Articles

Correction to: Biomarkers associating endothelial dysregulation in pediatric-onset systemic lupus erythematous

Following publication of the original article [1], we have been notified that the colour representation of the graph is not correct in Figure 6 legend.

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

Consensus protocols for the diagnosis and management of the hereditary autoinflammatory syndromes CAPS, TRAPS and MKD/HIDS: a German PRO-KIND initiative

Rare autoinflammatory diseases (AIDs) including Cryopyrin-Associated Periodic Syndrome (CAPS), Tumor Necrosis Receptor-Associated Periodic Syndrome (TRAPS) and Mevalonate Kinase Deficiency Syndrome (MKD)/ Hype...

Authors: Sandra Hansmann, Elke Lainka, Gerd Horneff, Dirk Holzinger, Nikolaus Rieber, Annette F. Jansson, Angela Rösen-Wolff, Gabi Erbis, Martina Prelog, Juergen Brunner, Susanne M. Benseler and Jasmin B. Kuemmerle-Deschner

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

Following publication of the original article [1], we have been notified that the corresponding author’s given name is spelled incorrectly. The given name, thus, should be as follows:

Authors: Mark Connelly and Jennifer E. Weiss

Adolescent Sjogren’s syndrome presenting as psychosis: a case series

Neurological involvement has been reported in up to 80% of adults with Primary Sjogren’s syndrome (pSS) with psychiatric abnormalities including anxiety, depression, and cognitive dysfunction being common. Psy...

Authors: Erin K. Hammett, Cristina Fernandez-Carbonell, Courtney Crayne, Alexis Boneparth, Randy Q. Cron and Suhas M. Radhakrishna

Correction to: 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)

Following publication of the original article [1], we have been notified that the author Joan Calzada should not have been included to the team of authors. The authors’ team, thus, should be as follows:

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…

Children’s fear of needle injections: a qualitative study of training sessions for children with rheumatic diseases before home administration

Treatment of rheumatic diseases in children often includes long-term needle injections, which represent a risk for refusing medication based on potential needle-fear. How nurses manage children’s fear and pain...

Authors: Kari Sørensen, Helge Skirbekk, Gunnvald Kvarstein and Hilde Wøien

Pharmacological conditioning for juvenile idiopathic arthritis: a potential solution to reduce methotrexate intolerance

Methotrexate (MTX) therapy has proven to be a successful and safe treatment for Juvenile Idiopathic Arthritis (JIA). Despite the high efficacy rates of MTX, treatment outcomes are often complicated by burdenso...

Authors: Rosanne M. Smits, Dieuwke S. Veldhuijzen, Henriet van Middendorp, Petra C. E. Hissink Muller, Wineke Armbrust, Elizabeth Legger, Nico M. Wulffraat and Andrea W. M. Evers

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

Following publication of the original article [1], we have been notified that the authors’ first names and last names are presented in wrong order. The presentation of names, thus, should be as follows:

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

Quantitative analysis of facial asymmetry based on three-dimensional photography: a valuable indicator for asymmetrical temporomandibular joint affection in juvenile idiopathic arthritis patients?

Juvenile idiopathic arthritis (JIA) can cause osseous deformity in the temporomandibular joint (TMJ) and may impair mandibular growth. This study aimed to evaluate whether facial asymmetry determined clinicall...

Authors: Joëlle M. Bernini, Christian J. Kellenberger, Martina Eichenberger, Theodore Eliades, Spyridon N. Papageorgiou and Raphael Patcas

Pregnancy outcomes and perinatal complications of Asian mothers with juvenile idiopathic arthritis – a case-control registry study

In order to provide juvenile idiopathic arthritis (JIA) patients with better pre-conceptional and prenatal counselling, we investigated the obstetrical and neonatal outcomes among women with Asian descent.

Authors: Shang Jun Zhang-Jian, Huang-Yu Yang, Meng-Jun Chiu, I-Jun Chou, Chang-Fu Kuo, Jing-Long Huang, Kuo-Wei Yeh and Chao-Yi Wu

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

Following publication of the original article [1], we have been notified that the corresponding author’s given name is spelled incorrectly.

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

The use of S100 proteins testing in juvenile idiopathic arthritis and autoinflammatory diseases in a pediatric clinical setting: a retrospective analysis

Serum phagocyte-derived alarmins S100A8/9 and S100A12 are considered useful for the assessment of inflammatory diseases. Our study evaluated the use of S100 proteins in a pediatric clinical setting for estimat...

Authors: Najla Aljaberi, Elena Tronconi, Grant Schulert, Alexei A. Grom, Daniel J. Lovell, Jennifer L. Huggins, Michael Henrickson and Hermine I. Brunner

Patients with enthesitis related arthritis show similar monocyte function pattern as seen in adult axial spondyloarthropathy

Axial SpA and Enthesitis related arthritis (ERA) patients show strong HLA-B27 association, gut dysbiosis, high toll like receptor (TLR)2 and 4 expression on monocytes, pro-inflammatory cytokine production and ...

Authors: Shruti Bhattacharya, Ramnath Misra and Amita Aggarwal

Patient and physician discordance of global disease assessment in juvenile dermatomyositis: findings from the Childhood Arthritis & Rheumatology Research Alliance Legacy Registry

Global disease activity scores (gVAS) capture patient or family (PF) and physician (MD) assessments of disease. This study sought to measure discordance between PF and MD global activity scores in juvenile der...

Authors: Heather Tory, David Zurakowski and Susan Kim

Pediatric chronic nonbacterial osteomyelitis of the mandible: Seattle Children’s hospital 22-patient experience

Studies evaluating treatment responses for chronic nonbacterial osteomyelitis (CNO) are lacking. We aimed to measure and compare response rates of medical treatments, time to response of medical treatments amo...

Authors: Austin Gaal, Matthew L. Basiaga, Yongdong Zhao and Mark Egbert

Development and validation of the CAREGIVERS questionnaire: multi-assessing the impact of juvenile idiopathic arthritis on caregivers

The primary caregiver is an important person in the life of patients with JIA. Their reactions depend on social, emotional and economic factors that affect the therapeutic alliance. Some generic instruments ha...

Authors: Marcia Daniela Torres-Made, Ingris Peláez-Ballestas, Fernando García-Rodríguez, Ana Victoria Villarreal-Treviño, Brenda de Jesús Fortuna-Reyna, Manuel Enrique de la O-Cavazos and Nadina Eugenia Rubio-Pérez

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