Rheumatology




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First clinical practice guideline for renal hypouricaemia: a rare disorder that aided the development of urate-lowering drugs for gout 

Akiyoshi Nakayama, Hirotaka Matsuo, Abhishek Abhishek, Kimiyoshi Ichida, Nariyoshi Shinomiya, for the members of Guideline Development Committee of Clinical Practice Guideline for Renal Hypouricaemia

doi : 10.1093/rheumatology/keab322

Rheumatology, Volume 60, Issue 9, September 2021, Pages 3961–3963

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Furthering the links between osteoarthritis and other health conditions 

Gillian A Hawker, Lauren K King

doi : 10.1093/rheumatology/keab376

Rheumatology, Volume 60, Issue 9, September 2021, Pages 3964–3965

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Osteoarthritis and associated comorbidities: new answers and more questions 

Fiona E Watt, Elspeth M Wise

doi : 10.1093/rheumatology/keab405

Rheumatology, Volume 60, Issue 9, September 2021, Pages 3966–3968

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Infections in immunosuppressed travellers with autoimmune inflammatory diseases—a narrative review and advice for clinical practice 

Victoria Allen, Nicky Longley

doi : 10.1093/rheumatology/keab445

Rheumatology, Volume 60, Issue 9, September 2021, Pages 3969–3976

The management of autoimmune, inflammatory diseases has been revolutionized by biologic therapies. A beneficial consequence of better disease control is that more patients are well enough to travel the world. There is now a class of traveller, the significantly immunosuppressed person with autoimmune disease, with specific risks and requirements. This review introduces the concept of the pre-travel risk assessment and discusses the major vaccine-preventable and non-vaccine-preventable travel-associated infections. The challenges and controversies around vaccination and immunosuppression are reviewed with advice for clinical practice.

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Precision medicine in autoimmune diseases: fact or fiction

Daniel Toro-Domínguez, Marta E Alarcón-Riquelme

doi : 10.1093/rheumatology/keab448

Rheumatology, Volume 60, Issue 9, September 2021, Pages 3977–3985

Much is said about precision medicine, but its real significance and potential are far from certain. Several studies in each of the autoimmune diseases have provided important insights into molecular pathways, but the use of molecular studies, particularly those looking into transcriptome pathways, has seldom approached the possibility of using the data for disease stratification and then for prediction, or for diagnosis. Only the type I IFN signature has been considered for therapeutic purposes, particularly in the case of SLE. This review provides an update on precision medicine, on what can be translated into clinical practice and on what single-cell molecular studies contribute to our knowledge of autoimmune diseases, focusing on a few examples. The main message is that we should try to move from precision medicine of established diseases to preventive medicine in order to predict the development of disease.

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Current and emerging biological therapy in adult-onset Still’s disease 

Yuning Ma, Jianfen Meng, Jinchao Jia, Mengyan Wang, Jialin Teng, Dehao Zhu, Chengde Yang, Qiongyi Hu

doi : 10.1093/rheumatology/keab485

Rheumatology, Volume 60, Issue 9, September 2021, Pages 3986–4000

Adult-onset Still’s disease (AOSD) is a rare, but characteristic non-familial, multi-genic systemic auto-inflammatory disorder, characterized by high spiking fever, salmon-like evanescent skin rash, polyarthritis, sore throat, hyperferritinemia and leucocytosis. The hallmark of AOSD is a cytokine storm triggered by dysregulation of inflammation. Nowadays, with advances in anti-cytokine biologic agents, the treatment of AOSD is no longer limited to NSAIDs, glucocorticoids or conventional synthetic DMARDs. In this review, we focussed on the roles of these cytokines in the pathogenesis of AOSD and summarized the current and emerging biological therapy.

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Prevalence and predictors of adverse events with methotrexate mono- and combination-therapy for rheumatoid arthritis: a systematic review

Ahmad A Sherbini, Seema D Sharma, James M Gwinnutt, Kimme L Hyrich, Suzanne M M Verstappen

doi : 10.1093/rheumatology/keab304

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4001–4017

This systematic review aims to summarize rates of adverse events (AEs) in patients with RA or inflammatory arthritis starting MTX as monotherapy or in combination with other csDMARDs, and to identify reported predictors of AEs.

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Maternal and neonatal outcomes in pregnant women with psoriasis and psoriatic arthritis: a systematic review and meta-analysis

Wenhui Xie, Hong Huang, Lanlan Ji, Zhuoli Zhang

doi : 10.1093/rheumatology/keab357

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4018–4028

Psoriasis and PsA are inflammatory diseases that affect women in their reproductive years. We aimed to investigate whether maternal psoriasis and PsA are associated with adverse pregnancy outcomes.

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Mortality in patients with primary Sjögren’s syndrome: a systematic review and meta-analysis

Hong Huang, Wenhui Xie, Yan Geng, Yong Fan, Zhuoli Zhang

doi : 10.1093/rheumatology/keab364

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4029–4038

It remains debated whether patients with primary Sjögren’s syndrome (pSS) are at greater risk of mortality. We aimed to determine the magnitude of all-cause mortality risk in patients with pSS compared with the general population through a systematic review and meta-analysis.

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Therapeutic options for cutaneous polyarteritis nodosa: a systematic review

Eleni Papachristodoulou, Loukas Kakoullis, Eleni Tiniakou, Konstantinos Parperis

doi : 10.1093/rheumatology/keab402

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4039–4047

Cutaneous polyarteritis nodosa (CPAN) is a necrotizing vasculitis of the middle-size vessels, confined to the skin. We conducted a systematic review in order to identify studies evaluating the different treatment modalities used in CPAN.

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Real-world single centre use of JAK inhibitors across the rheumatoid arthritis pathway 

John Fitton, Andrew R Melville, Paul Emery, Jacqueline L Nam, Maya H Buch

doi : 10.1093/rheumatology/keaa858

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4048–4054

To evaluate real-world efficacy of approved JAK inhibitors (JAKi) tofacitinib and baricitinib in a large, single-centre cohort of RA patients across the treatment pathway, including those refractory to multiple biologic drugs.

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Higher prevalence of non-skeletal comorbidity related to X-linked hypophosphataemia: a UK parallel cohort study using CPRD

Samuel Hawley, Nick J Shaw, Antonella Delmestri, Daniel Prieto-Alhambra, Cyrus Cooper, Rafael Pinedo-Villanueva, M Kassim Javaid

doi : 10.1093/rheumatology/keaa859

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4055–4062

X-Linked hypophosphataemic rickets (XLH) is a rare multi-systemic disease of mineral homeostasis that has a prominent skeletal phenotype. The aim of this study was to describe additional comorbidities in XLH patients compared with general population controls.

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Comparative effectiveness and persistence of TNFi and non-TNFi in juvenile idiopathic arthritis: a large paediatric rheumatology centre in the USA

Xiaomeng Yue, Bin Huang, Ana L Hincapie, Patricia R Wigle, Yuxiang Li, Tingting Qiu, Daniel J Lovell, Esi M Morgan, Jeff J Guo

doi : 10.1093/rheumatology/keaa877

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4063–4073

To evaluate the persistence and effectiveness of TNF inhibitors (TNFi) vs non-TNFi among newly diagnosed JIA patients after initiation of biologic DMARD (bDMARD).

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Anti-NuMA antibodies: clinical associations and significance in patients with primary Sjögren’s syndrome or systemic lupus erythematosus

Robin Arcani, Daniel Bertin, Nathalie Bardin, Karin Mazodier, Rodolphe Jean, Pierre Suchon, Geoffroy Venton, Aurélie Daumas, Estelle Jean, Patrick Villani, Gilles Kaplanski, Pierre-André Jarrot

doi : 10.1093/rheumatology/keaa881

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4074–4084

To determine the clinical significance of anti-nuclear mitotic apparatus (NuMA) antibodies (AC-26 or AC-25) in patients with primary Sjögren’s syndrome (pSS) and SLE.

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Anti-CD74 IgA autoantibodies in radiographic axial spondyloarthritis: a longitudinal Swedish study 

Lan Do, Gabriel Granåsen, Urban Hellman, Kristina Lejon, Mats Geijer, Xenofon Baraliakos, Torsten Witte, Helena Forsblad-d’Elia

doi : 10.1093/rheumatology/keaa882

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4085–4093

Antibodies against anti-CD74 are related to axial spondyloarthritis (axSpA). The objectives were (i) to study IgA anti-CD74 in radiographic (r)-axSpA patients in the Backbone cohort and to calculate the sensitivity and specificity of anti-CD74, (ii) to study the fluctuation of IgA anti-CD74 levels in prospectively collected samples, and (iii) to explore the relation between IgA anti-CD74 and radiographic spinal changes.

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Base of thumb osteoarthritis in UK interface services—a cohort and survey-based study to assess current practice 

Benjamin J F Dean, Stefan Kluzek, Andrew J Carr, Sally Hopewell, Duncan Richards, Nicholas Riley, Andrew Cuff, Oxford Base of Thumb Collaborative

doi : 10.1093/rheumatology/keaa884

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4094–4102

Base of thumb OA (BTOA) is a common age-related disease that has a significant negative impact on quality of life, while little is known about the structure and pathways of interface services. Our aim was to assess disease burden, referral pathways, service structure and management pathways in UK interface services.

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Sensitivity to change and clinical correlations of the novel DACtylitis glObal Sonographic (DACTOS) score in psoriatic arthritis

Nicolò Girolimetto, Alen Zabotti, Ilaria Tinazzi, Niccolò Possemato, Luisa Costa, Alberto Batticciotto, Marco Canzoni, Giorgia Citriniti, Orazio De Lucia, Fabiana Figus, Luca Idolazzi, Rebecca McConnel, Rosario Peluso, Garifallia Sakellariou, Annarita Tullio, Carlo Salvarani, Raffaele Scarpa, Annamaria Iagnocco, Francesco Caso, Pierluigi Macchioni

doi : 10.1093/rheumatology/keaa885

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4103–4111

The aim of the study is to assess the performance of the DACTOS (DACtylitis glObal Sonographic) score in a PsA dactylitis clinical setting. In particular, we evaluated the ability of DACTOS to identify the affected fingers, its sensitivity to change after treatment, the correlations between DACTOS and clinical parameters, and the capacity of the score to identify the treatment responders.

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Increased proportion of comorbidities but no deterioration of sexual quality of life during a 5-year follow-up in patients with axial spondyloarthritis in the biologic treatment era

Kari Hansen Berg, Gudrun Rohde, Are Pripp, Anne Prøven, Esben Ester Pirelli Benestad, Monika Østensen, Glenn Haugeberg

doi : 10.1093/rheumatology/keaa887

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4112–4120

To explore patient perception of sexual quality of life (SQOL), an important category of QOL, in male and female patients with axial SpA (axSpA) after a 5?year follow-up.

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The changing states of fibromyalgia in patients with axial spondyloarthritis: results from the British Society of Rheumatology Biologics Register for Ankylosing Spondylitis 

Sella A Provan, Linda E Dean, Gareth T Jones, Gary J Macfarlane

doi : 10.1093/rheumatology/keaa888

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4121–4129

To identify factors associated with FM development and recovery in patients with axial SpA (axSpA).

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Management of fatigue with physical activity and behavioural change support in vasculitis: a feasibility study

Lorraine Harper, Catherine A Hewitt, Ian Litchfield, Matthew D Morgan, Dimitrios Chanouzas, Hollie K Caulfield, Linda Coughlan, Caroline Dean, Kate Fletcher, Fiona Cramp, Sheila Greenfield, Natalie J Ives, Sue Jowett, Shalela Kodabuckus, Sarah Tearne, Sukhwant Sehmi, Charlotte Edwardson, Nathan P Dawkins, Amanda J Daley

doi : 10.1093/rheumatology/keaa890

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4130–4140

Patients with ANCA-associated vasculitis (AAV) experience high levels of fatigue, despite disease remission. This study assessed the feasibility and acceptability of a definitive randomized controlled trial of a behavioural-based physical activity intervention to support fatigue self-management in AAV patients.

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Changes in the pharmacological management of rheumatoid arthritis over two decades 

Samantha S R Crossfield, Maya H Buch, Paul Baxter, Sarah R Kingsbury, Mar Pujades-Rodriguez, Philip G Conaghan

doi : 10.1093/rheumatology/keaa892

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4141–4151

To assess whether modern management of RA has reduced the prescription of oral corticosteroids and NSAIDs and to evaluate use of pharmacological prophylaxis strategies.

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Adult-onset Still’s disease with neurological involvement: a single-centre report

Mengzhu Zhao, Di Wu, Min Shen

doi : 10.1093/rheumatology/keaa899

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4152–4157

Adult-onset Still’s disease (AOSD) is a multifactorial systemic autoinflammatory disease. Neurological damage has been rarely reported in AOSD. We aimed to characterize the clinical features of AOSD patients with neurological involvement.

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Comorbidity and response to TNF inhibitors in axial spondyloarthritis: longitudinal analysis of the BSRBR-AS 

Sizheng Steven Zhao, Gareth T Jones, Gary J Macfarlane, David M Hughes, Robert J Moots, Nicola J Goodson

doi : 10.1093/rheumatology/keaa900

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4158–4165

Comorbidities influence disease assessment in axial spondyloarthritis (axSpA), but their association with response to TNF inhibitors (TNFi) is unclear. We examined associations between comorbidity history at TNFi initiation and: (i) change in disease indices over time; (ii) binary response definitions; and (iii) time to treatment discontinuation.

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Demyelination with autoimmune features: a distinct clinical entity? Results from a longitudinal cohort

Dionysis Nikolopoulos, Dimitris Kitsos, Matilda Papathanasiou, Maria Chondrogianni, Aikaterini Theodorou, Panagiotis Garantziotis, Antigone Pieta, Triantafyllos Doskas, George Bertsias, Konstantinos Voumvourakis, Dimitrios T Boumpas, Antonis Fanouriakis

doi : 10.1093/rheumatology/keaa902

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4166–4174

CNS demyelinating syndromes occurring in the context of SLE may represent a manifestation of neuropsychiatric lupus, or an overlap of SLE and multiple sclerosis (MS). We evaluated prospectively patients presenting with demyelinating syndrome for clinical and serological evidence of SLE and characterized the evolution of their clinical syndrome to a defined disease.

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The cost-effectiveness of different approaches to exercise and corticosteroid injection for subacromial pain (impingement) syndrome

Raymond Oppong, Sue Jowett, Martyn Lewis, Edward Roddy, Reuben O Ogollah, Irena Zwierska, Praveen Datta, Alison Hall, Elaine Hay, Julie Shufflebotham, Kay Stevenson, Danielle A van der Windt, Julie Young, Nadine E Foster

doi : 10.1093/rheumatology/keaa903

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4175–4184

To determine whether physiotherapist-led exercise intervention and US-guided subacromial CS injection is cost-effective when compared with standard advice and exercise leaflet and unguided injection in patients with subacromial pain (impingement) syndrome.

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Total plaque area and plaque echogenicity are novel measures of subclinical atherosclerosis in patients with systemic lupus erythematosus

Sara C Croca, Maura Griffin, Filipa Farinha, David A Isenberg, Andrew Nicolaides, Anisur Rahman

doi : 10.1093/rheumatology/keaa905

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4185–4198

Patients with SLE have an increased risk of developing cardiovascular disease (CVD). Multiple studies have shown that these patients have increased numbers of carotid plaques and greater intima-media thickness (IMT) than healthy controls. Measures such as total plaque area (TPA) and plaque echogenicity may be more sensitive and more relevant to cardiovascular risk than presence of plaque and IMT alone. Our objective was to produce the first report of TPA and echogenicity in a population of patients with SLE.

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Initiation of febuxostat for acute gout flare does not prolong the current episode: a randomized clinical trial 

Ertao Jia, Yanying Zhang, Wukai Ma, Bo Li, Hongling Geng, Li Zhong, Xueming Yao, Jingjing Xie, Yuya Xiao, Yubao Jiang, Xia Qiu, Min Xiao, Xiang Cui, Jiaxin Wei, Jianyong Zhang

doi : 10.1093/rheumatology/keaa908

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4199–4204

Our objective was to determine whether initiation of febuxostat during an acute gout flare prolongs the current episode.

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Contribution of abnormal BMI to adverse health-related quality of life outcomes after a 52-week therapy in patients with SLE 

Alexander Borg, Alvaro Gomez, Arvid Cederlund, Flordelyn Cobar, Victor Qiu, Julius Lindblom, Sharzad Emamikia, Yvonne Enman, Susanne Pettersson, Ioannis Parodis

doi : 10.1093/rheumatology/keaa909

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4205–4217

To investigate whether abnormal BMI is associated with adverse health-related quality of life (HRQoL) outcome, including severe fatigue, after 52?weeks of standard therapy plus belimumab or placebo in patients with SLE.

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Imbalanced MMP-3 and MMP-12 serum levels in systemic lupus erythematosus patients with Jaccoud’s arthropathy and a distinctive MRI pattern

Matteo Piga, Mattia Congia, Antonella Balestrieri, Maria Maddalena Angioni, Ignazio Cangemi, Riccardo Cau, Elisabetta Chessa, Alberto Floris, Fabiana Figus, Annamaria Iagnocco, Alberto Cauli, Luca Saba

doi : 10.1093/rheumatology/keaa915

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4218–4228

Metalloproteinase (MMP)-3 and MMP-12 are proteolytic enzymes especially implicated in joint inflammation. This study aims to evaluate their association with arthritis features and hand MRI abnormalities in patients with SLE.

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Contextualizing temporal arterial magnetic resonance angiography in the diagnosis of giant cell arteritis: a retrospective cohort study

Mats Junek, Angela Hu, Stephanie Garner, Ryan Rebello, Kim Legault, Karen Beattie, Nader Khalidi

doi : 10.1093/rheumatology/keaa916

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4229–4237

Imaging modalities have become common in evaluating patients for a possible diagnosis of GCA. This study seeks to contextualize how temporal arterial magnetic resonance angiography (TA-MRA) can be used in facilitating the diagnosis of GCA.

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Mortality in SLE patients compared with population controls in Finland in years 2000–2015

Pia Elfving, Simo Kariniemi, Hannu Kautiainen, Lauri J Virta, Oili Kaipiainen-Seppänen, Kari Puolakka

doi : 10.1093/rheumatology/keaa917

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4238–4244

To estimate the risk of mortality in the Finnish incident SLE cohort in a 16-year period compared with the general population.

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Impact of aging on phenotype and prognosis in IgA vasculitis

Alexandra Audemard-Verger, Evangéline Pillebout, Aurélie Baldolli, Noémie Le Gouellec, Jean-François Augusto, Noémie Jourde-Chiche, Loic Raffray, Eric Thervet, Alban Deroux, Julie Goutte, Aurélie Hummel, Bertrand Lioger, Sébastien Sanges, Patrice Cacoub, Zahir Amoura, Guillaume Moulis, Francois Maurier, Christian Lavigne, Geoffrey Urbanski, Johan Chanal, Stanislas Faguer, Sophie Deriaz, Nicole Feirreira-Maldent, Elisabeth Diot, Francois Maillot, Loïc Guillevin, Benjamin Terrier

doi : 10.1093/rheumatology/keaa921

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4245–4251

Immunoglobulin A vasculitis (IgAV) is a small-vessel vasculitis most frequently benign in children while more severe in adults. We aimed to study the impact of age on presentation and outcome of adult IgAV.

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Effectiveness and safety of treat-to-target strategy in elderly-onset rheumatoid arthritis: a 3-year prospective observational study

Takahiko Sugihara, Tatsuro Ishizaki, Wataru Onoguchi, Hiroyuki Baba, Takumi Matsumoto, Shoko Iga, Kanae Kubo, Mari Kamiya, Fumio Hirano, Tadashi Hosoya, Nobuyuki Miyasaka, Masayoshi Harigai

doi : 10.1093/rheumatology/keaa922

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4252–4261

To evaluate 3-year outcomes of following a treat-to-target (T2T) strategy targeting low disease activity for patients with elderly-onset RA (EORA) and to confirm safety profile of T2T.

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Low rate of subsequent surgery and serious complications following intra-articular steroid injection for base of thumb osteoarthritis: national cohort analysis 

Jennifer C E Lane, Richard S Craig, Jonathan L Rees, Matthew D Gardiner, Abigail V Shaw, Michelle Spiteri, Rachel Kuo, Benjamin F Dean, Jane Green, Daniel Prieto-Alhambra, Dominic Furniss

doi : 10.1093/rheumatology/keaa925

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4262–4271

Intra-articular steroid injection is commonly used to treat base of thumb osteoarthritis (BTOA), despite a lack of large-scale data on safety and effectiveness. We estimate the incidence of serious complications and further procedures following BTOA injection, including the risk of post-operative serious surgical site infection for subsequent operative intervention.

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Prevalence of gastrointestinal, cardiovascular, autonomic and allergic manifestations in hospitalized patients with Ehlers-Danlos syndrome: a case-control study

Rachel S Brooks, James Grady, Thomas W Lowder, Svetlana Blitshteyn

doi : 10.1093/rheumatology/keaa926

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4272–4280

Previous observations suggest an association between Ehlers-Danlos syndrome (EDS) and gastrointestinal (GI), cardiovascular, immune, and autonomic nervous system dysfunction. We sought to determine whether a hospital diagnosis of EDS is associated with a higher prevalence of these manifestations vs hospitalized patients without EDS. We also evaluated hospital outcomes.

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High early mortality in idiopathic inflammatory myopathies: results from the inception cohort at a tertiary care centre in northern India

Pankti Mehta, Vikas Agarwal, Latika Gupta

doi : 10.1093/rheumatology/keab001

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4281–4290

We determined the mortality along with the proportion of disease related adverse events measured individually and by a composite adverse outcome (devised by including deaths, disability, relapses and minimal response) and its predictors in an inception cohort of idiopathic inflammatory myopathies (IIM).

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Algorithm for calculating high disease activity in SLE

Alberta Hoi, Hieu T Nim, Rachel Koelmeyer, Ying Sun, Amy Kao, Oliver Gunther, Eric Morand

doi : 10.1093/rheumatology/keab003

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4291–4297

The ability to identify lupus patients in high disease activity status (HDAS) without knowledge of the SLEDAI could have application in selection of patients for treatment escalation or enrolment in trials. We sought to generate an algorithm that could calculate via model fitting the presence of HDAS using simple demographic and laboratory values.

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Remission in SLE—do DORIS criteria match the treating physician’s judgment? A cross-sectional study to assess reasons for discordance

Johanna Mucke, Christina Düsing, Nora Klose, Matthias Schneider, Gamal Chehab

doi : 10.1093/rheumatology/keab005

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4298–4305

The definition of an accurate target for a treat-to-target approach in SLE has been challenging over past years, and recently the DORIS definitions of remission were presented by the international DORIS task force. It was our aim to assess the frequency of DORIS remission and LLDAS in our SLE cohort and their agreement with the treating physician’s (DORIS-) independent remission judgement. Patient characteristics leading to lack of agreement and incoherence ought to be identified.

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Patients and clinicians define symptom levels and meaningful change for PROMIS pain interference and fatigue in RA using bookmarking

Clifton O Bingham, III, Alessandra L Butanis, Ana Maria Orbai, Michelle Jones, Victoria Ruffing, Anne Lyddiatt, Mary Suzanne Schrandt, Vivian P Bykerk, Karon F Cook, Susan J Bartlett

doi : 10.1093/rheumatology/keab014

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4306–4314

Using patient-reported outcomes to inform clinical decision-making depends on knowing how to interpret scores. Patient-Reported Outcome Measurement Information System® (PROMIS®) instruments are increasingly used in rheumatology research and care, but there is little information available to guide interpretation of scores. We sought to identify thresholds and meaningful change for PROMIS Pain Interference and Fatigue scores from the perspective of RA patients and clinicians.

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High burden of infections in Indian patients with Idiopathic Inflammatory Myopathy: validation of observations from the MyoCite dataset

Rudrarpan Chatterjee, Pankti Mehta, Vikas Agarwal, Latika Gupta

doi : 10.1093/rheumatology/keab015

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4315–4326

To determine the prevalence, profile and predictors of infections in an Indian cohort with idiopathic inflammatory myopathies (IIM).

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Temporal relationship between osteoarthritis and comorbidities: a combined case control and cohort study in the UK primary care setting 

Subhashisa Swain, Carol Coupland, Christian Mallen, Chang Fu Kuo, Aliya Sarmanova, Sita M A Bierma-Zeinstra, Martin Englund, Daniel Prieto-Alhambra, Michael Doherty, Weiya Zhang

doi : 10.1093/rheumatology/keab067

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4327–4339

To determine the burden of comorbidities in OA and their temporal relationships in the UK.

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Osteoarthritis and risk of hospitalization for ambulatory care-sensitive conditions: a general population-based cohort study 

Ali Kiadaliri, Martin Englund

doi : 10.1093/rheumatology/keab161

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4340–4347

To determine the association between OA and risk of hospitalization for ambulatory care-sensitive conditions (HACSCs).

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De novo lupus nephritis during treatment with belimumab 

Ioannis Parodis, Edward M Vital, Sabih-Ul Hassan, Andreas Jönsen, Anders A Bengtsson, Per Eriksson, Dag Leonard, Iva Gunnarsson, Lars Rönnblom, Christopher Sjöwall

doi : 10.1093/rheumatology/keaa796

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4348–4354

In light of reports of de novo LN during belimumab (BLM) treatment, we sought to determine its frequency and contributing or protective factors in a real-life setting.

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Significance of PR3-ANCA positivity in eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Matthias Papo, Renato A. Sinico, Vítor Teixeira, Nils Venhoff, Maria-Letizia Urban, Michele Iudici, Juliane Mahrhold, Francesco Locatelli, Giulia Cassone, Franco Schiavon, Benjamin Seeliger, Thomas Neumann, Claus Kroegel, Matthieu Groh, Chiara Marvisi, Maxime Samson, Thomas Barba, David Jayne, Arianna Troilo, Jens Thiel, Bernhard Hellmich, Sara Monti, Carlomaurizio Montecucco, Carlo Salvarani, Jean-Emmanuel Kahn, Bernard Bonnotte, Cécile-Audrey Durel, Xavier Puéchal, Luc Mouthon, Loïc Guillevin, Giacomo Emmi, Augusto Vaglio, Benjamin Terrier, the French Vasculitis Study Group and the EGPA European Study Group

doi : 10.1093/rheumatology/keaa805

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4355–4360

Only a third of patients with eosinophilic granulomatosis with polyangiitis (EGPA) are ANCA-positive, mainly directed against MPO. ANCA directed against PR3 are rarely found in EGPA. We aimed to examine the significance of PR3-ANCA in EGPA.

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Ultrasonographic Halo Score in giant cell arteritis: association with intimal hyperplasia and ischaemic sight loss 

Kornelis S. M. van der Geest, Konrad Wolfe, Frances Borg, Alwin Sebastian, Abdul Kayani, Alessandro Tomelleri, Prisca Gondo, Wolfgang A. Schmidt, Raashid Luqmani, Bhaskar Dasgupta

doi : 10.1093/rheumatology/keaa806

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4361–4366

We investigated the relationship between the ultrasonographic Halo Score and temporal artery biopsy (TAB) findings in GCA.

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Improvement from ixekizumab treatment in patients with psoriatic arthritis who have had an inadequate response to one or two TNF inhibitors 

Bruce Kirkham, Carlos Sesin, Amanda M. Gellett, Aubrey T. Sprabery, Chen-Yen Lin, Anthony Turkiewicz

doi : 10.1093/rheumatology/keaa824

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4367–4372

To evaluate the efficacy of ixekizumab (IXE), a monoclonal antibody selectively targeting interleukin-17A, in patients with inadequate response to one or two TNF inhibitors (TNFi).

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Anti-tumour necrosis factor treatment for the prevention of ischaemic events in patients with deficiency of adenosine deaminase 2 (DADA2)

Samantha Cooray, Ebun Omyinmi, Ying Hong, Charalampia Papadopoulou, Lorraine Harper, Eslam Al-Abadi, Ruchika Goel, Shirish Dubey, Mark Wood, Stephen Jolles, Stefan Berg, Maria Ekelund, Kate Armon, Despina Eleftheriou, Paul A Brogan

doi : 10.1093/rheumatology/keaa837

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4373–4378

To evaluate the impact of anti-Tumour Necrosis Factor-? (anti-TNF) treatment on the occurrence of vasculitic ischaemic events in patients with deficiency of adenosine deaminase 2 (DADA2).

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Salivary glands involvement: a new indicator of juvenile idiopathic oligoarticular arthritis (preliminary results)

Patrizia Defabianis, Franco Garofalo, Federica Romano

doi : 10.1093/rheumatology/keaa891

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4379–4383

Early diagnosis is critical to reduce long-term disability in patients with JIA by ensuring prompt treatment. The aim of this cross-sectional study was to evaluate the salivary gland function in JIA, addressing specifically oligoarticular (JIA1) and polyarticular (JIA2) subtypes, compared with healthy controls. This may contribute to the identification of salivary risk indicators of the disease that may help diagnosis at an early stage or even before the onset of other clinical evidence.

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Use of 18F-fluorodeoxyglucose positron emission tomography to monitor tocilizumab effect on vascular inflammation in giant cell arteritis

Kaitlin A. Quinn, Himanshu Dashora, Elaine Novakovich, Mark A. Ahlman, Peter C. Grayson

doi : 10.1093/rheumatology/keaa894

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4384–4389

To evaluate the time-dependent effects of tocilizumab on vascular inflammation as measured by 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) in GCA.

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Evaluation of PR3- and MPO-ANCA line and dot immunoassays in ANCA-associated vasculitis

Nina Kempiners, Juliane Mahrhold, Bernhard Hellmich, Elena Csernok

doi : 10.1093/rheumatology/keaa776

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4390–4394

This study was performed to evaluate the diagnostic accuracy of novel line and dot immunoassays for detection of MPO and PR3 ANCA.

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The intracellular chloride channel 4 (CLIC4) activates systemic sclerosis fibroblasts

Christopher W. Wasson, Rebecca. L. Ross, Ruth Morton, Jamel Mankouri, Francesco Del Galdo

doi : 10.1093/rheumatology/keaa797

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4395–4400

Tissue fibrosis in SSc is driven by active fibroblasts (myofibroblasts). Previous studies have shown the intracellular chloride channel 4 (CLIC4) mediates the activation of cancer-associated fibroblasts. In this study we investigated the role of CLIC4 in SSc fibroblast activation.

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Decreased serum ACE2 levels in patients with connective tissue diseases

Xiaojun Tang, Linyu Geng, Xuebing Feng, Lingyun Sun

doi : 10.1093/rheumatology/keaa898

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4401–4406

To evaluate serum concentration and activity of angiotensin-converting enzyme 2 (ACE2) in patients with connective tissue diseases (CTDs).

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Genome-wide association study on Northern Chinese identifies KLF2, DOT1L and STAB2 associated with systemic lupus erythematosus

Qin Song, Yao Lei, Li Shao, Weiyang Li, Qingsheng Kong, Zhiming Lin, Xiao Qin, Wei Wei, Fei Hou, Jian Li, Xianghua Guo, Yujing Mao, Yujie Cao, Zhongyi Liu, Lichuan Zheng, Rui Liang, Yuping Jiang, Yan Liu, Lili Zhang, Jing Yang, Yu Lung Lau, Yan Zhang, Bo Ban, Yong-Fei Wang, Wanling Yang

doi : 10.1093/rheumatology/keab016

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4407–4417

To identify novel genetic loci associated with systemic lupus erythematosus (SLE) and to evaluate potential genetic differences between ethnic Chinese and European populations in SLE susceptibility.

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Solving the mystery of HBV-related mixed cryoglobulinemia: potential biomarkers of disease progression

Krizia Pocino, Cecilia Napodano, Laura Gragnani, Gabriele Ciasca, Stefania Colantuono, Silvia Marri, Lorenzo Vantaggio, Francesca Gulli, Serena Lorini, Antonella Barini, Annunziata Stefanile, Luca Miele, Milvia Casato, Anna Linda Zignego, Gian Ludovico Rapaccini, Mariapaola Marino, Marcella Visentini, Umberto Basile

doi : 10.1093/rheumatology/keab157

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4418–4427

The biomarkers of an immunological dysregulation due to a chronic HBV infection are indeed understudied. If untreated, this condition may evolve into liver impairment co-occurring with extrahepatic involvements. Here, we aim to identify a new panel of biomarkers [including immunoglobulin G (IgG) subclasses, RF, and Free Light Chains (FLCs)] that may be useful and reliable for clinical evaluation of HBV-related cryoglobulinemia.

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Anti-MDA-5-positive dermatomyositis associated rapidly progressive interstitial lung disease, a virus-triggered autoimmune-like symptom?

Lingxiao Xu, Lei Wang, Chengyin Lv, Wenfeng Tan

doi : 10.1093/rheumatology/keab224

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4428–4429

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An X chromosome-wide meta-analysis based on Japanese cohorts revealed that non-autosomal variations are associated with serum urate 

Masahiro Nakatochi, Yu Toyoda, Masahiro Kanai, Akiyoshi Nakayama, Yusuke Kawamura, Asahi Hishida, Haruo Mikami, Keitaro Matsuo, Toshiro Takezaki, Yukihide Momozawa, The Biobank Japan Project, Yoichiro Kamatani, Sahoko Ichihara, Nariyoshi Shinomiya, Mitsuhiro Yokota, Kenji Wakai, Yukinori Okada, Hirotaka Matsuo, the Japan Uric Acid Genomics Consortium (Japan Urate)

doi : 10.1093/rheumatology/keab404

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4430–4432

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New prescribing recommendations bring an opportunity to focus on better reproductive health for women with rheumatic disease

Iona Thorne

doi : 10.1093/rheumatology/keab409

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4432–4434

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Point-of-care ultrasound enables diagnosis of giant cell arteritis with a modern innovative handheld probe

Pantelis Karakostas, Christian Dejaco, Charlotte Behning, Florian Recker, Valentin Sebastian Schäfer

doi : 10.1093/rheumatology/keab424

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4434–4436

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No added value of optimal spectral transmission imaging in persons at risk of rheumatoid arthritis

Laurette van Boheemen, Annelies Blanken, Aja Kuin, Dirkjan van Schaardenburg

doi : 10.1093/rheumatology/keab435

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4436–4438

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Giant cell arteritis involving the posterior auricular artery

Kei Kobayashi, Shunichiro Hanai, Daiki Nakagomi

doi : 10.1093/rheumatology/keaa886

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4439–4440

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Dense calcifications of giant coronary aneurysms in an 11-year-old girl with Kawasaki disease

Rakesh Kumar Pilania, Manphool Singhal, Surjit Singh

doi : 10.1093/rheumatology/keaa907

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4441–4442

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A huge pancreatic cyst in eosinophilic granulomatosis with polyangitis

Maurizio Marvisi, Mario Uccelli

doi : 10.1093/rheumatology/keab008

Rheumatology, Volume 60, Issue 9, September 2021, Page 4443

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Pulmonary apical fibrosis as the initial symptom of ankylosing spondylitis 

Aydan Koken Avsar, Tuba Yuce Inel, Sinem Burcu Kocaer, Gercek Can

doi : 10.1093/rheumatology/keab012

Rheumatology, Volume 60, Issue 9, September 2021, Pages 4444–4445

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Osteoporosis circumscripta cranii with a pathological fracture of the skull

Matthew Colquhoun, Henry Penn, Clare Batten

doi : 10.1093/rheumatology/keab013

Rheumatology, Volume 60, Issue 9, September 2021, Page 4446

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Mycobacterium bovis infection under apremilast in Behçet's syndrome

Matheus Vieira, Mathieu Vautier, Frédéric Charlotte, David Saadoun

doi : 10.1093/rheumatology/keab199

Rheumatology, Volume 60, Issue 9, September 2021, Pages e301–e303

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A case of VEXAS syndrome manifesting as Kikuchi-Fujimoto disease, relapsing polychondritis, venous thromboembolism and macrocytic anaemia

Shang Ming Samuel Lee, Bingwen Eugene Fan, Joel Hua-Liang Lim, Liuh Ling Goh, Joyce Siong See Lee, Li Wearn Koh

doi : 10.1093/rheumatology/keab200

Rheumatology, Volume 60, Issue 9, September 2021, Pages e304–e306

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Severe hepatotoxicity as a rare side effect of anakinra in a patient with systemic JIA

G. M. Murray, S. Kheng Ng, D. Beasley, L. Johansen, A. V. Ramanan

doi : 10.1093/rheumatology/keab202

Rheumatology, Volume 60, Issue 9, September 2021, Pages e307–e308

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JAK inhibition for treatment of psoriatic arthritis in Down syndrome 

Andrew T. Pham, Angela L. Rachubinski, Belinda Enriquez-Estrada, Kayleigh Worek, Melissa Griffith, Joaquin M. Espinosa

doi : 10.1093/rheumatology/keab203

Rheumatology, Volume 60, Issue 9, September 2021, Pages e309–e311

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Contrary immediate effect of abatacept on skin and joint manifestations in psoriatic arthritis

Larissa Valor-Méndez, Arnd Kleyer, Georg Schett, Bernhard Manger, Michael Sticherling

doi : 10.1093/rheumatology/keab206

Rheumatology, Volume 60, Issue 9, September 2021, Pages e312–e313

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VEXAS syndrome in a patient with previous spondyloarthritis with a favourable response to intravenous immunoglobulin and anti-IL17 therapy

Marion Magnol, Loukianos Couvaras, Yannick Degboé, Eric Delabesse, Cristina Bulai-Livideanu, Adeline Ruyssen-Witrand, Arnaud Constantin

doi : 10.1093/rheumatology/keab211

Rheumatology, Volume 60, Issue 9, September 2021, Pages e314–e315

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Colitis expands the phenotype of PAAND patients: new case report and review of the literature

Juan L. Valdivieso Shephard, Chamaida Plasencia Rodríguez, Cristina Suárez Ferrer, Diana Peiteado López, Alejandro Balsa Criado, Eduardo López-Granados, María Bravo García-Morato

doi : 10.1093/rheumatology/keab215

Rheumatology, Volume 60, Issue 9, September 2021, Pages e316–e317

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MRI negative myeloradiculoneuropathy unmasking systemic lupus erythematosus

Shambaditya Das, Biman Kanti Ray, Alak Pandit, Souvik Dubey

doi : 10.1093/rheumatology/keab216

Rheumatology, Volume 60, Issue 9, September 2021, Pages e318–e320

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VEXAS syndrome: still expanding the clinical phenotype

Artem Oganesyan, Vincent Jachiet, Francois Chasset, Pierre Hirsch, Mehdi Hage-Sleiman, Bettina Fabiani, Paul Duriez, Sophie Georgin-Lavialle, Francois Delhommeau, Yervand Hakobyan, Olivier Fain, Arsène Mekinian, on behalf MINHEMON

doi : 10.1093/rheumatology/keab225

Rheumatology, Volume 60, Issue 9, September 2021, Pages e321–e323

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PET-CT scan to diagnose and demonstrate resolution of lupus lymphadenitis with belimumab

S. Garcia-Cirera, J. Calvet, J. C. Martin-Marimon, R. B. García-Chamón, J. Gratacós

doi : 10.1093/rheumatology/keab226

Rheumatology, Volume 60, Issue 9, September 2021, Pages e324–e325

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Severe thrombotic microangiopathy after autologous stem cell transplantation in systemic sclerosis: a case report

Maryam O. Sharif, Helen L. Leavis, Pieter van Paassen, Anna van Rhenen, Sjoerd A. M. E. G. Timmermans, Evelien Ton, Jacob M. van Laar, Julia Spierings

doi : 10.1093/rheumatology/keab239

Rheumatology, Volume 60, Issue 9, September 2021, Pages e326–e328

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Anti-PD-1 associated retroperitoneal fibrosis

Dimitrios Daoussis, Pantelis Kraniotis, Foteini Kalofonou, Haralabos Kalofonos

doi : 10.1093/rheumatology/keab264

Rheumatology, Volume 60, Issue 9, September 2021, Pages e329–e330

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A 72-year-old man with life-threatening dyspnoea due to a neck mass of unexpected origin 

Nick M.A. Schubert, Nynke A. Jager, Jeroen Vister, Boudewijn E.C. Plaat

doi : 10.1093/rheumatology/keab295

Rheumatology, Volume 60, Issue 9, September 2021, Pages e331–e333

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Comment on: Real-world single centre use of JAK inhibitors across the rheumatoid arthritis pathway

Francesca Romana Spinelli, Cristina Garufi, Silvia Mancuso, Fulvia Ceccarelli, Fabrizio Conti

doi : 10.1093/rheumatology/keab195

Rheumatology, Volume 60, Issue 9, September 2021, Pages e334–e336

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Comment on: Risk factors associated with pneumocystis jirovecii pneumonia in juvenile myositis in North America

Tomohiro Yoshida, Daisuke Waki

doi : 10.1093/rheumatology/keab411

Rheumatology, Volume 60, Issue 9, September 2021, Pages e337–e338

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Comment on: Beneficial effect of Mediterranean diet on disease activity and cardiovascular risk in systemic lupus erythematosus patients: a cross-sectional study

Yusuke Matsuo, Toshimasa Shimizu, Yoshie Gon, Motohiko Sato, Sho Matsushita, Takashi Yoshioka

doi : 10.1093/rheumatology/keab281

Rheumatology, Volume 60, Issue 9, September 2021, Pages e339–e340

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Comment on: Association between environmental air pollution and rheumatoid arthritis flares: reply

Giovanni Adami, Maurizio Rossini

doi : 10.1093/rheumatology/keab282

Rheumatology, Volume 60, Issue 9, September 2021, Page e341

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Comment on: Femoral vein wall thickness measurement: A new diagnostic tool for Behçet’s disease

Tetsuro Aita, Yusuke Matsuo, Yoshie Yamada, Jun Miyata, Takashi Yoshioka

doi : 10.1093/rheumatology/keab319

Rheumatology, Volume 60, Issue 9, September 2021, Pages e342–e343

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Comment on: What is the meaning of ANCA positivity in IgG4-related disease?

Hakan Apaydin, Berkan Arma?an, Serdar C. Güven, Ahmet Omma, Orhan Kucuksahin, Abdulsamet Erden

doi : 10.1093/rheumatology/keab356

Rheumatology, Volume 60, Issue 9, September 2021, Pages e344–e345

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Comment on ‘Axial And Peripheral spondyloarthritis: does Psoriasis influence the clinical expression and disease burden? Data from REGISPONSER registry’

Yen-Po Lin, Chih-Jung Yeh, James Cheng-Chung Wei

doi : 10.1093/rheumatology/keab390

Rheumatology, Volume 60, Issue 9, September 2021, Page e346

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Comment on: Acquired low immunoglobulin levels and risk of clinically relevant infection in adult patients with systemic lupus erythematosus

Wen-Kang Yang, Chih-Jen Tseng, James Cheng-Chung Wei

doi : 10.1093/rheumatology/keab408

Rheumatology, Volume 60, Issue 9, September 2021, Page e347

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