Articles

Pediatric rheumatology in Africa: thriving amidst challenges

Pediatric Rheumatology is an orphan specialty in Africa which is gradually gaining importance across the continent.

Authors: Angela N. Migowa, Djohra Hadef, Wafa Hamdi, Oscar Mwizerwa, Madeleine Ngandeu, Yassmin Taha, Faleye Ayodele, Kate Webb and Christiaan Scott

Severe COVID-19 in pediatric age: an update on the role of the anti-rheumatic agents

SARS-CoV-2 can induce an immune impairment and dysregulation, finally resulting in the massive release of inflammatory mediators (cytokine storm), strongly contributing to the pulmonary and systemic manifestat...

Authors: Giorgio Costagliola, Erika Spada and Rita Consolini

Electronic forms for patient reported outcome measures (PROMs) are an effective, time-efficient, and cost-minimizing alternative to paper forms

Patient reported outcome measures (PROMs) provide valuable insight on patients’ well-being and facilitates communication between healthcare providers and their patients. The increased integration of the techno...

Authors: Jennifer Y. Yu, Talia Goldberg, Nicholas Lao, Brian M. Feldman and Y. Ingrid Goh

Biomarkers of lipid metabolism in patients with juvenile idiopathic arthritis: relationship with disease subtype and inflammatory activity

To describe the biomarkers of lipid metabolism in children and adolescents with polyarticular and systemic JIA and to relate them to diseases subtypes, diseases activity markers, and nutritional status.

Authors: Wellington Douglas Rocha Rodrigues, Roseli Oselka Saccardo Sarni, Fernando Luiz Affonso Fonseca, Annelyse Araújo, Claudio Arnaldo Len and Maria Teresa Terreri

Time for change at PROJ in 2021

Authors: Charles H. Spencer and Alberto Martini

Trajectories of disease courses in the inception cohort of newly diagnosed patients with JIA (ICON-JIA): the potential of serum biomarkers at baseline

Juvenile idiopathic arthritis (JIA) is a heterogeneous group of inflammatory joint disorders with a chronic-remitting disease course. Treat-to-target approaches have been proposed but monitoring disease activi...

Authors: Margarita Ganeva, Sabrina Fuehner, Christoph Kessel, Jens Klotsche, Martina Niewerth, Kirsten Minden, Dirk Foell, Claas H. Hinze and Helmut Wittkowski

Disseminated coccidioidomycosis in a patient with juvenile idiopathic arthritis receiving infliximab

Coccidioides immitis is a dimorphic fungus endemic to the arid climates of the Southwest United States, Mexico and parts of Central and South America. Human infection occurs through inhalation of spores with less...

Authors: Megan Trainor, Emily Henkel, Lucia Z. Diaz and Ruy Carrasco

Factors associated with reduced infliximab exposure in the treatment of pediatric autoimmune disorders: a cross-sectional prospective convenience sampling study

Inadequate systemic exposure to infliximab (IFX) is associated with treatment failure. This work evaluated factors associated with reduced IFX exposure in children with autoimmune disorders requiring IFX therapy.

Authors: Ryan S. Funk, Valentina Shakhnovich, Yu Kyoung Cho, Kishore Polireddy, Taina Jausurawong, Kyle Gress and Mara L. Becker

Assessing preparation for care transition among adolescents with rheumatologic disease: a single-center assessment with patient survey

Despite the risk for poor outcomes and gaps in care in the transfer from pediatric to adult care, most pediatric rheumatology centers lack formal transition pathways. As a first step in designing a pathway, we...

Authors: Jordan E. Roberts, Olha Halyabar, Carter R. Petty and Mary Beth Son

Utilizing ultrasound findings of a single indicator joint to assess non-systemic juvenile idiopathic arthritis

Musculoskeletal ultrasound (MSUS) has been used worldwide in adult patients with rheumatoid arthritis (RA) but is beginning to play an increasing role in patients with juvenile idiopathic arthritis (JIA). The ...

Authors: Yung-Hsien Huang, Ya-Chiao Hu, Chun-Hua Liao, Bor-Luen Chiang, Cheng-Hsun Lu, Ko-Jen Li and Yao-Hsu Yang

Therapeutic drug monitoring of anti-TNF drugs: an overview of applicability in daily clinical practice in the era of treatment with biologics in juvenile idiopathic arthritis (JIA)

Anti-tumor necrosis factor (TNF) drugs have improved the prognosis for juvenile idiopathic arthritis (JIA) significantly. However, evidence for individual treatment decisions based on serum anti-TNF drug level...

Authors: A. Nassar-Sheikh Rashid, D. Schonenberg-Meinema, S. C. Bergkamp, S. Bakhlakh, A. de Vries, T. Rispens, T. W. Kuijpers, G. Wolbink and J. M. van den Berg

Musculoskeletal ultrasound assessment in pediatric knee hypermobility: a case control study

While musculoskeletal ultrasound (MSUS) use in pediatric rheumatology is becoming more common, the majority of pediatric MSUS literature continues to focus on ultrasound findings in healthy children and juveni...

Authors: Laura R. Ballenger, Melissa Moore-Clingenpeel and Edward J. Oberle

Is podocytopathy another image of renal affection in p-SLE?

Lupus podocytopathy (LP) is a renal affection described in systemic lupus erythematosus (SLE) patients with nephrotic range proteinuria, characterized by diffuse foot process effacement without immune deposits...

Authors: Hend H. Abdelnabi

Bone mineral density and explanatory factors in children and adults with juvenile dermatomyositis at long term follow-up; a cross sectional study

Juvenile dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy in children and adolescents. Both the disease and its treatment with glucocorticoids may negatively impact bone formation. In ...

Authors: Henriette Schermacher Marstein, Kristin Godang, Berit Flatø, Ivar Sjaastad, Jens Bollerslev and Helga Sanner

Fecal microbiota in children with juvenile idiopathic arthritis treated with methotrexate or etanercept

Alterations in the composition of the fecal microbiota in children with juvenile idiopathic arthritis (JIA) have been observed in several studies, but it has not been determined whether the standard treatment ...

Authors: Anders Öman, Johan Dicksved, Lars Engstrand and Lillemor Berntson

Homozygous variant p. Arg90His in NCF1 is associated with early-onset Interferonopathy: a case report

Biallelic loss-of-function variants in NCF1 lead to reactive oxygen species deficiency and chronic granulomatous disease (CGD). Heterozygosity for the p.Arg90His variant in NCF1 has been associated with susceptib...

Authors: Oskar Schnappauf, Liane Heale, Dilan Dissanayake, Wanxia L. Tsai, Massimo Gadina, Thomas L. Leto, Daniel L. Kastner, Harry L. Malech, Douglas B. Kuhns, Ivona Aksentijevich and Ronald M. Laxer

Neuropsychological functioning and academic abilities in patients with juvenile idiopathic arthritis

The involvement of the central nervous system is not rare in rheumatoid diseases. Even though children with juvenile idiopathic arthritis (JIA) may face academic difficulties until adulthood, very few studies ...

Authors: Marine Granjon, Odile Rohmer, Nadège Doignon-Camus, Maria Popa-Roch, Christine Pietrement and Nathalie Gavens

Dutch patients, caregivers and healthcare professionals generate first nationwide research agenda for juvenile idiopathic arthritis

Involving the end-users of scientific research (patients, carers and clinicians) in setting research priorities is important to formulate research questions that truly make a difference and are in tune with th...

Authors: Anouk Verwoerd, Wineke Armbrust, Katherine Cowan, Lotte van den Berg, Joke de Boer, Sanne Bookelman, Marjan Britstra, Jeannette Cappon, Maria Certan, Christine Dedding, Karin van den Haspel, Petra Hissink Muller, Karin Jongsma, Otto Lelieveld, Jorg van Loosdregt, Wendy Olsder…

Single nucleotide polymorphisms associated with methotrexate-induced nausea in juvenile idiopathic arthritis

Authors: Nini Kyvsgaard, Torben Stamm Mikkelsen, Thomas D. Als, Anne Estmann Christensen, Thomas J. Corydon and Troels Herlin

Paediatric non-infectious uveitis in Cape Town, South Africa: a retrospective review of disease characteristics and outcomes on immunomodulating treatment

Non-infectious uveitis is a well-reported cause of blindness in more developed countries, however data from sub-Saharan Africa is lacking. Here we aim to describe the diseases associated with paediatric non-in...

Authors: Waheba Slamang, Christopher Tinley, Nicola Brice and Christiaan Scott

Ruxolitinib treatment permits lower cumulative glucocorticoid dosing in children with secondary hemophagocytic lymphohistiocytosis

This study aimed to analyze the effects of ruxolitinib on children with secondary hemophagocytic lymphohistiocytosis (HLH).

Authors: Ying Chi, Rong Liu, Zhi-xuan Zhou, Xiao-dong Shi, Yu-chuan Ding and Jian-guo Li

Epidemiology of pediatric uveitis and associated systemic diseases

The early detection of uveitis associated with systemic inflammatory disease in children is important for proper treatment and prognosis. However, the diagnosis may be delayed because of difficulties in childh...

Authors: Yoonkyeom Shin, Ji-Man Kang, Junwon Lee, Christopher Seungkyu Lee, Sung Chul Lee and Jong Gyun Ahn

A systematic review exploring the bidirectional relationship between puberty and autoimmune rheumatic diseases

Autoimmune rheumatic diseases (ARDs) are associated with a significant sex-bias, which becomes more evident post-puberty. This systematic review aims to elucidate the bidirectional relationship between puberty...

Authors: Nina M. de Gruijter, Meena Naja, Hannah Peckham, Anna Radziszewska, Matthew Kinsella, James Glenister, Elizabeth C. Rosser, Gary E. Butler, Elizabeth C. Jury and Coziana Ciurtin

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

Biologic disease modifying antirheumatic drugs (bDMARDs) and Janus Kinase (JAK) inhibitors are prescribed in adult and paediatric rheumatology. Due to age-dependent changes, disease course, and pharmacokinetic...

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

Variability in phenotype and response to treatment in chronic nonbacterial osteomyelitis; the Irish experience of a national cohort

Chronic nonbacterial osteomyelitis (CNO) is an autoinflammatory disease affecting bone with considerable phenotypic heterogeneity and variable association with other autoinflammatory conditions. Disease pathog...

Authors: Daire O’Leary, Anthony G. Wilson, Emma-Jane MacDermott, Clodagh Lowry and Orla G. Killeen

Diagnosis and management of adenosine deaminase 2 deficiency children: the experience from China

Deficiency of adenosine deaminase 2 (DADA2) is a rare autoinflammatory disease caused by mutations in the ADA2 gene. Few Chinese cases have been reported. We describe and compare the clinical features, genotyp...

Authors: Wei Wang, Tiannan Zhang, Wenjie Zheng, Linqing Zhong, Lin Wang, Ji Li, Qian Liu, Yanqing Dong and Hongmei Song

‘Snakes & Ladders’: factors influencing access to appropriate care for children and young people with suspected juvenile idiopathic arthritis – a qualitative study

Many children and young people with juvenile idiopathic arthritis (JIA) experience delay in diagnosis and access to right care. The reasons for delay are multi-factorial and influenced by patient and family, c...

Authors: Tim Rapley, Carl May, Nicola Smith and Helen E. Foster

Magnetic resonance imaging findings in children with Parry-Romberg syndrome and en coup de sabre

The aim of this study was to: (i) describe the abnormalities seen on brain imaging in a group of children with en coup de sabre (EDCS) with/without Parry-Romberg syndrome (PRS); and (ii) identify clinical pred...

Authors: Harry Knights, Elizabeth Minas, Faraan Khan, Lindsay Shaw, Muthana Al Obaidi, Kshitij Mankad and Despina Eleftheriou

Outcome of children with oligoarticular juvenile idiopathic arthritis compared to polyarthritis on methotrexate- data of the German BIKER registry

Oligoarticular juvenile idiopathic arthritis (oligoJIA) is the most commonly diagnosed category of chronic arthritis in children. Nevertheless, there are no evidence- based guidelines for its treatment, in par...

Authors: A. Raab, T. Kallinich, D. Huscher, I. Foeldvari, F. Weller-Heinemann, F. Dressler, J. B. Kuemmerle-Deschner, A. Klein and G. Horneff

Monitoring patients with juvenile idiopathic arthritis using health-related quality of life

Pediatric patients with juvenile idiopathic arthritis (JIA) are at risk for a lower health-related quality of life compared to their healthy peers. Remote monitoring of health-related quality of life using ele...

Authors: Martijn J. H. Doeleman, Sytze de Roock, Nathan Buijsse, Mark Klein, Gouke J. Bonsel, Vicki Seyfert-Margolis, Joost F. Swart and Nico M. Wulffraat

Poor physical activity levels and cardiorespiratory fitness among patients with childhood-onset takayasu arteritis in remission: a cross‐sectional, multicenter study

It is currently unknown whether patients with childhood-onset Takayasu disease (c-TA) are prone to physical inactivity and poor aerobic capacity. In this study, we assessed physical activity levels and cardior...

Authors: Camilla Astley, Saulo Gil, Gleice Clemente, Maria Teresa Terreri, Clovis Artur Silva, Lucia Maria Arruda Campos, Nadia Emi Aikawa, Ana Lúcia de Sá Pinto, Rosa Maria R. Pereira, Hamilton Roschel and Bruno Gualano

Experiences with IL-1 blockade in systemic juvenile idiopathic arthritis – data from the German AID-registry

Systemic juvenile idiopathic arthritis (sJIA) is a complex disease with dysregulation of the innate immune system driven by cytokines. A major role is ascribed to interleukin-1β (IL-1β), supporting the autoinf...

Authors: Elke Lainka, Melanie Baehr, Bernadette Raszka, Johannes-Peter Haas, Boris Hügle, Nadine Fischer, Dirk Foell, Claas Hinze, Elisabeth Weissbarth-Riedel, Tilmann Kallinich, Gerd Horneff, Daniel Windschall, Eggert Lilienthal, Tim Niehues, Ulrich Neudorf, Rainer Berendes…

Joint contractures responsive to immunosuppressive therapy in a girl with childhood‐onset systemic sclerosis double‐seropositive for rare anti‐nucleolar autoantibodies: a case report

Systemic sclerosis (SSc; scleroderma) is an autoimmune connective tissue disease that affects the skin and subcutaneous tissue, in addition to the internal organs of the whole body. Onset in childhood is uncom...

Authors: Riki Tanaka, Yumi Tani, Yoichiro Kaburaki, Manao Kinoshita, Yasushi Kawaguchi, Yuka Okazaki, Masataka Kuwana, Masayoshi Harigai, Satoru Nagata and Takako Miyamae

Response to treatment with intra-articular triamcinolone hexacetonide and triamcinolone acetonide in oligo articular juvenile idiopathic arthritis

Oligo-articular juvenile idiopathic arthritis (Oligo JIA) is the most common subtype of juvenile idiopathic arthritis. Intra-articular corticosteroid (IAC) injection is a mainstay treatment of oligo JIA provid...

Authors: Rana Harhay, Wajiha Jeelani, Barbine Tchamba Agbor Agbor, Teresa Hennon, Brian H. Wrotniak and Rabheh Abdul-Aziz

Disease activity index is associated with subclinical atherosclerosis in childhood-onset systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is an independent risk factor for cardiovascular events. The present study determined the prevalence of subclinical atherosclerosis in childhood-onset SLE using the carotid i...

Authors: Priscila B. S. Medeiros, Roberta G. Salomão, Sara R. Teixeira, Diane M. Rassi, Luciana Rodrigues, Davi C. Aragon, Priscila G. Fassini, Fábio V. Ued, Rita C. Tostes, Jacqueline P. Monteiro, Virgínia P. L. Ferriani and Luciana M. de Carvalho

Anti-SAE autoantibody-positive Japanese patient with juvenile dermatomyositis complicated with interstitial lung disease - a case report

Clinical phenotypes and outcomes in juvenile dermatomyositis (JDM) have been defined by various myositis-specific autoantibodies (MSAs). One of the recently described MSAs associated with DM is targeted agains...

Authors: Takayuki Kishi, Yumi Tani, Naoko Okiyama, Kiyoshi Mizuochi, Yuki Ichimura, Masayoshi Harigai, Satoru Nagata and Takako Miyamae

Fatigue in young adults with juvenile idiopathic arthritis 18 years after disease onset: data from the prospective Nordic JIA cohort

To study fatigue in young adults with juvenile idiopathic arthritis (JIA) 18 years after disease onset, and to compare with controls.

Authors: Ellen Dalen Arnstad, Mia Glerup, Veronika Rypdal, Suvi Peltoniemi, Anders Fasth, Susan Nielsen, Marek Zak, Kristiina Aalto, Lillemor Berntson, Ellen Nordal, Troels Herlin, Pål Richard Romundstad and Marite Rygg

Genetics of chronic nonbacterial osteomyelitis in the irish population: no significant association with rare FBLIM1 variants

Authors: Daire O’Leary, Orla G Killeen and Anthony G Wilson

Cyclophosphamide use in treatment of refractory Kawasaki disease with coronary artery aneurysms

Despite timely administration of IVIG, some patients with Kawasaki disease (KD) develop rapidly progressive or giant coronary artery aneurysms (CAA).

Authors: Olha Halyabar, Kevin G. Friedman, Robert P. Sundel, Annette L. Baker, Margaret H. Chang, Patrick W. Gould, Jane W. Newburger and Mary Beth F. Son

Psychosocial and economic impact of rheumatic diseases on caregivers of Mexican children

Pediatric rheumatic disease (PRD) patients and their caregivers face a number of challenges, including the consequences of the PRD in patients and the impact on multiple dimensions of the caregivers’ daily liv...

Authors: Brenda de Jesús Fortuna-Reyna, Ingris Peláez-Ballestas, Fernando García-Rodríguez, Enrique Faugier-Fuentes, Samara Mendieta-Zerón, Ana Victoria Villarreal-Treviño, Sara Georgina Rosiles-De la Garza, Greta Reyes-Cordero, Sol Jiménez-Hernández, Jessica Haydee Guadarrama-Orozco, Manuel Enrique de la O-Cavazos and Nadina Rubio-Pérez

Defining Kawasaki disease and pediatric inflammatory multisystem syndrome-temporally associated to SARS-CoV-2 infection during SARS-CoV-2 epidemic in Italy: results from a national, multicenter survey

There is mounting evidence on the existence of a Pediatric Inflammatory Multisystem Syndrome-temporally associated to SARS-CoV-2 infection (PIMS-TS), sharing similarities with Kawasaki Disease (KD). The main o...

Authors: Marco Cattalini, Sara Della Paolera, Fiammetta Zunica, Claudia Bracaglia, Manuela Giangreco, Lucio Verdoni, Antonella Meini, Rita Sottile, Roberta Caorsi, Gianvincenzo Zuccotti, Marianna Fabi, Davide Montin, Alessandra Meneghel, Alessandro Consolaro, Rosa Maria Dellepiane, Maria Cristina Maggio…

Growth and puberty in children with juvenile idiopathic arthritis

Juvenile Idiopathic Arthritis is one of the most prevalent chronic diseases in children, with an annual incidence of 2–20 cases per 100,000 and a prevalence of 16–150 per 100,000. It is associated with several...

Authors: Debora Mariarita d’Angelo, Giulia Di Donato, Luciana Breda and Francesco Chiarelli

Sustained remission after haploidentical bone marrow transplantation in a child with refractory systemic juvenile idiopathic arthritis

Some patients with systemic juvenile idiopathic arthritis (SJIA) and severe, refractory disease achieved remission through intensive immunosuppressive treatment followed by autologous hematopoietic stem cell t...

Authors: Guillaume Morelle, Martin Castelle, Graziella Pinto, Sylvain Breton, Matthieu Bendavid, Charlotte Boussard, Richard Mouy, Brigitte Bader-Meunier, Michaela Semeraro, Albert Faye, Marina Cavazzana, Bénédicte Neven, Stéphane Blanche, Pierre Quartier and Despina Moshous

Response to influenza vaccination in immunocompromised children with rheumatic disease: a prospective cohort study

Prevention of illness due to infection by influenza viruses is important for children with rheumatic diseases. Biological disease modifying antirheumatic drugs have become increasingly important in the treatme...

Authors: Lotte Jensen, Susan Nielsen, Anne Estmann Christensen, Freddy Karup Pedersen, Ramona Trebbien, Thea Kølsen Fischer, Susanne Rosthøj, Peter Toftedal, Anna-Helene Bohr, Peder Skov Wehner and Anja Poulsen

Monocyte platelet aggregates in children with Kawasaki disease- a preliminary study from a tertiary care centre in North-West India

Platelet activation is an integral part of pathogenesis of Kawasaki disease (KD). However, there is paucity of literature on flow-cytometry based assessment of platelet activation in KD. We aimed to analyse mo...

Authors: Pandiarajan Vignesh, Amit Rawat, Jitendra Kumar Shandilya, Man Updesh Singh Sachdeva, Jasmina Ahluwalia and Surjit Singh

Predictive value of C-reactive protein to albumin ratio as a biomarker for initial and repeated intravenous immunoglobulin resistance in a large cohort of Kawasaki disease patients: a prospective cohort study

Intravenous immunoglobulin (IVIG) resistance prediction is one pivotal topic of interests in Kawasaki disease (KD). This study aimed to prospectively investigated the value of C-reactive protein-to-albumin (CA...

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

Lung clearance index and diffusion capacity for CO to detect early functional pulmonary impairment in children with rheumatic diseases

In adults with rheumatic diseases pulmonary complications are relevant contributors to morbidity and mortality. In these patients diffusion capacity for CO (DLCO) is an established method to detect early pulmo...

Authors: Julia Hildebrandt, Anja Rahn, Anja Kessler, Fabian Speth, Dagmar-Christiane Fischer and Manfred Ballmann

Juvenile primary Fibromyalgia Syndrome: epidemiology, etiology, pathogenesis, clinical manifestations and diagnosis

Juvenile primary fibromyalgia syndrome (JPFS) is a chronic, musculoskeletal pain syndrome affecting children and adolescents, most commonly adolescent girls. The syndrome has a multifactorial etiology, with al...

Authors: Maya Levy Coles, Rotem Weissmann and Yosef Uziel

Distinguishing active pediatric COVID-19 pneumonia from MIS-C

Active pediatric COVID-19 pneumonia and MIS-C are two disease processes requiring rapid diagnosis and different treatment protocols.

Authors: Daniel D. Reiff, Melissa L. Mannion, Nichole Samuy, Paul Scalici and Randy Q. Cron

Childhood‐onset systemic lupus erythematosus with trisomy X and the increased risk for bone complications: a case report

Systemic lupus erythematosus is a multi-organ inflammatory autoimmune disease; immune complexes are part of the pathogenesis, but not entirely responsible. Trisomy X is the most common female chromosomal abnor...

Authors: Susumu Yamazaki, Yuko Akutsu, Asami Shimbo, Masaki Shimizu, Yuko Segawa and Masaaki Mori