Sarah L Mackie

• BA(Hons) BMBCh MRCP PhD
• Professor (Associate) at University of Leeds

Objectives The Steroid PRO is a treatment-specific patient-reported outcome questionnaire which measures the impact of glucocorticoids on health-related quality of life. It has 15 items grouped into 4 domains (Social impact, Impact on Appearance, Psychological Impact and Treatment Concerns). Initially developed and validated in rheumatic diseases,...

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 This project aimed to determine whether cranial ischaemic complications at the presentation of giant cell arteritis (GCA) were associated with pre-existing cardiovascular (CV) risk factors, CV disease or genetic risk of CV-related traits. Methods 1946 GCA patients with clinicodemographic data at GCA presentation were included. Associati...

Objectives Giant cell arteritis (GCA) may be confirmed by temporal artery biopsy (TAB) but false negatives can occur. GCA may be overdiagnosed in TAB-negative cases, or if neither TAB nor imaging is done. We used Human Leucocyte Antigen (HLA) genetic association of TAB-positive GCA as an “unbiased umpire” test to estimate historic overdiagnosis of...

OBJECTIVE To perform a systematic literature review on definitions and instruments used to measure remission, relapse, and disease activity in polymyalgia rheumatica (PMR), to inform an OMERACT project to endorse instruments for these outcomes. METHODS A search of Pubmed/MEDLINE, EMBASE, CINAHL, Cochrane, and Epistemonikos was performed May 2021 a...

Treatment of giant cell arteritis (GCA) aims initially to prevent acute visual loss, and subsequently to optimise long-term quality of life. Initial prevention of acute visual loss in GCA is well-standardised with high-dose glucocorticoid therapy but in the longer term optimising quality of life requires tailoring of treatment to the individual. Th...

Background The lack of standardized definitions for clinical manifestations of giant cell arteritis (GCA) leads to variable interpretations in clinical practice and research settings. An international task force developing response criteria for GCA filled this gap by developing a glossary for signs and symptoms of GCA. Objectives To create a conse...

Background Giant Cell Arteritis (GCA) is a large vessel vasculitis associated with severe morbidity and requiring prompt assessment and treatment. The National Early Inflammatory Arthritis Audit (NEIAA) is a mandated audit of rheumatology care in England and Wales. In April 2023 GCA was added to the list of conditions included in NEIAA to character...

Background Glucocorticoids (GCs) are important for the management of systemic lupus erythematosus (SLE); however, chronic use is associated with significant adverse effects and organ damage. Despite the general recommendation to taper GCs in order to minimise organ damage accrual, detailed guidance on how to taper is lacking. Objectives We outline...

Objective Polymyalgia rheumatica (PMR) is an age-related inflammatory disease of unknown cause. We aimed to identify potentially modifiable risk factors and therapeutic targets for preventing or treating PMR. Methods We meta-analysed genetic association data from 8,156 cases of PMR (defined using diagnostic codes and self-report) and 416,495 contro...

Background Giant cell arteritis is an age-related vasculitis that mainly affects the aorta and its branches in individuals aged 50 years and older. Current options for diagnosis and treatment are scarce, highlighting the need to better understand its underlying pathogenesis. Genome-wide association studies (GWAS) have emerged as a powerful tool for...

Background/Aims Glucocorticoids (GCs) used in the treatment of inflammatory rheumatic conditions can impact on patients in different ways. The objective of this study was to identify socio-demographic and clinical factors associated with health-related quality of life (HRQoL) in patients undertaking GCs for a rheumatic disease. Methods This was a...

Background/Aims People with PMR report experiencing pain, stiffness and weakness, often impacting everyday tasks. Many fear exercise will exacerbate symptoms and need reassurance that exercise is helpful and not harmful. However, no PMR-specific, exercise educational resource currently exists. The aim of this funded study was to co-create an exerci...

Background/Aims To explore patients’ perspectives on the impact of appearance changes attributed to glucocorticoid treatment. Methods We conducted a secondary analysis of semi-structured interviews with patients with rheumatic conditions receiving glucocorticoids in the UK, USA, and Australia. Interview data were analysed inductively to develop pr...

Objective: Polymyalgia rheumatica (PMR) is an age-related inflammatory disease of unknown cause. We aimed to identify potentially modifiable risk factors and therapeutic targets for preventing or treating PMR. Methods: We meta-analysed genetic association data from 8,156 cases of PMR (defined using diagnostic codes and self-report) and 416,495 cont...

The last British Society for Rheumatology (BSR) guideline on PMR was published in 2009. The guideline needs to be updated to provide a summary of the current evidence for pharmacological and non-pharmacological management of adults with PMR. This guideline is aimed at healthcare professionals in the UK who directly care for people with PMR, includi...

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 The National Health Service in England funds 12 months of weekly subcutaneous tocilizumab (qwTCZ) for patients with relapsing or refractory giant cell arteritis (GCA). During the COVID-19 pandemic, some patients were allowed longer treatment. We sought to describe what happened to patients after cessation of qwTCZ. Methods Multicentre s...

Objectives Glucocorticoids used in the treatment of inflammatory rheumatic conditions can impact on health-related quality of life. An underpinning qualitative study developed a long-list of candidate items for a treatment-specific patient-reported outcome (PRO) measure. The objective of this paper is to determine scale structure and psychometric p...

Objective The aim was to determine prevalent co-morbidities in cases with PMR or GCA compared with matched controls. Methods This was a nested, cross-sectional case–control study within the UK Biobank, which recruited participants aged 40–69 years. Case status was defined as self-reported prior diagnosis of PMR or GCA. Ten controls per case were m...

Objectives Extracellular vesicles (EVs) are abundant in body fluids, contributing to intercellular signalling by transferring cargo that includes microRNAs (miRs)—themselves implicated in pathobiology. For the first time we evaluated the potential of EV miRs to contribute diagnostic information in early RA, predict methotrexate (MTX) efficacy or sh...

Objective Jaw symptoms can be a vital clue to the diagnosis of GCA. Guidelines recommend more intensive treatment if jaw claudication is present. We sought to explore how patients with GCA described their jaw symptoms. Methods We carried out a secondary, qualitative analysis of interview data from 36 participants from the UK (n = 25) and Australia...

Background Giant cell arteritis (GCA) is primarily treated with glucocorticoids (GCs), which have substantial toxicity. Tocilizumab, an interleukin-6-receptor inhibitor (IL-6Ri), showed beneficial effects in GCA, leading to its approval. This study investigated the efficacy and safety of sarilumab (another IL-6Ri) in GCA. Methods This Phase 3, dou...

Introduction A 67-year-old female, diagnosed with giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) in 2016, had remained on high doses of prednisolone since her relapse in 2020. Critical evaluation of her symptoms was needed to understand that her generalised pain was likely driven by muscle weakness, rather than PMR. Clear and honest co...

Objectives To update the evidence on imaging for diagnosis, monitoring and outcome prediction in large vessel vasculitis (LVV) to inform the 2023 update of the European Alliance of Associations for Rheumatology recommendations on imaging in LVV. Methods Systematic literature review (SLR) (2017–2022) including prospective cohort and cross-sectional...

Dear Editor, PMR is a symmetrical, glucocorticoid-sensitive, inflammatory disorder of extracapsular structures [1]. PMR affects chronically mechanically stressed fibrocartilage-containing structures such as sternoclavicular joints, pubic symphysis, entheses and interspinous ligaments. IL-1 generated by activation of the NLRP3 inflammasome, from aut...

Objectives To update the EULAR recommendations for the use of imaging modalities in primary large vessel vasculitis (LVV). Methods A systematic literature review update was performed to retrieve new evidence on ultrasound, MRI, CT and [ ¹⁸ F]-fluorodeoxyglucose positron emission tomography (FDG-PET) for diagnosis, monitoring and outcome prediction...

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...

Objective: To examine the test-retest reliability of four measurement instruments in polymyalgia rheumatica (PMR): pain severity visual analogue scale (VAS) / numerical rating score (NRS), stiffness severity VAS/NRS, the Health Assessment Questionnaire-Disability Index (HAQ-DI) and the modified Health Assessment Questionnaire (mHAQ). Method: Two...

The temporal artery biopsy (TAB) is regarded as the gold-standard test in the diagnosis of giant cell arteritis (GCA). There is a lack of agreement among experienced pathologists regarding the diagnostic features and classification of inflammation observed in TAB sections in the diagnosis of GCA. Aims The aim of this research study was to establis...

Imaging is increasingly being used to guide clinical decision-making in patients with giant cell arteritis (GCA). While ultrasound has been rapidly adopted in fast-track clinics worldwide as an alternative to temporal artery biopsy for the diagnosis of cranial disease, whole-body PET/CT is emerging as a potential gold standard test for establishing...

Background Giant cell arteritis (GCA) is a chronic large-vessel vasculitis that affects mainly the aorta and its primary branches, and in Western countries is the most common form of vasculitis in people older than 50 years. The most severe manifestations of GCA include blindness and ischemic stroke caused by occlusion of affected arteries. GCA pre...

Background Imaging recommendations for primary large vessel vasculitis (LVV) were developed in 2018. Several new studies have emerged since then, and an update of the original statements was required. Objectives To update the recommendations for the use of imaging in LVV. Methods A systematic literature review update was performed to retrieve new...

Background Since the development of the EULAR recommendations for the use of imaging in large vessel vasculitis (LVV) in 2017, new data has emerged in the field of imaging techniques and their application in the diagnosis and follow-up of patients with giant cell arteritis (GCA) and Takayasu arteritis (TAK). Objectives To summarize the evidence on...

Background Glucocorticoids are used in the treatment of a broad range of rheumatic conditions but can have a significant impact on health-related quality of life[1]. In-depth qualitative interviews with patients who have experience of glucocorticoid treatment for their rheumatic disease, from the UK, USA and Australia, were used to develop candidat...

Objective. To determine prevalent comorbidities in cases with polymyalgia rheumatica (PMR) or giant cell arteritis (GCA) compared to matched controls. Methods. Nested, cross sectional case-control study within UK Biobank. Case status was defined as self-reported prior diagnosis of PMR or GCA. 10 controls per case were matched for age, sex, ethnicit...

Objectives: Giant cell arteritis (GCA) is systemic vasculitis manifesting as cranial, ocular, or large vessel vasculitis. A prior qualitative study developed 40 candidate items to assess the impact of GCA on health-related quality of life (HRQoL). This study aimed to determine final scale structure and measurement properties of the GCA patient rep...

Objectives To determine whether the halo count (HC) on temporal and axillary artery ultrasound (TAUS) predicts time to relapse in giant cell arteritis (GCA). Methods We conducted a single-centre retrospective study of GCA patients. HC, the number of vessels with non-compressible halo on the TAUS at diagnosis, was determined by retrospective review...

Background/Aims Recognition of jaw claudication is crucial to expedite diagnosis and treatment of giant cell arteritis (GCA). The ACR 1990 criteria defined claudication of the jaw, tongue or deglutition as “development or worsening of fatigue or discomfort in muscles of mastication, tongue, or swallowing muscles while eating”. We sought to describe...

Background/Aims Early diagnosis and prompt and effective intervention for people with immune-mediated inflammatory diseases (IMIDs) such as rheumatoid arthritis (RA) improves outcomes. Non-invasive biomarkers that reflect unique aspects of underlying biology could facilitate this. Extracellular vesicles (EVs) released by many cell types are abundan...

Objectives: Prompt diagnosis of giant cell arteritis (GCA) is important to avert visual loss. False-negative temporal artery biopsy (TAB) can occur. Without vascular imaging, GCA may be overdiagnosed in TAB-negative cases, but it is unclear how often this occurs. An unbiased test is a way to address an imperfect reference standard. We used the know...

Background/Aims Glucocorticoid toxicities are well-known, but patient involvement work (PMR Voices 2021) had highlighted a need for better patient education/counselling about side-effects, including early neuropsychiatric toxicity, and support for self-management. In our audit of steroid-induced hyperglycaemia, our attempts to improve counselling h...

Background/Aims Giant cell arteritis (GCA) is considered a medical emergency, and relapses are common, affecting around 50% of patients. Many of the early symptoms of GCA are non-specific and highly prevalent in older people, making the diagnosis challenging. The Clinical Practice Research Datalink (CPRD) is a large primary care database that provi...

Background/Aims To determine the diagnostic test accuracy of compression ultrasound compared to combined compression and halo techniques in the diagnosis of giant cell arteritis. Methods A retrospective secondary data analysis was conducted of 111 consecutive patients referred with suspected giant cell arteritis (GCA). All patients had an ultrasou...

Background/Aims Systemic glucocorticoids are key in the management of life- and organ-threatening rheumatic diseases, but have wide-ranging adverse effects of concern to patients and clinicians. We aimed to validate a Patient Reported Outcome (PRO) measure (the Steroid PRO) for assessing the impact of glucocorticoids on health-related quality of li...

Background: High myopia (HM), defined as a spherical equivalent refractive error (SER) ≤ -6.00 diopters (D), is a leading cause of sight impairment, through myopic macular degeneration (MMD). We aimed to derive an improved polygenic score (PGS) for predicting children at risk of HM and to test if a PGS is predictive of MMD after accounting for SER...

Objectives: Glucocorticoids (GCs) ('steroids') are used to treat rheumatic diseases but adverse effects are common. We aimed to explore the impact of GC therapy on health-related quality of life (HRQoL), to inform the development of a treatment-specific patient-reported outcome measure (PROM) for use in clinical trials and practice. Methods: Sem...

The aim of this study was to develop and validate an automated pipeline that could assist the diagnosis of active aortitis using radiomic imaging biomarkers derived from [18F]-Fluorodeoxyglucose Positron Emission Tomography-Computed Tomography (FDG PET-CT) images. The aorta was automatically segmented by convolutional neural network (CNN) on FDG PE...

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 explore current management practices for polymyalgia rheumatica (PMR) by general practitioners (GPs) and rheumatologists including implications for clinical trial recruitment. Methods: An English language questionnaire was constructed by a working group of rheumatologists and GPs from 6 countries. The questionnaire focused on: 1:...

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...

Background/objectives This study aims to describe the grading methods and baseline characteristics for UK Biobank (UKBB) participants who underwent retinal imaging in 2009–2010, and to characterise individuals with retinal features suggestive of age-related macular degeneration (AMD), glaucoma and retinopathy. Methods Non-mydriatic colour fundus p...

Objectives: Angiotensin II is implicated in giant cell arteritis (GCA) pathology. We examined whether the use of angiotensin receptor blockers (ARB) is associated with GCA risk, compared with ACE inhibitors (ACEi) or other antihypertensives. Methods: We performed a matched cohort study including adults who were initiators of antihypertensives in...

Background In most countries polymyalgia rheumatica (PMR) is diagnosed and managed by both general practitioners (GP) and rheumatologists. However, the referral pattern from GP’s to specialist around the world has not been described. The initial prednisolone dose recommended by EULAR/ACR is between 12.5 and 25 mg¹, but little is known about whether...

Background About half of patients with giant cell arteritis (GCA) relapse while tapering glucocorticoid therapy ¹ . A previous observational study reported that blockade of the renin-angiotensin system, with angiotensin II receptor blockers (ARBs), was associated with lower relapse risk ² . Objectives To determine whether angiotensin blockade, wit...

Background Polymyalgia rheumatica (PMR) is diagnosed and treated by both general practitioners (GP) and rheumatologists. How rheumatologists around the world manage the referral process of patients with PMR from GP’s has not been described. EULAR/ACR guidelines recommend initial prednisolone doses between 12.5 and 25 mg, but it is unknown if guidel...

Background Giant cell arteritis (GCA) is caused by inflammation of the blood vessels of the head and neck; patients can present with cranial, ocular or large vessel vasculitis involvement. Treatment is with glucocorticoids, steroid sparing agents and biologics to control inflammation and protect sight. Objectives The aim of this study was to produ...

Background Glucocorticoids (GCs) are a key treatment for inflammatory rheumatic diseases, but they cause a wide range of adverse side-effects which are of concern both to patients and clinicians. Objectives The objective of this study was to explore the impact of GC therapy on health-related quality of life (HRQoL) during treatment for rheumatic d...

Background/Aims Glucocorticoids (GCs) are widely used to treat inflammatory rheumatic diseases. GCs carry a range of adverse effects of concern to patients and clinicians. The objective of this study was to explore the impact of GC therapy on health-related quality of life (HRQoL) during treatment for rheumatic diseases, as a basis for development...

Background/Aims In patients starting steroids for giant cell arteritis (GCA), to evaluate adherence to BSR guideline: HbA1c monitoring, documentation of counselling, and detection of steroid-induced hyperglycaemia. Methods Data were gathered from the electronic medical records. Steroid-induced hyperglycaemia was defined as new HbA1c >48 measured 3...

Background/Aims Giant cell arteritis (GCA) presents in people over the age of 50 with cranial, ocular, and large vessel vasculitis. This study aims to validate a disease-specific patient-reported outcome measure (PROM) for patients with GCA, to capture the impact of GCA and its treatment on health-related quality of life. Methods This cross-sectio...

Background/Aims Giant cell arteritis (GCA) presents in people over 50, with headaches, visual involvement and large vessel vasculitis. A 30-item GCA-specific patient reported outcome measure (GCA PRO) was developed and tested in a clinical setting to pilot its feasibility and acceptability to patients and clinicians as a communication tool. Method...

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...

Background/Aims In our trust, patients with suspected giant cell arteritis (GCA) were promptly started on high-dose prednisolone. However, until 2019, patients were waiting on average 28.5 days (median) for confirmation of diagnostic decision, which was mostly due to the time taken for temporal artery biopsy (TAB) to be performed and reported. We h...

The Delphi method is a well‐established research tool, used for consensus building across a number of fields. Despite its widespread use, and popularity in many medical specialities, there is a paucity of literature on the use of the Delphi method in Histopathology. This literature review seeks to critique the Delphi methodology and explore its pot...

Importance: Understanding the effects of modifiable risk factors on risk for multiple sclerosis (MS) and associated neurodegeneration is important to guide clinical counseling. Objective: To investigate associations of alcohol use, smoking, and obesity with odds of MS diagnosis and macular ganglion cell layer and inner plexiform layer (mGCIPL) t...

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 The aim of this study was to explore the feasibility of assisted diagnosis of active (peri-)aortitis using radiomic imaging biomarkers derived from [ ¹⁸ F]-Fluorodeoxyglucose Positron Emission Tomography–Computed Tomography (FDG PET–CT) images. Methods The aorta was manually segmented on FDG PET–CT in 50 patients with aortitis and 25 co...

Objective To explore patient perceptions of physical activity in giant cell arteritis (GCA). Methods This was a multinational qualitative study, analyzing interview data collected from participants from the UK (n = 25) and Australia (n = 11) with a definitive diagnosis of GCA from imaging or biopsy. Interview transcripts were analyzed using themat...

Somatic mutations in the gene encoding the major E1 ubiquitin ligase, UBA1, were recently identified as a cause of VEXAS, a late-onset acquired auto-inflammatory syndrome. Differential diagnoses for patients subsequently found to have VEXAS include relapsing polychondritis, Sweet’s syndrome, myelodysplastic syndrome (MDS), giant cell arteritis (GCA...

An amendment to this paper has been published and can be accessed via the original article.

BACKGROUND: Patients with primary systemic vasculitis or polymyalgia rheumatica might be at a high risk for poor COVID-19 outcomes due to the treatments used, the potential organ damage cause by primary systemic vasculitis, and the demographic factors associated with these conditions. We therefore aimed to investigate factors associated with COVID-...

Background Acute kidney injury (AKI) is common among patients hospitalised with COVID-19 and associated with worse prognosis. The aim of this study was to investigate the epidemiology, risk factors and outcomes of AKI in patients with COVID-19 in a large UK tertiary centre. Methods We analysed data of consecutive adults admitted with a laboratory-...

Background Patients with primary systemic vasculitis or polymyalgia rheumatica might be at a high risk for poor COVID-19 outcomes due to the treatments used, the potential organ damage cause by primary systemic vasculitis, and the demographic factors associated with these conditions. We therefore aimed to investigate factors associated with COVID-1...

We thank Sauret et al ¹ for their interest in our systematic literature review that explored potential diagnostic confusion between giant cell arteritis (GCA) and the coronavirus disease 2019 (COVID-19). This was a particularly important consideration during the early months of the COVID-19 pandemic, when community testing for SARS-CoV-2 was limite...