Articles

Development of a benchmarking toolkit for adolescent and young adult rheumatology services (BeTAR)

Young people (YP; 12–24 years old) with rheumatic diseases face many challenges associated with chronic illness in addition to the physiological and psychosocial changes of adolescence. Timely access to develo...

Authors: Ran A. Cai, Hema Chaplin, Polly Livermore, Martin Lee, Debajit Sen, Lucy R. Wedderburn, Nick Wilkinson, Rachel Jeffery, Andrea Kempa, Imogen Norton, Rachel Tattersall, Yiannis Ioannou and Despina Eleftheriou

10th Congress of International Society of Systemic Auto-Inflammatory Diseases (ISSAID)

Authors:

Periodic fever syndromes: beyond the single gene paradigm

Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease in Canada and is characterized by a clinical syndrome of episodic inflammatory symptoms. Traditionally, the disease is d...

Authors: Clara Westwell-Roper, Iwona Niemietz, Lori B. Tucker and Kelly L. Brown

Patient-proxy agreement on health-related quality of life in juvenile fibromyalgia syndrome

Health-related quality of life (HRQoL) measures serve as important indicators of pain-related physical and psychosocial function in youth with juvenile fibromyalgia syndrome (JFMS). While the administration of...

Authors: Sabrina Gmuca, Rui Xiao and David D. Sherry

Seeking the state of the art in standardized measurement of health care resource use and costs in juvenile idiopathic arthritis: a scoping review

This study aims to describe current practice in identifying and measuring health care resource use and unit costs in economic evaluations or costing studies of juvenile idiopathic arthritis (JIA).

Authors: Michelle M. A. Kip, Gillian Currie, Deborah A. Marshall, Luiza Grazziotin Lago, Marinka Twilt, Sebastiaan J. Vastert, Joost F. Swart, Nico Wulffraat, Rae S. M. Yeung, Susanne M. Benseler and Maarten J. IJzerman

Disease course and treatment effects of a JAK inhibitor in a patient with CANDLE syndrome

CANDLE syndrome (an acronym for Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated Temperature) is a recently described rare autosomal recessive disorder charaterized by systemic autoinfl...

Authors: M. Boyadzhiev, L. Marinov, V. Boyadzhiev, V. Iotova, I. Aksentijevich and S. Hambleton

Intravenous abatacept in Japanese patients with polyarticular-course juvenile idiopathic arthritis: results from a phase III open-label study

To investigate efficacy and safety of intravenous abatacept in Japanese patients with active polyarticular-course juvenile idiopathic arthritis (pJIA).

Authors: Ryoki Hara, Hiroaki Umebayashi, Syuji Takei, Nami Okamoto, Naomi Iwata, Yuichi Yamasaki, Yasuo Nakagishi, Toshitaka Kizawa, Ichiro Kobayashi, Tomoyuki Imagawa, Noriko Kinjo, Norihito Amano, Yoko Takahashi, Masaaki Mori, Yasuhiko Itoh and Shumpei Yokota

A qualitative study examining the validity and comprehensibility of physical activity items: developed and tested in children with juvenile idiopathic arthritis

Not all physical activity (PA) questionnaires (PAQ) gather information regarding PA intensity, duration, and modes and only a few were developed specifically for children. We assessed children’s comprehensibil...

Authors: August Flodén, Eva W. Broström, Johan von Heideken, Sara Rostlund, Rikard Nilsson, Kristina Löwing and Maura D. Iversen

Clinical features and characteristics of uveitis associated with juvenile idiopathic arthritis in Japan: first report of the pediatric rheumatology association of Japan (PRAJ)

Although there are many reports on Juvenile Idiopathic arthritis-associated uveitis (JIA-U) from various countries, especially from Europe and North America, there are few reports from Asia. Our aim was to inv...

Authors: Junko Yasumura, Masato Yashiro, Nami Okamoto, Kosuke Shabana, Hiroaki Umebayashi, Naomi Iwata, Yuka Okura, Tomohiro Kubota, Masaki Shimizu, Minako Tomiita, Yasuo Nakagishi, Kenichi Nishimura, Ryoki Hara, Mao Mizuta, Takahiro Yasumi, Fumiya Yamaide…

Measurement properties and performance of an eight-minute submaximal treadmill test in patients with juvenile idiopathic arthritis: a controlled study

Poor cardiorespiratory fitness is previously reported in patients with juvenile idiopathic arthritis (JIA) measured both by maximal and submaximal exercise tests, but a submaximal exercise test with acceptable...

Authors: Kristine Risum, Elisabeth Edvardsen, Anne M. Selvaag, Hanne Dagfinrud and Helga Sanner

Increase expression of CD177 in Kawasaki disease

Kawasaki disease (KD) is the most common acute coronary vasculitis disease to occur in children. Its incidence has been attributed to the combined effects of infection, genetics, and immunity. Although the eti...

Authors: Ying-Hsien Huang, Mao-Hung Lo, Xin-Yuan Cai, Shih-Feng Liu and Ho-Chang Kuo

Screening of asymptomatic rheumatic heart disease among refugee/migrant children and youths in Italy

Rheumatic heart disease (RHD) is a chronic condition responsible of congestive heart failure, stroke and arrhythmia. Almost eradicated in high-income countries (HIC), it persists in low- and middle-income coun...

Authors: Fortunata Condemi, Gabriele Rossi, Miguel Lupiz, Antonio Pagano, Federica Zamatto, Stefano Marini, Francesco Romeo and Gianfranco De Maio

Efficacy and cost savings with the use of a minimal sedation / anxiolysis protocol for intra-articular corticosteroid injections in children with juvenile idiopathic arthritis: a retrospective review of prospectively collected data

Intra-articular corticosteroid injections (IACI) are frequently used in the treatment of juvenile idiopathic arthritis. There is a paucity of evidence-based research describing methods of pain and anxiety cont...

Authors: Rotem Elitsur, April Hollenbeck, Laura Tasan, Kathryn S. Torok, Elaine Cassidy, Brian Blasiole, Erika Parsons, Chelsea Acock, Joseph Angelelli and Isabela-Cajiao Angelelli

Different histological classifications for Henoch-Schönlein purpura nephritis: which one should be used?

Nephritis is the most important chronic complication of IgA Vasculitis (IgAV)/Henoch-Schönlein purpura (IGAV/HSP) and thus the main prognostic factor of this most common childhood vasculitis. Since the prognos...

Authors: Marija Jelusic, Mario Sestan, Rolando Cimaz and Seza Ozen

Development and pilot testing of the treatment and education approach for childhood-onset lupus (TEACH): a cognitive behavioral treatment

To develop and test the feasibility and initial effectiveness of the Treatment and Education Approach for Childhood-onset Lupus (TEACH) protocol, a 6-session cognitive behavioral therapy (CBT) intervention for...

Authors: Natoshia R. Cunningham, Lauren M. Fussner, Erin Moorman, Pinar O. Avar Aydin, Hermine I. Brunner and Susmita Kashikar-Zuck

Pediatric anti-NMDA receptor encephalitis with catatonia: treatment with electroconvulsive therapy

Anti-NMDA receptor encephalitis, an autoimmune disease associated with antibodies against N-methyl-D-aspartate (NMDA) receptors, is being diagnosed more frequently, especially in children and young adults. Acu...

Authors: Taha Moussa, Khalid Afzal, Joseph Cooper, Ryan Rosenberger, Karyn Gerstle and Linda Wagner-Weiner

Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome

Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) were historically thought to be distinct entities, often managed in isolation. In fact, these conditions are closely related. A...

Authors: Olha Halyabar, Margaret H. Chang, Michelle L. Schoettler, Marc A. Schwartz, Ezgi H. Baris, Leslie A. Benson, Catherine M. Biggs, Mark Gorman, Leslie Lehmann, Mindy S. Lo, Peter A. Nigrovic, Craig D. Platt, Gregory P. Priebe, Jared Rowe, Robert P. Sundel, Neeraj K. Surana…

Participation in school sports among children and adolescents with juvenile idiopathic arthritis in the German National Paediatric Rheumatologic Database, 2000–2015: results from a prospective observational cohort study

Regular school sports can help adolescents achieve the recommended amount of daily physical activity and provide knowledge, attitudes and behavioral skills that are needed in order to adopt and maintain a phys...

Authors: Florian Milatz, Jens Klotsche, Martina Niewerth, Nils Geisemeyer, Ralf Trauzeddel, Elisabeth Weißbarth-Riedel, Tilmann Kallinich, Joachim Peitz, Matthias Hartmann and Kirsten Minden

The pediatric rheumatology objective structured clinical examination: progressing from a homegrown effort toward a reliable and valid national formative assessment

Of 37 pediatric rheumatology fellowship training programs in the United States, many have three or fewer fellows at a given time, making large-scale assessment of fellow performance difficult. An objective str...

Authors: Megan L. Curran, Emma E. Martin, Erin C. Thomas, Rashmi Singh, Saima Armana, Asnia Kauser, Eesha A. Zaheer and David D. Sherry

The effects of TNF-alpha inhibitor therapy on the incidence of infection in JIA children: a meta-analysis

Juvenile Idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood. The diagnosis is based on the underlying symptoms of arthritis with an exclusion of other diseases Biologic agents...

Authors: Arnold Nagy, Péter Mátrai, Péter Hegyi, Hussain Alizadeh, Judit Bajor, László Czopf, Zoltán Gyöngyi, Zoltán Kiss, Katalin Márta, Mária Simon, Ágnes Lilla Szilágyi, Gábor Veres and Bernadett Mosdósi

Coronary artery dilation associated with anti-synthetase syndrome in an adolescent

Idiopathic inflammatory myopathies (IIM) are a group of systemic autoimmune disorders primarily affecting skeletal muscle. Pediatric coronary artery dilation is frequently discussed in Kawasaki disease. Howeve...

Authors: Karim Asi, Anand Gourishankar and Ankur Kamdar

The effectiveness of a multidisciplinary intervention strategy for the treatment of symptomatic joint hypermobility in childhood: a randomised, single Centre parallel group trial (The Bendy Study)

Joint hypermobility is common in childhood and can be associated with musculoskeletal pain and dysfunction. Current management is delivered by a multidisciplinary team, but evidence of effectiveness is limited...

Authors: Peter Bale, Vicky Easton, Holly Bacon, Emma Jerman, Laura Watts, Garry Barton, Allan Clark, Kate Armon and Alex J. MacGregor

Kawasaki disease shock syndrome: clinical characteristics and possible use of IL-6, IL-10 and IFN-γ as biomarkers for early recognition

As an acute febrile and inflammatory disease, Kawasaki disease (KD) could develop Kawasaki disease shock syndrome (KDSS) sometimes. However its pathogenesis was still not well known. This study was to learn mo...

Authors: Yandie Li, Qi Zheng, Lixia Zou, Jianqiang Wu, Li Guo, Liping Teng, Rongjun Zheng, Lawrence Kwok Leung Jung and Meiping Lu

Arterial properties in adults with long-lasting active juvenile idiopathic arthritis compared to healthy controls

The data on cardiovascular risk and systemic arterial properties in patients with long-lasting juvenile idiopathic arthritis (JIA) is limited. The objective of this study was to describe systemic arterial prop...

Authors: Hanne Aaserud Aulie, Mette-Elise Estensen, Anne Marit Selvaag, Vibke Lilleby, Berit Flatø and Svend Aakhus

Clinical phenotypes and biologic treatment use in juvenile dermatomyositis-associated calcinosis

Few risk factors have been identified for the development of calcinosis among patients with Juvenile Dermatomyositis, and currently no clinical phenotype has been associated with its development. We analyzed a...

Authors: Amir B. Orandi, Vikas R. Dharnidharka, Noor Al-Hammadi and Kevin W. Baszis

Coronary artery status of patients with transient fever 24–36 h after first IVIG infusion did not differ from that seen in responsive patients

Current management guidelines for patients with Kawasaki disease (KD) differ in their recommendations for fever observation times when determining resistance to initial intravenous immunoglobulin (IVIG). This ...

Authors: Jae Suk Baek, Jeong Jin Yu, Mi Jin Kim, Jihye You, Hyun Ok Jun, Young-Hwue Kim and Jae-Kon Ko

Body composition and phase angle as an indicator of nutritional status in children with juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA) is the most common chronic, systemic autoimmune connective tissue disease diagnosed in children and adolescents. An important aspect of monitoring of children with JIA is a ...

Authors: Paweł Więch, Izabela Sałacińska, Dariusz Bazaliński and Mariusz Dąbrowski

Living with autoinflammatory diseases: identifying unmet needs of children, adolescents and adults

Autoinflammatory diseases (AIDs) illnesses of the innate immunity resulting in clinical signs and symptoms of systemic inflammation and loss of organ functions. While pathophysiological mechanisms are heavily ...

Authors: Gabriele Erbis, Kirstin Schmidt, Sandra Hansmann, Tetiana Sergiichuk, Christine Michler, Jasmin B. Kuemmerle-Deschner and Susanne M. Benseler

Multi-centre national audit of juvenile localised scleroderma: describing current UK practice in disease assessment and management

To describe current United Kingdom practice in assessment and management of patients with juvenile localised scleroderma (JLS) compared to Paediatric Rheumatology European Society (PRES) scleroderma working pa...

Authors: Hanna Lythgoe, Beverley Almeida, Joshua Bennett, Chandrika Bhat, Amarpal Bilkhu, Mary Brennan, Samundeeswari Deepak, Pamela Dawson, Despina Eleftheriou, Kathryn Harrison, Daniel Hawley, Eleanor Heaf, Valentina Leone, Ema Long, Sarah Maltby, Flora McErlane…

Off-label use of tocilizumab to treat non-juvenile idiopathic arthritis in pediatric rheumatic patients: a literature review

Tocilizumab, an anti-interleukin-6 (IL-6) agent, is indicated as a treatment for several autoimmune or inflammatory diseases, including rheumatoid arthritis and juvenile idiopathic arthritis (JIA). IL-6 plays ...

Authors: Ju-Yang Jung, Moon-Young Kim, Chang-Hee Suh and Hyoun-Ah Kim

Disease burden and social impact of pediatric chronic nonbacterial osteomyelitis from the patient and family perspective

Chronic nonbacterial osteomyelitis (CNO) is an autoinflammatory bone disorder that if left untreated can result in bone destruction and severe continuing pain due to persistent inflammation. The impact this ch...

Authors: Melissa Oliver, Tzielan C. Lee, Bonnie Halpern-Felsher, Elizabeth Murray, Rebecca Schwartz and Yongdong Zhao

Educational initiatives and training for paediatric rheumatology in Europe

The Paediatric Rheumatology European Society (PReS) has over many years, developed a portfolio of educational activities to address increasing educational needs of workforce and support young clinicians to acq...

Authors: Helen E. Foster, Jelena Vojinovic, Tamas Constantin, Alberto Martini, Pavla Dolezalova, Yosef Uziel, E.M.D Smith, Lovro Lamot, Carine Wouters, Tadej Avcin and Nico Wulffraat

Protective parents and permissive children: what qualitative interviews with parents and children can tell us about the feasibility of juvenile idiopathic arthritis trials

Patient recruitment can be very challenging in paediatric studies, especially in relatively uncommon conditions, such as juvenile idiopathic arthritis (JIA). However, involving children and young people (CYP) ...

Authors: Frances C. Sherratt, Louise Roper, Simon R. Stones, Flora McErlane, Matthew Peak, Michael W. Beresford, Helen Foster, Athimalaipet V. Ramanan, Madeleine Rooney, Eileen Baildam and Bridget Young

Feasibility of a musculoskeletal ultrasound intervention to improve adherence in juvenile idiopathic arthritis: a proof-of concept trial

Non-adherence is a prevalent and modifiable issue in juvenile idiopathic arthritis (JIA) that currently lacks provider-based intervention. Education surrounding disease status is one way in which families rema...

Authors: Leslie A. Favier, Tracy V. Ting and Avani C. Modi

Chronic non bacterial osteitis- a multicentre study

To understand the demographics, clinical features and treatment outcomes of Chronic Non-bacterial Osteitis (CNO) from three tertiary paediatric rheumatology services in the United Kingdom.

Authors: Chandrika S. Bhat, Catriona Anderson, Aoibhinn Harbinson, Liza J. McCann, Marion Roderick, Adam Finn, Joyce E. Davidson and Athimalaipet V. Ramanan

Health-related quality of life in children with inflammatory brain disease

To quantify the impact of inflammatory brain diseases in the pediatric population on health-related quality of life, including the subdomains of physical, emotional, school and social functioning.

Authors: Elina Liu, Marinka Twilt, Pascal N. Tyrrell, Anastasia Dropol, Shehla Sheikh, Mark Gorman, Susan Kim, David A. Cabral, Rob Forsyth, Heather Van Mater, Suzanne Li, Adam M. Huber, Elizabeth Stringer, Eyal Muscal, Dawn Wahezi, Mary Toth…

Long-term follow-up of autologous hematopoietic stem cell transplantation for refractory juvenile dermatomyositis: a case-series study

To follow up the refractory juvenile dermatomyositis (JDM) with autologous hematopoietic stem cell transplantation (AHSCT) in a long time and to investigate whether AHSCT is effective and safe to treat refract...

Authors: Jia Zhu, Gaixiu Su, Jianming Lai, Boya Dong, Min Kang, Shengnan Li, Zhixuan Zhou and Fengqi Wu

Rituximab treatment for chronic steroid-dependent Henoch-Schonlein purpura: 8 cases and a review of the literature

Henoch-Schonlein purpura (HSP) is a small vessel vasculitis that is characterized by non-thrombocytopenic purpura, abdominal pain, arthritis, and glomerulonephritis. Typically, HSP is self-limited requiring on...

Authors: Courtney B. Crayne, Esraa Eloseily, Melissa L. Mannion, Saji P. Azerf, Peter Weiser, Timothy Beukelman, Matthew L. Stoll, Daniel I. Feig, T. Prescott Atkinson and Randy Quentin Cron

Inpatient burden of juvenile dermatomyositis among children in the United States

Juvenile dermatomyositis (JDM) is a rare autoimmune disease that causes significant morbidity and quality of life impairment. Little is known about the inpatient burden of JDM in the US. Our goal was to determ...

Authors: Michael C. Kwa, Jonathan I. Silverberg and Kaveh Ardalan

Juvenile idiopathic arthritis managed in the new millennium: one year outcomes of an inception cohort of Australian children

The advent of new treatments for Juvenile Idiopathic Arthritis (JIA) has prompted interest in systematically studying the outcomes of patients treated in the ‘modern era’. Such data provide both benchmarks for...

Authors: Georgina Tiller, Joanne Buckle, Roger Allen, Jane Munro, Peter Gowdie, Angela Cox and Jonathan Akikusa

Self-management needs of Irish adolescents with Juvenile Idiopathic Arthritis (JIA): how can a Canadian web-based programme meet these needs?

Juvenile Idiopathic Arthritis (JIA) affects over 1000 children and adolescents in Ireland, potentially impacting health-related quality-of-life. Accessible self-management strategies, including Internet-based int...

Authors: Grace O’Sullivan, Siobhán O’Higgins, Line Caes, Sophia Saetes, Brian E. McGuire and Jennifer Stinson

Feasibility and safety of a 6-month exercise program to increase bone and muscle strength in children with juvenile idiopathic arthritis

Arthritis in childhood can be associated with muscle weakness around affected joints, low bone mass and low bone strength. Exercise is recognized as an important part of management of children with juvenile id...

Authors: Kristin M. Houghton, Heather M. Macdonald, Heather A. McKay, Jaime Guzman, Ciarán Duffy and Lori Tucker

A wearable activity tracker intervention for promoting physical activity in adolescents with juvenile idiopathic arthritis: a pilot study

Children and adolescents with juvenile idiopathic arthritis (JIA) are less physically active than their healthy peers and are at high risk of missing out on the general health benefits of physical activity. We...

Authors: Liane D. Heale, Saunya Dover, Y. Ingrid Goh, Victoria A. Maksymiuk, Greg D. Wells and Brian M. Feldman

Pilot study comparing the childhood arthritis and rheumatology research alliance consensus treatment plans for induction therapy of juvenile proliferative lupus nephritis

To reduce treatment variability and facilitate comparative effectiveness studies, the Childhood Arthritis and Rheumatology Research Alliance (CARRA) published consensus treatment plans (CTPs) including one for...

Authors: Jennifer C Cooper, Kelly Rouster-Stevens, Tracey B Wright, Joyce J Hsu, Marisa S Klein-Gitelman, Stacy P Ardoin, Laura E Schanberg, Hermine I Brunner, B Anne Eberhard, Linda Wagner-Weiner, Jay Mehta, Kathleen Haines, Deborah K McCurdy, Thomas A Phillips, Zhen Huang and Emily von Scheven

Physical activity in patients with oligo- and polyarticular juvenile idiopathic arthritis diagnosed in the era of biologics: a controlled cross-sectional study

Knowledge about objectively measured levels of physical activity (PA) and PA participation (included facilitators and barriers for PA) in patients with juvenile idiopathic arthritis (JIA) diagnosed in the era ...

Authors: Kristine Risum, Bjørge Herman Hansen, Anne Marit Selvaag, Øyvind Molberg, Hanne Dagfinrud and Helga Sanner

Psychological characteristics of caregivers of pediatric patients with chronic rheumatic disease in relation to treatment adherence

Adherence to treatment for chronic diseases is lower in children than in adults, less extensively studied in children and is associated with multiple related factors. The aim of this study is to perform a desc...

Authors: Livia de Freitas Keppeke, Juliana Molina, Vanessa Bugni Miotto e Silva, Maria Teresa de Sande e Lemos Ramos Ascensão Terreri, Gerson Dierley Keppeke, Teresa Helena Schoen and Claudio Arnaldo Len

Improvement of survival rates in the last decade in Thai childhood-onset systemic lupus erythematosus

Morbidity and mortality in childhood onset systemic lupus erythematosus (SLE) is more severe than adult onset SLE. Long-term follow up is needed to determine the prognosis. The objectives of this study are to ...

Authors: Pondtip Jongvilaikasem, Edward B. McNeil, Pornsak Dissaneewate and Prayong Vachvanichsanong

Successful use of ofatumumab in two cases of early-onset juvenile SLE with thrombocytopenia caused by a mutation in protein kinase C δ

We previously described an endogamous Pakistani kindred in whom we identified a novel homozygous missense mutation in the PRKCD gene encoding for protein kinase C δ (PKCδ) as a cause of monogenic systemic lupus e...

Authors: Linda Lei, Sabina Muhammad, Muthana Al-Obaidi, Neil Sebire, Iek Leng Cheng, Despina Eleftheriou and Paul Brogan

Towards a new set of classification criteria for PFAPA syndrome

Diagnosis of Periodic Fever, Aphthous stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) syndrome is currently based on the modified Marshall’s criteria, but no validated evidence based classification crite...

Authors: Federica Vanoni, Roberta Caorsi, Sandra Aeby, Marie Cochard, Jordi Antón, Stefan Berg, Riva Brik, Pavla Dolezalova, Isabelle Koné-Paut, Benedicte Neven, Seza Ozen, Pascal Pillet, Silvia Stojanov, Carine Wouters, Marco Gattorno and Michaël Hofer

“I just want to get better”: experiences of children and youth with juvenile idiopathic arthritis in a home-based exercise intervention

Physical activity is essential for ensuring optimal physical function and fitness in children with juvenile idiopathic arthritis (JIA). Although exercise intervention trials informed current clinical practice,...

Authors: Joanie Sims-Gould, Douglas L. Race, Heather Macdonald, Kristin M. Houghton, Ciarán M. Duffy, Lori B. Tucker and Heather A. McKay