Articles

Tocilizumab modifies clinical and laboratory features of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

This study aimed to determine the influence of tocilizumab (TCZ) in modifying the clinical and laboratory features of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (s...

Authors: Masaki Shimizu, Mao Mizuta, Nami Okamoto, Takahiro Yasumi, Naomi Iwata, Hiroaki Umebayashi, Yuka Okura, Noriko Kinjo, Tomohiro Kubota, Yasuo Nakagishi, Kenichi Nishimura, Mariko Mohri, Masato Yashiro, Junko Yasumura, Hiroyuki Wakiguchi and Masaaki Mori

Persistent osteoarticular pain in children: early clinical and laboratory findings suggestive of acute lymphoblastic leukemia (a multicenter case-control study of 147 patients)

The aim of this study was to identify early clinical and laboratory features that distinguish acute lymphoblastic leukemia (ALL) from juvenile idiopathic arthritis (JIA) in children presenting with persistent ...

Authors: Mathilde Louvigné, Josué Rakotonjanahary, Laurence Goumy, Aude Tavenard, Jean-François Brasme, Fanny Rialland, André Baruchel, Marie-Françoise Auclerc, Véronique Despert, Marie Desgranges, Sylvie Jean, Albert Faye, Ulrich Meinzer, Mathie Lorrot, Chantal Job-Deslandre, Brigitte Bader-Meunier…

Chronic idiopathic musculoskeletal pain in youth: a qualitative study

Chronic musculoskeletal pain (MSP) is frequent in adolescents and has major medical and social consequences. In many cases, when no cause has been clearly established, this pain may be considered to be chronic...

Authors: Hervé Lefèvre, Alexandra Loisel, Brigitte Bader Meunier, Chantal Deslandre, Noémie Lemoine, Marie Rose Moro, Pierre Quartier and Jonathan Lachal

Patient and caregiver engagement in research: factors that influence co-enrollment in research

Recruitment of pediatric participants in studies is difficult due to the vulnerability of this population and the scarcity of certain conditions. Co-enrolling in multiple studies is a strategy that may help ov...

Authors: Leanne K. Elliott, Herman Bami, Maxwell J. Gelkopf, Ryan C. Yee, Brian M. Feldman and Y. Ingrid Goh

Is fibrodysplasia ossificans progressiva an interleukin-1 driven auto-inflammatory syndrome?

Fibrodysplasia ossificans progressiva (FOP) is the most catastrophic form of heterotopic ossification, due to ongoing intracellular signaling through the bone morphogenic protein pathway. The paroxysmal appear...

Authors: Ruby Haviv, Veronica Moshe, Fabrizio De Benedetti, Giusi Prencipe, Noa Rabinowicz and Yosef Uziel

Orofacial pain in juvenile idiopathic arthritis is associated with stress as well as psychosocial and functional limitations

The aim of this study was to investigate relations between psychosocial factors, signs and symptoms of orofacial pain and jaw dysfunction in patients with juvenile idiopathic arthritis (JIA).

Authors: Alexandra Dimitrijevic Carlsson, Kerstin Wahlund, Erik Kindgren, Thomas Skogh, Carin Starkhammar Johansson and Per Alstergren

Arthritis in children with LRBA deficiency – case report and literature review

Lipopolysaccharide (LPS)-responsive and beige like anchor (LRBA) deficiency is categorized as a subtype of common variable immune deficiency (CVID). A growing number of case reports and cohorts reveal a broad ...

Authors: Rotem Semo Oz and Melissa S. Tesher

Oral health and plaque microbial profile in juvenile idiopathic arthritis

The oral microbiota has been implicated in the pathogenesis of rheumatoid arthritis through activation of mucosal immunity. This study tested for associations between oral health, microbial communities and juv...

Authors: Sriharsha Grevich, Peggy Lee, Brian Leroux, Sarah Ringold, Richard Darveau, Gretchen Henstorf, Joel Berg, Amy Kim, Elizabeth Velan, Joseph Kelly, Camille Baltuck, Anne Reeves, Hannah Leahey, Kyle Hager, Mitchell Brittnacher, Hillary Hayden…

Health-related quality of life during early aggressive treatment in patients with polyarticular juvenile idiopathic arthritis: results from randomized controlled trial

Juvenile Idiopathic Arthritis (JIA) may cause significant impairment in health-related quality of life (HrQoL), despite effective therapies. The aim of this study was to assess HrQoL during first-year treatmen...

Authors: Maarit Tarkiainen, Pirjo Tynjälä, Paula Vähäsalo, Liisa Kröger, Kristiina Aalto and Pekka Lahdenne

Proceedings of the 2019 Childhood Arthritis and Rheumatology Research Alliance (CARRA) Annual Scientific Meeting

Authors:

Effectiveness of long-term infliximab use and impact of treatment adherence on disease control in refractory, non-infectious pediatric uveitis

Refractory non-infectious uveitis is a serious condition that leads to ocular complications and vision loss and requires effective systemic treatment to control disease. The effectiveness of long-term inflixim...

Authors: Virginia Miraldi Utz, Sabrina Bulas, Sarah Lopper, Matthew Fenchel, Ting Sa, Mitul Mehta, Daniel Ash, Daniel J. Lovell and Adam H. Kaufman

Predictive value of serum procalcitonin for both initial and repeated immunoglobulin resistance in Kawasaki disease: a prospective cohort study

Intravenous immunoglobulin (IVIG) resistance prediction is one pivotal topic of interests in Kawasaki disease (KD) since those patients with KD resistant to IVIG might improve of an early-intensified therapy. ...

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

Comparison of second-line therapy in IVIg-refractory Kawasaki disease: a systematic review

Evidence remains contradictory regarding second-line therapy in patients with Kawasaki disease (KD) refractory to initial intravenous immunoglobulin (IVIg). The objective of this study aims to evaluate the eff...

Authors: Courtney B. Crayne, Chace Mitchell and Timothy Beukelman

Pediatric systemic lupus erythematosus patients in South Africa have high prevalence and severity of cardiac and vascular manifestations

Pediatric onset of systemic lupus erythematosus (SLE) is associated with major organ involvement, and African patients tend to develop more aggressive disease than patients of European descent. Although cardio...

Authors: Michael J. Harrison, Liesl J. Zühlke, Laura B. Lewandowski and Christiaan Scott

Henoch-Schönlein Purpura in children: not only kidney but also lung

Henoch-Schönlein Purpura (HSP) is the most common vasculitis of childhood and affects the small blood vessels. Pulmonary involvement is a rare complication of HSP and diffuse alveolar hemorrhage (DAH) is the m...

Authors: Giada Maria Di Pietro, Massimo Luca Castellazzi, Antonio Mastrangelo, Giovanni Montini, Paola Marchisio and Claudia Tagliabue

Can quantitative MRI be used in the clinical setting to quantify the impact of intra-articular glucocorticoid injection on synovial disease activity in juvenile idiopathic arthritis?

Juvenile idiopathic arthritis (JIA), the most common chronic rheumatic disease of childhood, is characterised by synovitis. Clinical assessments of synovitis are imperfect, relying on composite and indirect me...

Authors: Joshua L. Bennett, Amanda Wood, Nicola Smith, Ravi Mistry, Karen Allen, Sharmila Jandial, John D. Tuckett, S. Claire Gowdy, Helen E. Foster, Flora McErlane and Kieren G. Hollingsworth

Pain, functional disability, and their Association in Juvenile Fibromyalgia Compared to other pediatric rheumatic diseases

Severe pain and impairments in functioning are commonly reported for youth with juvenile fibromyalgia. The prevalence and impact of pain in other diseases commonly managed in pediatric rheumatology comparative...

Authors: Mark Connelly and Jennifer E. Weiss

Juvenile systemic lupus erythematosus presenting as pancarditis

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with marked variation in its clinical presentation. Juvenile SLE (jSLE) accounts for 15–20% of all cases and is diagnosed when SLE manifests b...

Authors: D. O’Leary, C. O’Connor, L. Nertney, E. J. MacDermott, D. Mullane, O. Franklin and O. G. Killeen

Complexity in unclassified auto-inflammatory disease: a case report illustrating the potential for disease arising from the allelic burden of multiple variants

Despite recent advances in the diagnosis and understanding of many autoinflammatory diseases, there are still a great number of patients with phenotypes that do not fit any clinically- and/or genetically-defin...

Authors: Lori B. Tucker, Lovro Lamot, Iwona Niemietz, Brian K. Chung, David A. Cabral, Kristin Houghton, Ross E. Petty, Kimberly A. Morishita, Gillian I. Rice, Stuart E. Turvey, William T. Gibson and Kelly L. Brown

Biomarkers associating endothelial dysregulation in pediatric-onset systemic lupus erythematous

Endothelium is a key element in the regulation of vascular homeostasis and its alteration can lead to the development of vascular diseases. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease w...

Authors: Wan-Fang Lee, Chao-Yi Wu, Huang-Yu Yang, Wen-I Lee, Li-Chen Chen, Liang-Shiou Ou and Jing-Long Huang

Infrared thermography in children: a reliable tool for differential diagnosis of peripheral microvascular dysfunction and Raynaud’s phenomenon?

Infrared Thermography (IRT) has been used for over 30 years in the assessment of Raynaud Phenomenon (RP) and other peripheral microvascular dysfunctions in adults but, to date, very little experience is availa...

Authors: Giorgia Martini, Michela Cappella, Roberta Culpo, Fabio Vittadello, Monica Sprocati and Francesco Zulian

Same but different? A thematic analysis on adalimumab biosimilar switching among patients with juvenile idiopathic arthritis

Biologic medications have dramatically enhanced the treatment of many chronic paediatric inflammatory conditions. Their high cost is a factor that prohibits their broader use. Cheaper generic versions, or bios...

Authors: William D. Renton, Helen Leveret, Catherine Guly, Heather Smee, Jamie Leveret and Athimalaipet V. Ramanan

Proposal for a definition for response to treatment, inactive disease and damage for JIA associated uveitis based on the validation of a uveitis related JIA outcome measures from the Multinational Interdisciplinary Working Group for Uveitis in Childhood (MIWGUC)

JIA-associated uveitis (JIAU) is a serious, sight-threatening disease with significant long-term complications and risk of blindness, even with improved contemporary treatments. The MIWGUC was set up in order ...

Authors: Ivan Foeldvari, Jens Klotsche, Gabriele Simonini, Clive Edelsten, Sheila T. Angeles-Han, Regitze Bangsgaard, Joke de Boer, Gabriele Brumm, Rosa Bou Torrent, Tamas Constantin, Cinzia DeLibero, Jesus Diaz, Valeria Maria Gerloni, Margarida Guedes, Arnd Heiligenhaus, Kaisu Kotaniemi…

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