLE) to inform the 2023 update of the European League Against Rheumatism (EULAR) recommendations. Methods Systematic literature reviews were performed in the Medline and the Cochrane Library databases capturing publications from 1 January 2018 th...

Background/Aims Ultrasonography (US) for diagnosis of large vessel vasculitis (LVV) is a recommended first line diagnostic procedure. We have been running an US led diagnostic pathway in our centre since January 2017. This pathway is also used to diagnose relapsing disease. We present US defined anatomical distribution of relapses in consecutive pa...

Background/Aims Ultrasonography (US) for diagnosis of large vessel vasculitis (LVV) is a recommended first line diagnostic procedure. Our county has an annual incidence of 57.5/million for primary LVV (giant cell arteritis (GCA) 48.3/million; Takayasu 2.5/million; unclassified LVV 6.4/million). Our protocolised fast-track US pathway has reduced vis...

Background/Aims Glucocorticoids continue to be the main treatment for giant cell arteritis (GCA). It is widely recognised that treatment with oral glucocorticoids is associated with adverse events including adrenal insufficiency. Reports suggest the risk of adrenal insufficiency is dependent on the dose and the duration of therapy. The Norwich regi...

Background/Aims Patient education in rheumatology is of proven value to educate as well as to reduce demand on health care services. This can be delivered in a multitude of ways, in person and more recently with the use of multi-media modalities. There is limited evidence to specifically demonstrate the benefit of patient education for patients wit...

Background/Objectives: Interstitial Lung Disease (ILD) in ANCA-associated Vasculitis (AAV) has the appearance of a progressive fibrotic disease on imaging and primarily affects the interstitial lung compartment and is usually associated with MPO- ANCA. Whether patients with AAV-ILD display a similar fibrotic pattern in their kidneys is unclear. Met...

Objective To develop international consensus-based recommendations for early referral of individuals with suspected polymyalgia rheumatica (PMR). Methods A task force including 29 rheumatologists/internists, 4 general practitioners, 4 patients and a healthcare professional emerged from the international giant cell arteritis and PMR study group. Th...

Objectives To update the EULAR recommendations for the management of systemic lupus erythematosus (SLE) based on emerging new evidence. Methods An international Task Force formed the questions for the systematic literature reviews (January 2018–December 2022), followed by formulation and finalisation of the statements after a series of meetings. A...

Introduction: Prompt diagnosis and treatment of polymyalgia rheumatica (PMR) is crucial to prevent long-term complications and improve patient outcomes. However, there is currently no standardized approach to referral of suspected PMR patients to rheumatologists, leading to inconsistent management practices. The objective of this systematic review...

Objectives: To report the annual incidence of primary large vessel vasculitis (LVV) in the adult population of Norfolk County, UK, including giant cell arteritis (GCA) (in those ≥50 years) and Takayasu arteritis (TAK). Methods: Individuals diagnosed by histology or imaging who lived in NR1-NR30 postcode districts were included. Validated criteri...

Background There are no data on the collective incidence of the large vessel vasculitides. The data of incidence of GCA and Takayasu arteritis in the UK has been based on clinical coding in routine administrative datasets. There are no data on the incidence of these diseases based on clinically verified diagnoses. We studied the incidence of the la...

Background GCA and Takayasu arteritis are the two recognised forms of LVV. In addition, there is recognition of LVV which are not easily classified into the two forms. Classification criteria have been proposed since 1990 and have been recently updated. Classification criteria should be able to minimise the number of cases that are unclassifiable a...

Background: We present the largest study of the frequency and nature of visual complications in a cohort of 350 patients consecutively diagnosed with giant cell arteritis (GCA). Methods: All individuals were assessed using structured forms and diagnosed using imaging or biopsy. A binary logistic regression model was used to analyse data for pred...

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of several organs. The diagnosis and management of EGPA are often challenging and require an integrated, multidisciplinary approach. Curre...

Background/Aims There are no data on the collective incidence of the large vessel vasculitides. The incidence of GCA and Takayasu arteritis in the UK has been based on clinical coding in routine administrative datasets. There are no data on the incidence of these diseases based on clinically verfied diagnoses. We studied the incidence of the large...

Background/Aims Giant cell arteritis and Takayasu arteritis are the two recognised forms of large vessel vasculitis. Clinically there is recognition of large vessel vasculitides which are not easily classified into the two forms. Classification criteria have been proposed since 1990 and have been updated in 2022. Classification criteria should be a...

Background Since the publication of the EULAR recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in 2016, several randomised clinical trials have been published that have the potential to change clinical care and support the need for an update. Methods Using EULAR standardised operating pro...

Giant cell arteritis (GCA) is a rare condition that causes inflammation of blood vessels. Early diagnosis and treatment is essential to prevent ischaemic complications, including blindness, tongue necrosis and stroke. GCA can present with orofacial symptoms, such as toothache and pain/difficulty in chewing, which may cause individuals with GCA to f...

Dear Sir, I read the paper from Yamaguchi et al. published in the January 2023 edition of Modern Rheumatology with great interest [1]. I thank them for sharing their observations and insights with the world about the differences between those individuals who appear to have extracranial giant cell arteritis (GCA) and those with just cranial arterial...

Objective GCA is the commonest primary systemic vasculitis in adults, with significant health economic costs and societal burden. There is wide variation in access to secondary care GCA services, with 34% of hospitals in England not having any formal clinical pathway. Quality standards provide levers for change to improve services. Methods The mul...

Objectives To develop an Outcome Measures in Rheumatology (OMERACT) ultrasonography score for monitoring disease activity in giant cell arteritis (GCA) and evaluate its metric properties. Methods The OMERACT Instrument Selection Algorithm was followed. Forty-nine members of the OMERACT ultrasonography large vessel vasculitis working group were inv...

Dear Editor, Ultrasonography is a diagnostic modality that has been tested to OMERACT standards for the diagnosis of GCA [1]. It is a bedside tool with ubiquitously available technology. It can lead to earlier diagnosis or exclusion of GCA, potentially improving outcomes and minimizing glucocorticoid toxicity. Ultrasonography is more cost effective...

Dear Editor, A 68-year-old man of Northern European ancestry presented with a 3-month history of constitutional symptoms to his general practitioner. The detection of a normochromic normocytic anaemia, an acute phase response and an elevated prostate-specific antigen prompted a referral to a rapid diagnostic service for cancer. A CT scan of the tho...

Background The ophthalmic features of giant cell arteritis (GCA) involve a spectrum of severity from transient symptoms to bilateral visual loss from anterior ischaemic optic neuropathy or less commonly central retinal or cilioretinal artery occlusion. Reported ocular involvement varies between 10-70%.1,2. At the Norfolk and Norwich University Hosp...

Background While the number of patients with rheumatic musculoskeletal diseases (RMDs) is increasing worldwide, there is no adequate increment in the number of health care professionals, leading to the urgent need for new forms of care to take pressure from health care systems.1 2 Telehealth comprises a number of different types of interventions wi...

Background Glucocorticoid therapy is the mainstay treatment for GCA. There is no consensus on the dose of prednisolone to be used. Prednisolone regimens used in clinical trials have reported relapse rates of 66 to 92%. The long-term follow-up of Tocilizumab and prednisolone for 1-year showed a relapse rate of 74% at 2 years. The Norwich Regimen is...

Background The definition of Takayasu arteritis includes the age of onset of disease as being <50 years. It has been reported most frequently in young females from Asian populations. Objectives We investigated whether the epidemiological features are the same in Norfolk, UK, where the population is predominantly of Northern European ancestry. Met...

Background Ultrasonography has become the recommended first line investigation for suspected giant cell arteritis (GCA). However, it is still not yet widely available in the UK due to a paucity of skilled rheumatologists. Effective training programmes have been developed, but subsequent hands-on practice is needed to become truly proficient. There...

Objective To perform a systematic literature review (SLR) on different outcomes of remote care compared with face-to-face (F2F) care, its implementation into clinical practice and to identify drivers and barriers in order to inform a task force formulating the EULAR Points to Consider for remote care in rheumatic and musculoskeletal diseases (RMDs)...

Background: Remote care and telehealth have the potential to expand healthcare access, and the COVID-19 pandemic has called for alternative solutions to conventional face-to-face follow-up and monitoring. However, guidance is needed on the integration of telehealth into clinical care of people with rheumatic and musculoskeletal diseases (RMD). Ob...

Background/Aims Glucocorticoid therapy is the mainstay treatment for giant cell arteritis (GCA). There is no consensus on the dose of prednisolone to be used. Prednisolone regimens used in clinical trials have reported relapse rates of 66-92%. The long-term follow-up of tocilizumab and prednisolone for one year showed a relapse rate of 74% at two y...

Background/Aims The objective of this project is to map services essential to delivering high quality care in giant cell arteritis (GCA) across England, identifying gaps in provision and thereby help to remove inequalities. To do this however, there must first be agreement on what these best practice services and standards are. Methods A steering...

Abstract Background/Aims The definition of Takayasu arteritis includes the age of onset of disease as being <50 years. It has been reported most frequently in young females from Asian populations. We investigated whether the epidemiological features are the same in Norfolk, where the population is predominantly of northern European ancestry. Metho...

Dear Editor, Setting up a validated ultrasonography service for the diagnosis of GCA is a worthwhile but laborious task [1]. Ho et al. should be complimented on setting up such a service and sharing this interesting vignette with us [2]. They have shared a case where an individual with typical clinical and laboratory features of GCA was also found...

Dear Editor, We read with interest the article by Andel et al. [1], which provides valuable guidance for rheumatologists for managing suspected GCA. They define a ‘fast-track clinic’ (FTC) as a service that can see patients within 48 h of referral, ideally with vascular ultrasound imaging. Although urgency of treatment is universally recommended, t...

Background/Aims Ultrasonography has become the recommended first line investigation for suspected giant cell arteritis (GCA). However, it is still not yet widely available in the UK due to a paucity of skilled rheumatologists. Effective training programmes have been developed, but subsequent hands-on practice is needed to become truly proficient. T...

Background/Aims The ophthalmic features of giant cell arteritis involve a spectrum of severity, from transient symptoms to bilateral visual loss due to anterior ischaemic optic neuropathy or, less commonly, central retinal or cilioretinal artery occlusion. Data of reported ocular involvement are varied, with a range of 10-70%. The variation may be...

We have created definitions for ultrasonographic abnormalities of Giant Cell Arteritis. The 'halo' sign is a 'homogenous, hypoechoic wall thickening, well delineated towards the luminal side, visible both in longitudinal and transverse planes, most commonly concentric in transverse scans.' At the superficial temporal artery, the interobserver relia...

Objective: To develop recommendations for cardiovascular risk (CVR) management in gout, vasculitis, systemic sclerosis (SSc), myositis, mixed connective tissue disease (MCTD), Sjögren's syndrome (SS), systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). Methods: Following European League against Rheumatism (EULAR) standardised...

We describe two cases of giant cell arteritis where involvement of the superficial temporal artery and maxillary artery were demonstrated using colour doppler ultrasonography. Maxillary artery involvement is responsible for the symptoms of jaw claudication and toothache, and even headaches might be due to the involvement of the middle meningeal art...

Giant cell arteritis (GCA) is a systemic vasculitis with numerous potential complications and societal costs. After the publication of international guidelines, we found a number of deficiencies in the local care pathway of patients suspected to have GCA. These included poor referral and management pathways, and absence of dedicated monitoring and...

Objective : Our primary objective was to develop an Outcome Measures in Rheumatology (OMERACT) core domain set to capture the impact of glucocorticoids (GC), both positive and negative, on patients with Rheumatic conditions. Methods : The OMERACT Filter 2.1 was used to guide core domain selection. Systematic literature reviews, qualitative studies...

Objectives To define chronic ultrasound lesions of the axillary artery (AA) in long-standing giant cell arteritis (GCA) and to evaluate the reliability of the new ultrasound definition in a web-based exercise. Methods A structured Delphi, involving an expert panel of the Large Vessel Vasculitis subgroup of the Outcome Measures in Rheumatology (OME...

Colour-doppler ultrasonography is the first measure to allow objective bedside assessment of Giant Cell Arteritis (GCA). This paper discusses the evidence using the OMERACT filter. Consensus definitions for ultrasonography changes were agreed by Delphi process, with the 'halo' and 'compression' sign being characteristic. The 'halo' is sensitive to...

Objectives Longer life expectancy has resulted in people living with an increasing number of comorbidities. The ‘average individual’ with inflammatory arthritis (IA) has 2 comorbidities which contribute to higher mortality, poorer functional outcomes and increased healthcare utilisation and cost. Whilst a number of studies have investigated the pre...

Purpose We report two patients who presented initially to ophthalmology clinics with symptoms and signs of orbital inflammation that led to a diagnosis of Erdheim-Chester Disease (ECD). Observations ECD is a rare form of non-Langerhans cell histiocytosis (LCH) which is characterised by multi-system organ involvement and poor prognosis with standar...

Background/Purpose: The OMERACT Ultrasound (US) large vessel vasculitis task force has recently defined the US appearance of normal axillary arteries (AA) and the key elementary US lesions in acute giant cell arteritis (GCA) of the AA (i.e.:“non-compressible halo sign”). The aim of the present study was to assess the reliability of a new OMERACT de...

Conception, pregnancy and childbirth are a biological necessity, a unique privilege and birthright and yet something that mothers suffering with vasculitis cannot take for granted. Takayasu arteritis and Behcet’s disease mainly, but also ANCA associated vasculitis and IgA vasculitis are of relevance in this population. A diagnosis of systemic vascu...

Background EULAR has recommended ultrasonography (US) as first imaging modality for diagnosis of Giant Cell Arteritis (GCA) ¹ . For patients with a high pre-test probability who have a negative scan, the recommendation is to use another diagnostic modality like temporal artery biopsy (TAB) to make a diagnosis ¹ . We know that a fast-track pathway i...

Background Biological drugs have revolutionized the treatment of immune-mediated inflammatory diseases (IMIDs). Current guidelines reserve these drugs for patients with severe refractory disease.Biologic drugs are expensive, but as they reach patent expiry, the introduction of lower-cost biosimilars reduces their impact on health care budgets. It i...

Objectives The EULAR core dataset for observational studies in giant cell arteritis (GCA) does not include glycated haemoglobin (HbA1c). A multivariable score to stratify pre-test probability of GCA also does not include HbA1c. There have been contradictory reports about Diabetes Mellitus (DM) being a risk factor for GCA. We report the first study...

Background The National Congenital Anomaly and Rare Disease Registration Service (NCARDRS) records those people with congenital anomalies and rare diseases across the whole of England. The NCARDRS Rare Rheumatology Project is establishing methodologies to identify and register people with rare rheumatic diseases. Hospital Episode Statistics (HES),...

Background The contemporary prevalence of ANCA-associated vasculitis (AAV) in England is unknown. Hospital Episode Statistics (HES) contain data on every hospital and day case NHS admission in England since 1997. In collaboration with the National Congenital Anomaly and Rare Disease Registration Service (NCARDRS) we validated the diagnosis of AAV u...

Background Giant cell arteritis (GCA) has a relapsing, remitting course with ischemic/vascular damage in a number of cases. Glucocorticoids (GC) remain mainstay of treatment with SAEs e.g. diabetes and fractures commonly seen. The GiACTA trial of tocilizumab (TCZ) in GCA led to NICE approval for 12 month’s therapy in relapsing/ refractory disease....

Background Inflammatory arthritis (IA) predisposes to comorbidities (CC) including cardiovascular disease, osteoporosis, depression, infections, and cancer. CC are associated with higher mortality, poorer quality of life, and greater utilisation of health resources. Treating CC can be challenging and their impact is not addressed by current guideli...

Vasculitis is rare in the context of testicular lesions but, when found, can be classified as a single organ vasculitis or part of a multi-organ inflammatory process. In the context of a patient with a pre-existing autoimmune disorder, this finding might cause diagnostic confusion and preferentially bias a physician towards attributing the conditio...

[This is the executive summary of Rituximab for maintenance of remission in ANCA-associated vasculitis: expert consensus guidelines: full guideline, doi: 10.1093/rheumatology/kez640]

Background Since the publication of the European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis (LVV) in 2009, several relevant randomised clinical trials and cohort analyses have been published, which have the potential to change clinical care and therefore supporting the need to update the original...

Objective The aim of this study was to survey participants with PMR to evaluate the face validity, acceptability and domain match of proposed candidate outcome measures. Methods A structured, online, anonymous survey was disseminated by patient support groups via their networks and online forums. The candidate outcome measures comprised: 1. visual...

Currently, there is no mechanism for service validation of diagnostic ultrasonography (US) for giant cell arteritis (GCA). Temporal artery biopsy (TAB) and classification criteria are poor benchmarks. We validated our service against physician-verified diagnosis at 100 weeks (100wD). Twenty-five patients underwent US within 7 days, and TAB within 2...

A 25-year-old woman presented with ophthalmic and neurological manifestations. Her ocular manifestations included bilateral uveitis, multifocal retinal phlebitis, vitreitis and multiple retinal haemorrhages. Her neurological manifestations included migrainous headaches with visual aura, transient sensory symptoms and posterior circulation Transient...