Articles

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

Reliability of ultrasonography to detect inflammatory lesions and structural damage in juvenile idiopathic arthritis

Musculoskeletal Ultrasonography (MSUS) is an important tool for the clinical assessment in Juvenile Idiopathic Arthritis (JIA). The objective of this study was to evaluate the reliability of MSUS to detect ele...

Authors: Lucio Ventura-Ríos, Enrique Faugier, Laura Barzola, L. B. De la Cruz-Becerra, Guadalupe Sánchez-Bringas, Andrés Rodríguez García, Rocío Maldonado, Johannes Roth and Cristina Hernández-Díaz

Dutch juvenile idiopathic arthritis patients, carers and clinicians create a research agenda together following the James Lind Alliance method: a study protocol

Research on Juvenile Idiopathic Arthritis (JIA) should support patients, caregivers/parents (carers) and clinicians to make important decisions in the consulting room and eventually to improve the lives of pat...

Authors: Casper G. Schoemaker, Wineke Armbrust, Joost F. Swart, Sebastiaan J. Vastert, Jorg van Loosdregt, Anouk Verwoerd, Caroline Whiting, Katherine Cowan, Wendy Olsder, Els Versluis, Rens van Vliet, Marlous J. Fernhout, Sanne L. Bookelman, Jeannette Cappon, J. Merlijn van den Berg, Ellen Schatorjé…

Risk factors for damage in childhood-onset systemic lupus erythematosus in Asians: a case control study

Accumulated damage is an important prognostic factor in systemic lupus erythematous. However, the pattern of disease damage and its risk factors have not been well studied in childhood-onset systemic lupus ery...

Authors: Jacqueline K. K. Sit and Winnie K. Y. Chan

Progressive pseudorheumatoid dysplasia with new-found gene mutation of Wntl inducible signaling pathway protein 3

As one kind of osteochondrodysplasia, progressive pseudorheumatoid dysplasia (PPD) is also known as spondyloepiphyseal dysplasia tarda with progressive arthropathy or arthropathy progressive pseudorheumatoid o...

Authors: Wenji Chen, Shiyan Mo, Gui Luo, Yanyan Wang, Xiaohu Deng, Jian Zhu and Wei Zhao

A case of Henoch-Schonlein Purpura with dilated coronary arteries

Henoch-Schonlein Purpura (HSP) is one of the most common vasculitides of childhood, with 10–20 cases per 100,000 children. It frequently occurs following an infectious trigger and involves IgA and C3 depositio...

Authors: Jessica L. Bloom, Jeffrey R. Darst, Lori Prok and Jennifer B. Soep

Correction to: Health-related quality of life in girls and boys with juvenile idiopathic arthritis: self- and parental reports in a cross-sectional study

Following publication of the original article [1], the authors reported an error in the data of their article: one girl was by mistake scored as a boy. The authors have made new analyses of the corrected data....

Authors: Veronica Lundberg, Viveca Lindh, Catharina Eriksson, Solveig Petersen and Eva Eurenius

Proceedings of the 25th European Paediatric Rheumatology Congress (PReS 2018)

Authors:

Outcomes of non-infectious Paediatric uveitis in the era of biologic therapy

There is a paucity of data on the ocular outcomes in paediatric non-infectious uveitis since the introduction of the biologic agents. The purpose of this study was to outline the clinical characteristics of ch...

Authors: Megan Cann, Athimalaipet V. Ramanan, Andrew Crawford, Andrew D. Dick, Sarah L. N. Clarke, Fatima Rashed and Catherine M. Guly

Successful implementation of a clinical transition pathway for adolescents with juvenile-onset rheumatic and musculoskeletal diseases

In 2008 a clinical transition pathway for young people with juvenile-onset rheumatic and musculoskeletal diseases (jRMD) aiming at improving transitional care was instituted. Historical data on drop-out rate i...

Authors: Margot Walter, Sylvia Kamphuis, Philomine van Pelt, Annemarie de Vroed and Johanna M. W. Hazes

Unusual case of chronic recurrent multifocal osteomyelitis

Chronic recurrent multifocal osteomyelitis (CRMO) is a rare auto-inflammatory bone disorder that primarily affects young girls, with a mean age of 10 years at onset. Generally, it is a self-limited disease. Ho...

Authors: Ausra Snipaitiene, Rima Sileikiene, Justina Klimaite, Edita Jasinskiene, Rimantas Uktveris and Lina Jankauskaite

Kawasaki disease: abnormal initial echocardiogram is associated with resistance to IV Ig and development of coronary artery lesions

Kawasaki disease (KD) is an acute febrile systemic vasculitis that affects small and medium blood vessels. Intensified treatments for the most severely affected patients have been proposed recently, and the ea...

Authors: Dima Chbeir, Jean Gaschignard, Ronan Bonnefoy, Constance Beyler, Isabelle Melki, Albert Faye and Ulrich Meinzer

Orofacial symptoms and oral health-related quality of life in juvenile idiopathic arthritis: a two-year prospective observational study

Little is known about the chronicity of orofacial symptoms and how this influences the oral health-related quality of life in juvenile idiopathic arthritis (JIA). Therefore, our objectives were to study the lo...

Authors: Hanna Rahimi, Marinka Twilt, Troels Herlin, Lynn Spiegel, Thomas Klit Pedersen, Annelise Küseler and Peter Stoustrup

Methotrexate in juvenile idiopathic arthritis: advice and recommendations from the MARAJIA expert consensus meeting

Conventional pharmacological therapies for the treatment of juvenile idiopathic arthritis (JIA) consist of non-biological, disease-modifying antirheumatic drugs, among which methotrexate (MTX) is the most comm...

Authors: Giovanna Ferrara, Greta Mastrangelo, Patrizia Barone, Francesco La Torre, Silvana Martino, Giovanni Pappagallo, Angelo Ravelli, Andrea Taddio, Francesco Zulian and Rolando Cimaz