Articles

The role of age-specific N-terminal pro-brain natriuretic peptide cutoff values in predicting intravenous immunoglobulin resistance in Kawasaki disease: a prospective cohort study

The prediction of resistance to intravenous immunoglobulins (IVIG) is currently still one of the main research areas in Kawasaki disease (KD). Several studies have reported on the use of N-terminal pro-brain n...

Authors: Shuran Shao, Chunyan Luo, Kaiyu Zhou, Yimin Hua, Mei Wu, Lei Liu, Xiaoliang Liu and Chuan Wang

Reduction in the utilization of prednisone or methotrexate in Canadian claims data following initiation of etanercept in pediatric patients with juvenile idiopathic arthritis

In adult patients with arthritis, use of the tumor necrosis factor (TNF) inhibitor etanercept (ETN) is often associated with a reduction in the utilization of co-medications, particularly steroids. Comparative...

Authors: Majed Khraishi, Brad Millson, John Woolcott, Heather Jones, Lisa Marshall and Nicolino Ruperto

Complement lectin pathway protein levels reflect disease activity in juvenile idiopathic arthritis: a longitudinal study of the Nordic JIA cohort

To determine the serum levels of the lectin pathway proteins early in the disease course and 17 years after disease onset and to correlate the protein levels to markers of disease activity in participants from...

Authors: Mia Glerup, Steffen Thiel, Veronika Rypdal, Ellen Dalen Arnstad, Maria Ekelund, Suvi Peltoniemi, Kristiina Aalto, Marite Rygg, Susan Nielsen, Anders Fasth, Lillemor Berntson, Ellen Nordal and Troels Herlin

No radiographic wrist damage after treatment to target in recent-onset juvenile idiopathic arthritis

To evaluate radiographic progression of patients with new-onset juvenile idiopathic arthritis (JIA) in response to an early, tightly-controlled, treatment-to-target.

Authors: P. C. E. Hissink Muller, W. G. van Braak, D. Schreurs, C. M. Nusman, S. A. Bergstra, R. Hemke, D. Schonenberg-Meinema, J. M. van den Berg, T. W. Kuijpers, Y. Koopman-Keemink, M. A. J. van Rossum, L. W. A. van Suijlekom-Smit, D. M. C. Brinkman, C. F. Allaart, R. ten Cate and M. Maas

Rituximab-associated Hypogammaglobulinemia in pediatric patients with autoimmune diseases

Despite the increased use of rituximab in treating pediatric patients with autoimmune diseases in the last decade, there are limited data on rituximab safety in those subjects who have a developing immune syst...

Authors: Amer M. Khojah, Michael L. Miller, Marisa S. Klein-Gitelman, Megan L. Curran, Victoria Hans, Lauren M. Pachman and Ramsay L. Fuleihan

The pain trajectory of juvenile idiopathic arthritis (JIA): translating from adolescent patient report to behavioural sensitivity in a juvenile animal model

While pain is a common symptom in JIA patients, it remains unclear why some JIA patients develop ongoing or persistent pain. Complex clinical and social settings confound analysis of individual factors that ma...

Authors: Annastazia E. Learoyd, Debajit Sen and Maria Fitzgerald

An unprecedented COPA gene mutation in two patients in the same family: comparative clinical analysis of newly reported patients with other known COPA gene mutations

The COPA syndrome is a newly recognized monogenic, autosomal dominant autoimmune disease presenting mostly presenting in childhood. Clinical features include inflammation of the lungs, kidneys, and joints. App...

Authors: Anjali Patwardhan and Charles H. Spencer

Enthesitis-related arthritis is the most common category of juvenile idiopathic arthritis in Taiwan and presents persistent active disease

Juvenile idiopathic arthritis (JIA) has been categorized into seven different categories according to the International League of Associations for Rheumatology (ILAR) criteria. Enthesitis-related arthritis (ER...

Authors: Yang-Jen Shih, Yao-Hsu Yang, Chun-Ying Lin, Chia-Ling Chang and Bor-Luen Chiang

Intravenous dosing of tocilizumab in patients younger than two years of age with systemic juvenile idiopathic arthritis: results from an open-label phase 1 clinical trial

The anti–interleukin-6 receptor-alpha antibody tocilizumab was approved for intravenous (IV) injection in the treatment of patients with systemic juvenile idiopathic arthritis (sJIA) aged 2 to 17 years based o...

Authors: Navita L. Mallalieu, Sunethra Wimalasundera, Joy C. Hsu, Wendy Douglass, Chris Wells, Inmaculada Calvo Penades, Ruben Cuttica, Hans-Iko Huppertz, Rik Joos, Yukiko Kimura, Diana Milojevic, Margalit Rosenkranz, Kenneth Schikler, Tamas Constantin and Carine Wouters

Biologics in refractory myositis: experience in juvenile vs. adult myositis; part II: emerging biologic and other therapies on the horizon

The idiopathic inflammatory myopathies (IIM) until recently have been considered a heterogeneous broad group of six autoimmune muscle diseases. Initially, autoantibodies in IIM (including JDM) and CD8+ T cell-...

Authors: Anjali Patwardhan and Charles H. Spencer

RAS-associated Autoimmune Leukoproliferative disease (RALD) manifested with early-onset SLE-like syndrome: a case series of RALD in Chinese children

Primary immunodeficiency diseases (PIDs) patients may show systemic lupus erythematosus (SLE)-like autoimmunity disorders, such as cytopenias, as well as polyarthritis, which leads to concerns of misdiagnosis....

Authors: Wei Wang, Yu Zhou, Linqing Zhong, Lin Wang, Xiaoyan Tang, Mingsheng Ma, Ji Li and Hongmei Song

A UK study: vocational experiences of young adults with juvenile idiopathic arthritis

Authors: Laura E. Lunt, Ailsa Bosworth, Matthew Bezzant, Karen Walker-Bone, Kimme L. Hyrich, Wendy Thomson, Janet E. McDonagh and Suzanne M. M. Verstappen

The time option of IVIG treatment is associated with therapeutic responsiveness and coronary artery abnormalities but not with clinical classification in the acute episode of Kawasaki disease

In the last decade, incomplete Kawasaki disease (KD), intravenous immunoglobulin (IVIG) non-response and coronary artery abnormalities (CAA) have experienced the increasing trends in China. In addition, the en...

Authors: Sama Samadli, Fei Fei Liu, Goshgar Mammadov, Jing Jing Wang, Hui Hui Liu, Yang Fang Wu, Huang Huang Luo, Yue Wu, Wei Xia Chen, Dong Dong Zhang, Wei Wei and Peng Hu

Whole exome sequencing in childhood-onset lupus frequently detects single gene etiologies

Systemic lupus erythematosus (SLE) comprise a diverse range of clinical manifestations. To date, more than 30 single gene causes of lupus/lupus like syndromes in humans have been identified. In the clinical se...

Authors: Irit Tirosh, Shiri Spielman, Ortal Barel, Reut Ram, Tali Stauber, Gideon Paret, Marina Rubinsthein, Itai M. Pessach, Maya Gerstein, Yair Anikster, Rachel Shukrun, Adi Dagan, Katerina Adler, Ben Pode-Shakked, Alexander Volkov, Marina Perelman…

Demographic, clinical, and treatment characteristics of the juvenile primary fibromyalgia syndrome cohort enrolled in the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry

To describe the demographic, clinical, and treatment characteristics of youth diagnosed with juvenile primary fibromyalgia syndrome (JPFS) who are seen in pediatric rheumatology clinics.

Authors: Jenifer E. Weiss, Kenneth N. Schikler, Alexis D. Boneparth and Mark Connelly

A prediction rule for lack of achievement of inactive disease with methotrexate as the sole disease-modifying antirheumatic therapy in juvenile idiopathic arthritis

To investigate the frequency of achievement of inactive disease (ID) in children with juvenile idiopathic arthritis (JIA) treated with methotrexate (MTX) as the sole disease-modifyng antirheumatic (DMARD) ther...

Authors: Cecilia Bava, Federica Mongelli, Angela Pistorio, Marta Bertamino, Giulia Bracciolini, Sara Dalprà, Sergio Davì, Stefano Lanni, Valentina Muratore, Silvia Pederzoli, Silvia Rosina, Benedetta Schiappapietra, Chiara Suffia, Giulia Varnier, Sara Verazza, Gabriella Giancane…

Chronic non-bacterial osteomyelitis: a comparative study between children and adults

To compare clinical presentation, diagnostic and treatment strategies, and outcome between pediatric and adult patients with chronic non-bacterial osteomyelitis (CNO).

Authors: Andrea Skrabl-Baumgartner, Peter Singer, Theresa Greimel, Gregor Gorkiewicz and Josef Hermann

Physician practices for withdrawal of medications in inactive systemic juvenile arthritis, Childhood Arthritis and Rheumatology Research Alliance (CARRA) survey

We describe a Childhood Arthritis and Rheumatology Research Alliance (CARRA) survey of North American pediatric rheumatologists that assesses physician attitudes on withdrawal of medications in systemic juveni...

Authors: Susan Shenoi, Kabita Nanda, Grant S. Schulert, John F. Bohnsack, Ashley M. Cooper, Bridget Edghill, Miriah C. Gillispie-Taylor, Baruch Goldberg, Olha Halyabar, Thomas G. Mason, Tova Ronis, Rayfel Schneider, Richard K. Vehe and Karen Onel

TNF blockade contributes to restore lipid oxidation during exercise in children with juvenile idiopathic arthritis

Children with juvenile idiopathic arthritis (JIA) have impaired physical abilities. TNF-α plays a crucial role in this pathogenesis, but it is also involved in the use of lipids and muscle health. Objective of...

Authors: Emmanuelle Rochette, Pierre Bourdier, Bruno Pereira, Eric Doré, Anthony Birat, Sébastien Ratel, Stéphane Echaubard, Pascale Duché and Etienne Merlin

Association between maternal age and outcomes in Kawasaki disease patients

The etiology of Kawasaki disease (KD) is still unknown; perinatal factors may have role with few studies. This study was aim to survey the perinatal factors and clinical outcome of KD, including coronary arter...

Authors: Wei-Dong Huang, Yu-Ting Lin, Zi-Yu Tsai, Ling-Sai Chang, Shih-Feng Liu, Yi-Ju Lin and Ho-Chang Kuo

Physical activity and health-related quality of life in chronic non-bacterial osteomyelitis

Chronic non-bacterial osteomyelitis (CNO) is an autoinflammatory disorder of the skeletal system of yet unknown etiology. Patients present with local bone pain and inflammation and - to our experience - often ...

Authors: Nentwich Julia, Ruf Katharina, Girschick Hermann, Holl-Wieden Annette, Morbach Henner, Hebestreit Helge and Hofmann Christine

Participation in school and physical education in juvenile idiopathic arthritis in a Nordic long-term cohort study

The aim of the study was to describe school attendance and participation in physical education in school among children with juvenile idiopathic arthritis (JIA).

Authors: Ellen Nordal, Veronika Rypdal, Ellen Dalen Arnstad, Kristiina Aalto, Lillemor Berntson, Maria Ekelund, Anders Fasth, Mia Glerup, Troels Herlin, Susan Nielsen, Suvi Peltoniemi, Marek Zak, Nils Thomas Songstad and Marite Rygg

Developing comparative effectiveness studies for a rare, understudied pediatric disease: lessons learned from the CARRA juvenile localized scleroderma consensus treatment plan pilot study

We designed and initiated a pilot comparative effectiveness study for juvenile localized scleroderma (jLS), for which there is limited evidence on best therapy. We evaluated the process we used, in relation to...

Authors: Suzanne C. Li, Robert C. Fuhlbrigge, Ronald M. Laxer, Elena Pope, Maria F. Ibarra, Katie Stewart, Thomas Mason II, Mara L. Becker, Sandy Hong, Fatma Dedeoglu, Kathryn S. Torok, C. Egla Rabinovich, Polly J. Ferguson, Marilynn Punaro, Brian M. Feldman, Tracy Andrews…

Retrospective review of immobilization vs. immediate resumption of activity in patients with Oligoarticular juvenile idiopathic arthritis following knee injections

Intraarticular corticosteroid injection (IACI) is one of the most common treatments in oligoarticular Juvenile Idiopathic Arthritis (JIA). Activity recommendations following injection vary, as there are no pub...

Authors: Elaine R. Flanagan, Heather Benham, Janet Figueroa, Janille Diaz, Jenna Tress and David D. Sherry

Plasma exosomes from children with juvenile dermatomyositis are taken up by human aortic endothelial cells and are associated with altered gene expression in those cells

The pathology of juvenile dermatomyositis (JDM) is characterized by prominent vessel wall and perivascular inflammation. This feature of the disease has remained unexplained and under-investigated. We have hyp...

Authors: Kaiyu Jiang, Rie Karasawa, Zihua Hu, Yanmin Chen, Lucy Holmes, Kathleen M. O’Neil and James N. Jarvis

Defining outcome measures in juvenile idiopathic arthritis associated uveitis by a systematic review analysis: do we need a consensus?

Juvenile Idiopathic Arthritis associated Uveitis (JIA-U) represents its most frequent extra-articular manifestation and the main cause of childhood uveitis in in developed countries. The broad variety of outco...

Authors: Greta Mastrangelo, Ivan Foeldvari, Jordi Anton and Gabriele Simonini

Testicular ischemia in deficiency of adenosine deaminase 2 (DADA2)

Deficiency of adenosine deaminase 2 (DADA2) is a rare autosomal recessive autoinflammatory condition. Recognised features include vasculitis predominantly affecting medium sized vessels, livedoid skin rash, ce...

Authors: Katherine Clarke, Cathy Campbell, Ebun Omoyinmi, Ying Hong, Muthana Al Obaidi, Neil Sebire and Paul A. Brogan

Whole exome sequencing approach to childhood onset familial erythrodermic psoriasis unravels a novel mutation of CARD14 requiring unusual high doses of ustekinumab

Autosomal dominant gain of function mutations in caspase recruitment domain family member 14 (CARD14) is a rare condition associated with plaque-type psoriasis, generalized pustular psoriasis, palmoplantar pus...

Authors: S. Signa, E. Campione, M. Rusmini, S. Chiesa, A. Grossi, A. Omenetti, R. Caorsi, G. M. Viglizzo, M. Galluzzo, L. Bianchi, M. Talamonti, A. Orlandi, A. Martini, I. Ceccherini and M. Gattorno

Scoping review of biological treatment of deficiency of interleukin-36 receptor antagonist (DITRA) in children and adolescents

Deficiency of interleukin-36 receptor antagonist (DITRA) is a life threatening monogenic autoinflammatory disease caused by loss of function mutations in the IL36RN gene. Affected patients develop recurrent episo...

Authors: Toni Hospach, Fabian Glowatzki, Friederike Blankenburg, Dennis Conzelmann, Christian Stirnkorb, Chris Sandra Müllerschön, Peter von den Driesch, Lisa Maria Köhler, Meino Rohlfs, Christoph Klein and Fabian Hauck

Retrospective case series describing the efficacy, safety and cost-effectiveness of a vial-sharing programme for canakinumab treatment for paediatric patients with cryopyrin-associated periodic syndrome

Cryopyrin-associated periodic syndrome (CAPS) is a rare autoinflammatory disease, caused by gain of function mutation in NLRP3 resulting in excess production of interleukin-1 (IL-1). Canakinumab is a human monocl...

Authors: Abdulkadir A. Elmi, Karen Wynne, Iek L. Cheng, Despina Eleftheriou, Helen J. Lachmann, Philip N. Hawkins and Paul Brogan

Efficacy of pamidronate in children with chronic non-bacterial osteitis using whole body MRI as a marker of disease activity

To study the response to pamidronate using whole body magnetic resonance imaging (WB-MRI) in children with chronic non-bacterial osteitis (CNO) in a tertiary health centre.

Authors: C. S. Bhat, M. Roderick, E. S. Sen, A. Finn and A. V. Ramanan

A low-frequency IL4R locus variant in Japanese patients with intravenous immunoglobulin therapy-unresponsive Kawasaki disease

Kawasaki disease (KD) is a systemic vasculitis which may be associated with coronary artery aneurysms. A notable risk factor for the development of coronary artery aneurysms is resistance to intravenous immuno...

Authors: Yuji Amano, Yohei Akazawa, Jun Yasuda, Kazuhisa Yoshino, Katsuhiko Kojima, Norimoto Kobayashi, Satoshi Matsuzaki, Masao Nagasaki, Yosuke Kawai, Naoko Minegishi, Noriko Ishida, Noriko Motoki, Akira Hachiya, Yozo Nakazawa, Masayuki Yamamoto, Kenichi Koike…

Heavy metals in fish and its association with autoimmunity in the development of juvenile idiopathic arthritis: a prospective birth cohort study

The etiology of Juvenile Idiopathic Arthritis (JIA) is poorly understood. The purpose of this study was to examine the possible influence of early nutrition on later development of JIA.

Authors: Erik Kindgren, Carlos Guerrero-Bosagna and Johnny Ludvigsson

Research priorities in childhood-onset lupus: results of a multidisciplinary prioritization exercise

Childhood-onset systemic erythematosus lupus (cSLE) is characterized by more severe disease, widespread organ involvement and higher mortality compared to adult-onset SLE. However, cSLE is largely underfunded ...

Authors: Stacy P. Ardoin, R Paola Daly, Lyna Merzoug, Karin Tse, Kaveh Ardalan, Lisa Arkin, Andrea Knight, Tamar Rubinstein, Natasha Ruth, Scott E. Wenderfer and Aimee O. Hersh

Hallmark trials in ANCA-associated vasculitis (AAV) for the pediatric rheumatologist

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) refers to a complex group of systemic vasculitides that are characterized by primary small-to-medium sized blood vessel inflammation with...

Authors: Jennifer J. Y. Lee, Alhanouf Alsaleem, Grace P. K. Chiang, Elizaveta Limenis, Watchareewan Sontichai, Rae S. M. Yeung, Jonathan Akikusa and Ronald M. Laxer

Being on the juvenile dermatomyositis rollercoaster: a qualitative study

Juvenile Dermatomyositis is a rare, potentially life-threatening condition with no known cure. There is no published literature capturing how children and young people feel about their condition, from their pe...

Authors: Polly Livermore, Suzanne Gray, Kathleen Mulligan, Jennifer N. Stinson, Lucy R. Wedderburn and Faith Gibson

Early administration of adalimumab for paediatric uveitis due to Behçet’s disease

Behçet’s disease is a chronic inflammatory multisystem disorder that is characterised by oral and/or genital ulcerations as well as intraocular inflammation. Recurrent retinal vasoocclusive episodes and macula...

Authors: Tomona Hiyama, Yosuke Harada, Takehiko Doi and Yoshiaki Kiuchi

Relapsing periodic arthritis, palindromic rheumatism and MEFV gene-related variants alleles in children

Relapsing periodic arthritis is a general term used for a group of diseases with recurring and periodic nature, in which the joints are intermittently involved. The aim of this study is to evaluation of the po...

Authors: Farhad Salehzadeh, Manuchehr Barak and Roghayeh Nematdoust Haghi

Efficacy and safety of duloxetine versus placebo in adolescents with juvenile fibromyalgia: results from a randomized controlled trial

Currently, there are no medications approved for the treatment of juvenile fibromyalgia (JFM). We evaluated the safety and efficacy of duloxetine 30/60 mg once daily (QD) versus placebo in adolescents with JFM.

Authors: Himanshu P. Upadhyaya, Lesley M. Arnold, Karla Alaka, Meihua Qiao, David Williams and Renata Mehta

A delayed diagnosis of eosinophilic granulomatosis with polyangiitis complicated with extensive artery occlusion of lower extremities in children: case report and literature review

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis in children. A delayed or missed diagnosis of pediatric EGPA is common, owing to the atypical clinical manifestation and limit...

Authors: Xiaoliang Liu, Lin Wang, Kaiyu Zhou, Yimin Hua, Xiaoqing Shi and Chuan Wang

An unusual presentation of purine nucleoside phosphorylase deficiency mimicking systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome

Systemic juvenile idiopathic arthritis (sJIA) is an inflammatory condition that presents with fever, rash and arthritis. At onset systemic features are predominant and the diagnosis may be a challenge. Seconda...

Authors: Alessia Arduini, Emiliano Marasco, Giulia Marucci, Manuela Pardeo, Antonella Insalaco, Ivan Caiello, Gian Marco Moneta, Giusi Prencipe, Fabrizio De Benedetti and Claudia Bracaglia

The PRINTO evidence-based proposal for glucocorticoids tapering/discontinuation in new onset juvenile dermatomyositis patients

Prednisone (PDN) in juvenile dermatomyositis (JDM), alone or in association with other immunosuppressive drugs, namely methotrexate (MTX) and cyclosporine (CSA), represents the first-line treatment option for ...

Authors: Gabriella Giancane, Claudio Lavarello, Angela Pistorio, Sheila K. Oliveira, Francesco Zulian, Ruben Cuttica, Michel Fischbach, Bo Magnusson, Serena Pastore, Roberto Marini, Silvana Martino, Anne Pagnier, Christine Soler, Valda Staņēvicha, Rebecca Ten Cate, Yosef Uziel…

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