Articles

Changes over time in inflammatory and structural lesions at the sacroiliac joint in children with spondyloarthritis exposed and unexposed to tumor necrosis factor inhibitor

The objective of this work was to describe magnetic resonance imaging (MRI) changes over time in inflammatory and structural lesions at the sacroiliac joint (SIJ) in children with spondyloarthritis (SpA) expos...

Authors: Timothy G. Brandon, Rui Xiao, Rosemary G. Peterson, Nancy A. Chauvin, Michael L. Francavilla, David M. Biko, Dax G. Rumsey, Matthew L. Stoll and Pamela F. Weiss

Epidemiology and demographics of juvenile idiopathic arthritis in Africa and Middle East

Juvenile Idiopathic Arthritis (JIA) is a group of chronic heterogenous disorders that manifests as joint inflammation in patients aged <16 years. Globally, approximately 3 million children and young adults are...

Authors: Sulaiman M. Al-Mayouf, Muna Al Mutairi, Kenza Bouayed, Sara Habjoka, Djohra Hadef, Hala M. Lotfy, Cristiaan Scott, Elsadeg M. Sharif and Nouran Tahoun

Correction to: Evaluating the performance of ACR, SLICC and EULAR/ACR classification criteria in childhood onset systemic lupus erythematosus

Authors: Reem Abdwani, Eman Al Masroori, Eiman Abdullah, Safiya Al Abrawi and Ibrahim Al-Zakwani

A novel transition clinic structure for adolescent and young adult patients with childhood onset rheumatic disease improves transition outcomes

The transition of health care from Pediatric to Adult providers for adolescents and young adults with chronic disease is associated with poor outcomes. Despite the importance of this transition, over 80% of th...

Authors: Rebecca S. Overbury, Kelly Huynh, John Bohnsack, Tracy Frech and Aimee Hersh

Infection with SARS-CoV-2 causes flares in patients with juvenile idiopathic arthritis in remission or inactive disease on medication

Flares of juvenile idiopathic arthritis (JIA) have been described in the context of various infections. Flares of rheumatic diseases in adults have been described following infection with SARS-CoV-2 in several...

Authors: Boris Hügle, Manuela Krumrey-Langkammerer and Johannes-Peter Haas

Clinical characteristics and COVID-19 outcomes in a regional cohort of pediatric patients with rheumatic diseases

This study aimed to assess the baseline characteristics and clinical outcomes of coronavirus disease 2019 (COVID-19) in pediatric patients with rheumatic and musculoskeletal diseases (RMD) and identify the ris...

Authors: Daniel Clemente, Clara Udaondo, Jaime de Inocencio, Juan Carlos Nieto, Pilar Galán del Río, Antía García Fernández, Jaime Arroyo Palomo, Javier Bachiller-Corral, Juan Carlos Lopez Robledillo, Claudia Millán Longo, Leticia Leon, Lydia Abasolo and Alina Boteanu

Vasculitis in a patient with mevalonate kinase deficiency (MKD): a case report

Mevalonate kinase deficiency (MKD) is a rare autoinflammatory condition caused by biallelic loss-of-function (LOF) mutations in mevalonate kinase (MVK) gene encoding the enzyme mevalonate kinase. Patients with MK...

Authors: Ebun Omoyinmi, Dorota Rowczenio, Neil Sebire, Paul A. Brogan and Despina Eleftheriou

Tofacitinib effectiveness in Blau syndrome: a case series of Chinese paediatric patients

Blau syndrome (BS), a rare, autosomal-dominant autoinflammatory syndrome, is characterized by a clinical triad of granulomatous recurrent uveitis, dermatitis, and symmetric arthritis and associated with mutati...

Authors: Song Zhang, Zhe Cai, Xiaolan Mo and Huasong Zeng

Home-based exercise program for adolescents with juvenile dermatomyositis quarantined during COVID-19 pandemic: a mixed methods study

Exercise has been suggested to prevent deterioration of health-related quality of life (HRQL) and overall health in pediatric rheumatologic diseases during the COVID-19 pandemic. Herein we describe the effects...

Authors: Camilla Astley, Sofia Mendes Sieczkowska, Isabela Gouveia Marques, Bianca Pires Ihara, Livia Lindoso, Sofia Simão Martins Lavorato, Lucia Maria Arruda Campos, Rosa Maria Rodrigues Pereira, Adriana Maluf Elias, Nadia Emi Aikawa, Katia Kozu, Amanda Yuri Iraha, Tathiane Christine Franco, Hamilton Roschel, Ligia Bruni Queiroz, Guilherme Vanoni Polanczyk…

Explaining juvenile idiopathic arthritis to paediatric patients using illustrations and easy-to-read texts: improvement of disease knowledge and adherence to treatment

Juvenile idiopathic arthritis (JIA) is the leading chronic rheumatic disease in childhood. To achieve adherence to therapy, in-depth understanding of disease and treatment options are important.

Authors: Christiane Reiser, Nina A. Zeltner, Beatrix Rettenbacher, Petra Baumgaertner, Martina Huemer and Christian Huemer

Giant bilateral axillary artery aneurysms with left complete obstructive thrombus in intravenous immunoglobulin-sensitive Kawasaki disease: a case report

Kawasaki disease (KD) is a systemic vasculitis that predominantly affects medium-sized arteries. In addition to well-known coronary artery aneurysms (CAAs), peripheral systemic artery aneurysms (SAAs) have als...

Authors: Chen Chu, Lan He, Yi-xiang Lin, Li-ping Xie and Fang Liu

Proceedings of the 27th European Paediatric Rheumatology Congress (PReS 2021)

Authors:

How children with juvenile idiopathic arthritis view participation and communication in healthcare encounters: a qualitative study

Children report that they do not participate in their healthcare as much as they want, despite having the lawful right to form their own views and the right to express those views freely in all matters affecti...

Authors: Veronica Lundberg, Catharina Eriksson, Torbjörn Lind, Imelda Coyne and Anncristine Fjellman-Wiklund

A case report of a severe neonatal systemic vasculitis on the first day of life

Neonatal systemic vasculitis syndromes have been reported in infants born to mothers with systemic lupus erythematosus, Sjögren’s syndrome, Behҫet’s disease, cutaneous polyarteritis nodosa and anti-neutrophil ...

Authors: Stephanie Wong, Erkan Demirkaya and Roberta Berard

Intra-articular venous malformations of the knee: a diagnostic challenge

Intra-articular venous malformations (IAVM) are rare benign vascular anomalies that usually affect young patients and most common locate in the knee. The terminology of these lesions is still ill-defined, as t...

Authors: Federico Diomeda, Maria Santaniello, Giulia Bracciolini, Angelo Ravelli and Adele Civino

Economic impact of Juvenile Idiopathic Arthritis: a systematic review

Juvenile Idiopathic Arthritis (JIA) requires complex care that generate elevated costs, which results in a high economic impact for the family. The aim of this systematic review was to collect and cluster the ...

Authors: Fernando García-Rodríguez, Augusto Gamboa-Alonso, Sol Jiménez-Hernández, Lucero Ochoa-Alderete, Valeria Alejandra Barrientos-Martínez, Neri Alejandro Alvarez-Villalobos, Gabriela Andrea Luna-Ruíz, Ingris Peláez-Ballestas, Ana Victoria Villarreal-Treviño, Manuel Enrique de la O-Cavazos and Nadina Rubio-Pérez

Clinical and genetic characteristics of PSTPIP1-associated myeloid-related proteinemia inflammatory syndrome

To summarise the clinical and genetic characteristics of three children with PSTPIP1-associated myeloid-related proteinemia inflammatory (PAMI) syndrome.

Authors: Dan Zhang, Gaixiu Su, Yan Liu and Jianming Lai

IgA vasculitis nephritis clinical course and kidney biopsy – national study in children

The aim of the study was to investigate the relationship between the severity of typical clinical symptoms, severity of histopathological lesions in kidney biopsies in IgA vasculitis nephritis (IgAVN) and to p...

Authors: Małgorzata Mizerska-Wasiak, Agnieszka Turczyn, Karolina Cichoń-Kawa, Jadwiga Małdyk, Monika Miklaszewska, Dorota Drożdż, Beata Bieniaś, Przemysław Sikora, Magdalena Drożyńska-Duklas, Aleksandra Żurowska, Maria Szczepańska and Małgorzata Pańczyk-Tomaszewska

Knowledge about juvenile idiopathic arthritis-associated uveitis: more frequent reminders are associated with higher patient and family uveitis knowledge

Chronic anterior uveitis is a sight-threatening complication of juvenile idiopathic arthritis (JIA) and a primary contributor to long-term morbidity in people with JIA. Levels of knowledge about uveitis among ...

Authors: Ashley M. Cooper, Elaine R. Flanagan, Tova Ronis, Baruch Goldberg, Ashley K. Sherman, Chelsey Smith and Gary N. Holland

Utility of telemedicine in pediatric rheumatology during the COVID-19 pandemic

During the COVID-19 pandemic, telemedicine has provided an alternative to in-person visits for patients practicing social distancing and undergoing quarantine. During this time, there has been a rapid expansio...

Authors: Ashley Perdue, Charles Mullett, Amna Umer and Paul Rosen

Predictive value of Albumin-Bilirubin grade for intravenous immunoglobulin resistance in a large cohort of patients with Kawasaki disease: a prospective study

Intravenous immunoglobulin (IVIG) resistance prediction is one of the primary clinical issues and study hotspots in KD. This study aimed to prospectively investigate the value of albumin-bilirubin grade (ALBI)...

Authors: Yu Yan, Lina Qiao, Yimin Hua, Shuran Shao, Nanjun Zhang, Mei Wu, Lei Liu, Kaiyu Zhou, Xiaoliang Liu and Chuan Wang

JAK inhibitors: a potential treatment for JDM in the context of the role of interferon-driven pathology

Juvenile Idiopathic Inflammatory Myopathies (IIM) are a group of rare diseases that are heterogeneous in terms of pathology that can include proximal muscle weakness, associated skin changes and systemic invol...

Authors: Meredyth G. Ll Wilkinson, Claire T. Deakin, Charalampia Papadopoulou, Despina Eleftheriou and Lucy R. Wedderburn

Infections and antibiotics during fetal life and childhood and their relationship to juvenile idiopathic arthritis: a prospective cohort study

The aetiology of juvenile idiopathic arthritis (JIA) is poorly understood. It has been shown that use of antibiotics is associated with JIA. However, whether the association is due to increased occurrence of i...

Authors: Erik Kindgren and Johnny Ludvigsson

Tocilizumab for massive refractory pleural effusion in an adolescent with systemic lupus erythematosus

Pleural effusion in systemic lupus erythematous (SLE) is a common symptom, and recent studies demonstrated that IL-6 has a pivotal role in its pathogenesis.

Authors: Arianna De Matteis, Emanuela Sacco, Camilla Celani, Andrea Uva, Virginia Messia, Rebecca Nicolai, Manuela Pardeo, Fabrizio De Benedetti and Claudia Bracaglia

Reasons for canakinumab initiation among patients with periodic fever syndromes: a retrospective medical chart review from the United States

Although canakinumab has demonstrated efficacy in multiple trials in patients with periodic fever syndromes (PFS), the evidence on initiation of canakinumab among PFS patients in real world setting is not well...

Authors: Peter Hur, Kathleen G. Lomax, Raluca Ionescu-Ittu, Ameur M. Manceur, Jipan Xie, Jordan Cammarota, Raju Gautam, Navneet Sanghera, Nina Kim and Alexei A. Grom

Colchicine – an effective treatment for children with a clinical diagnosis of autoinflammatory diseases without pathogenic gene variants

Autoinflammatory diseases (AID) are rare chronic conditions with high disease burden, affecting children and adults. Clinically and genetically confirmed, AID can be effectively treated with targeted cytokine ...

Authors: Tatjana Welzel, Anna L. Wildermuth, Norbert Deschner, Susanne M. Benseler and Jasmin B. Kuemmerle-Deschner

Evaluating the performance of ACR, SLICC and EULAR/ACR classification criteria in childhood onset systemic lupus erythematosus

The ACR 1997, SLICC 2012 and EULAR/ACR 2019 classification criteria were validated based on adult patients. To date, there are no classification criteria specific for children with SLE. The aim of the study is...

Authors: Reem Abdwani, Eman Al Masroori, Eiman Abdullah, Safiya Al Abrawi and Ibrahim Al-Zakwani

Biologic medicine inclusion in 138 national essential medicines lists

Essential medicines lists (EMLs) are intended to reflect the priority health care needs of populations. We hypothesized that biologic disease-modifying antirheumatic drugs (DMARDs) are underrepresented relativ...

Authors: Raphaël Kraus, Rae S. M. Yeung and Nav Persaud

Immunomodulatory treatment and surgical management of idiopathic uveitis and juvenile idiopathic arthritis-associated uveitis in children: a French survey practice

Surgeries for idiopathic uveitis and juvenile idiopathic arthritis-associated uveitis in children are complex because of the high risk of inflammatory postoperative complications. There is no consensus about t...

Authors: Julie Molimard, Christine Pajot, Priscille Olle, Alexandre Belot, Pierre Quartier, Florence Uettwiller, Chloé Couret, Valentine Coste, Camille Costet, Bahram Bodaghi, Pascal Dureau, Marion Bailhache and Pascal Pillet

Blau syndrome: a case report from Palestine

This case study documents the first familial case of Blau syndrome (BS) in Palestine characterized with mutation in CARD15/NOD2.

Authors: Salam Iriqat, Mohammed Abu Safieh, Manuel Fatouleh and Abdulsalam Alkaiyat

Differences in rituximab use between pediatric rheumatologists and nephrologists for the treatment of refractory lupus nephritis and renal flare in childhood-onset SLE

Consensus treatment plans have been developed for induction therapy of newly diagnosed proliferative lupus nephritis (LN) in childhood-onset systemic lupus erythematosus. However, patients who do not respond t...

Authors: Mileka Gilbert, Beatrice Goilav, Joyce J. Hsu, Paul J. Nietert, Esra Meidan, Annabelle Chua, Stacy P. Ardoin, Scott E. Wenderfer, Emily von Scheven and Natasha M. Ruth

Developmentally appropriate transitional care during the Covid-19 pandemic for young people with juvenile-onset rheumatic and musculoskeletal diseases: the rationale for a position statement

The importance of developmentally appropriate transitional care in young people with juvenile-onset rheumatic and musculoskeletal disease is well recognised. The Paediatric Rheumatology European Society (PReS)...

Authors: Janet E. McDonagh, Rachel Tattersall, Jacqui Clinch, Joanne Swan, Helen E. Foster and Liza McCann

Juvenile idiopathic arthritis: from aetiopathogenesis to therapeutic approaches

Juvenile idiopathic arthritis (JIA) is the most common paediatric rheumatological disorder and is classified by subtype according to International League of Associations for Rheumatology criteria. Depending on...

Authors: Lina N. Zaripova, Angela Midgley, Stephen E. Christmas, Michael W. Beresford, Eileen M. Baildam and Rachel A. Oldershaw

Frequency of juvenile idiopathic arthritis and associated uveitis in pediatric rheumatology clinics in Turkey: A retrospective study, JUPITER

Juvenile idiopathic arthritis (JIA), is the most common pediatric rheumatologic disorder with unknown etiology. Currently, no population-based data are available regarding the distribution of categories and fr...

Authors: Sezgin Sahin, Ceyhun Acari, Hafize Emine Sonmez, Fatma Zehra Kilic, Erdal Sag, Hatice Adiguzel Dundar, Amra Adrovic, Selcan Demir, Kenan Barut, Yelda Bilginer, Betul Sozeri, Erbil Unsal, Seza Ozen and Ozgur Kasapcopur

Correction to: Targeted provider education and pre-visit planning increase rates of formal depression screening in childhood-onset SLE

Authors: Evan Mulvihill, Rebecca Furru, Alana Goldstein-Leever, Kyla Driest, Stephanie Lemle, Darby MacDonald, Emily Frost and Vidya Sivaraman

Golimumab in juvenile idiopathic arthritis-associated uveitis unresponsive to Adalimumab

To assess the efficacy of golimumab (GLM) as a treatment option for juvenile idiopathic arthritis (JIA)-associated uveitis refractory to adalimumab (ADA).

Authors: Sofia Lanz, Gerald Seidel and Andrea Skrabl-Baumgartner

Patterns of etanercept use in juvenile idiopathic arthritis in the Childhood Arthritis and Rheumatology Research Alliance Registry

We aimed to characterize etanercept (ETN) use in juvenile idiopathic arthritis (JIA) patients enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry.

Authors: Timothy Beukelman, Aimee Lougee, Roland A. Matsouaka, David Collier, Dax G. Rumsey, Jennifer Schenfeld, Scott Stryker, Marinka Twilt and Yukiko Kimura

Clinical remission and subsequent relapse in patients with juvenile idiopathic arthritis: predictive factors according to therapeutic approach

Juvenile idiopathic arthritis constitutes a significant cause of disability and quality of life impairment in pediatric and adult patients. The aim of this study was to ascertain clinical remission (CR) and su...

Authors: Mireia Castillo-Vilella, Nuria Giménez, Jose Luis Tandaipan, Salvador Quintana and Consuelo Modesto

Identifying the content and context of pain within paediatric rheumatology healthcare professional curricula in the UK: a summative content analysis

The curriculum for professionals working in paediatric rheumatology should include pain but it is unclear to what extent this currently occurs. The aim of this study was to identify pain-related curriculum con...

Authors: Rebecca Rachael Lee, Janet E. McDonagh, Mark Connelly, Sarah Peters and Lis Cordingley

A qualitative study of risk and resilience in young adult women with a history of juvenile-onset fibromyalgia

Juvenile-onset Fibromyalgia (JFM) is a chronic pain condition characterized by widespread musculoskeletal pain, fatigue, sleep difficulties, mood concerns, and other associated symptoms. Although diagnosed in ...

Authors: Morgan Daffin, Mary K. Lynch-Milder, Robert C. Gibler, Caitlin Murray, Carly M. Green and Susmita Kashikar-Zuck

Favorable outcomes with reduced steroid use in juvenile dermatomyositis

High-intensity glucocorticoid regimens are commonly used to induce and maintain remission in Juvenile Dermatomyositis but are associated with several adverse side-effects. Evidence-based treatment guidelines f...

Authors: Amir B. Orandi, Lampros Fotis, Jamie Lai, Hallie Morris, Andrew J. White, Anthony R. French and Kevin W. Baszis

Immunological repertoire linked to PSTPIP1-associated myeloid-related inflammatory (PAMI) syndrome

Mutations along PSTPIP1 gene are associated to two specific conditions, PAPA syndrome and PAMI syndrome, both autoinflammatory disorders associated to disturbances in cytoskeleton formation. Immunological aspe...

Authors: Leonardo Oliveira Mendonça, Maria Teresa Terreri, Fabiane Mitie Osaku, Samar Freschi Barros, Karen Francine Köhler, Alex Isidoro Prado, Myrthes Toledo Barros, Jorge Kalil and Fabio Fernandes Morato Castro

Comment on: homozygous variant p. Arg90His in NCF1 is associated with early-onset interferonopathy: a case report

Authors: George N. Goulielmos, Maria I. Zervou and Elias Eliopoulos

Correction to: Biologic disease modifying antirheumatic drugs and Janus kinase inhibitors in paediatric rheumatology – what we know and what we do not know from randomized controlled trials

Authors: Tatjana Welzel, Carolyn Winskill, Nancy Zhang, Andreas Woerner and Marc Pfister

Assessment of disease activity using a whole-body MRI derived radiological activity index in chronic nonbacterial osteomyelitis

Based on the recently developed ChRonic nonbacterial Osteomyelitis MRI Scoring tool (CROMRIS), we developed a radiological activity index (RAI-CROMRIS) to obtain a quantification of the overall bone involvemen...

Authors: Martina Capponi, Denise Pires Marafon, Flaminia Rivosecchi, Yongdong Zhao, Manuela Pardeo, Virginia Messia, Laura Tanturri de Horatio, Paolo Tomà, Fabrizio De Benedetti and Antonella Insalaco

Community poverty level influences time to first pediatric rheumatology appointment in Polyarticular Juvenile Idiopathic Arthritis

The impact of social determinants of health on children with polyarticular juvenile idiopathic arthritis (pJIA) is poorly understood. Prompt initiation of treatment for pJIA is important to prevent disease mor...

Authors: Nayimisha Balmuri, William Daniel Soulsby, Victoria Cooley, Linda Gerber, Erica Lawson, Susan Goodman, Karen Onel and Bella Mehta

Leukocytoclastic vasculitis in patients with IL12B or IL12RB1 deficiency: case report and review of the literature

Mendelian susceptibility to mycobacterial disease (MSMD) is an inborn error of immunity, resulting in susceptibility to weakly virulent mycobacteria and other intramacrophagic pathogens. Rheumatologic manifest...

Authors: Niusha Sharifinejad, Seyed Alireza Mahdaviani, Mahnaz Jamee, Zahra Daneshmandi, Afshin Moniri, Majid Marjani, Payam Tabarsi, Parisa Farnia, Mahsa Rekabi, Mazdak Fallahi, Seyedeh Atefeh Hashemimoghaddam, Masoumeh Mohkam, Jacinta Bustamante, Jean-Laurent Casanova, Davood Mansouri and Ali Akbar Velayati

Anasarca as the presenting symptom of juvenile dermatomyositis: a case series

Juvenile Dermatomyositis (JDM) is an autoimmune disease that typically presents with classic skin rashes and proximal muscle weakness. Anasarca is a rare manifestation of this disease and is associated with a ...

Authors: Emily E. Schildt and Deirdre De Ranieri

US NHANES Data 2013–2016: increased risk of severe obesity in individuals with history of juvenile idiopathic arthritis

Authors: Stephanie Merwin, Eleanor Mackey and Sangeeta Sule

Changes in total body fat and body mass index among children with juvenile dermatomyositis treated with high-dose glucocorticoids

High-dose glucocorticoids (GC) remain the primary therapy to induce remission in Juvenile Dermatomyositis (JDM). Studies of the natural history of GC associated weight gain in children are very limited, especi...

Authors: Amer Khojah, Victoria Liu, Gabrielle Morgan, Richard M. Shore and Lauren M. Pachman