Articles

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

Pediatric Rheumatology Collaborative Study Group – over four decades of pivotal clinical drug research in pediatric rheumatology

Specialized research networks are essential to achieve drug approvals for rare pediatric diseases. Such networks help realize the potential of global legislation enacted upon the recognition that most children...

Authors: Hermine I. Brunner, Lisa G. Rider, Daniel J. Kingsbury, Dominic Co, Rayfel Schneider, Ellen Goldmuntz, Karen B. Onel, Edward H. Giannini and Daniel J. Lovell

Proceedings of the 2018 Childhood Arthritis and Rheumatology Research Alliance (CARRA) Scientific Meeting

Authors:

Frequency of CD19⁺CD24^hiCD38^hi regulatory B cells is decreased in peripheral blood and synovial fluid of patients with juvenile idiopathic arthritis: a preliminary study

To understand the relationship between regulatory B cells (Bregs) and juvenile idiopathic arthritis (JIA), we analyzed the percentages of Bregs and their function in peripheral blood (PB) and synovial fluid (S...

Authors: Qianzi Zhao and Lawrence K. Jung

Kawasaki Disease in the neonate: case report and literature review

Kawasaki Disease (KD), the leading cause of acquired heart disease in children in the developed world, is extremely rare in neonates. We present a case of incomplete KD in a neonate and a review of the literat...

Authors: Fajer Altammar and Bianca Lang

A 6-month, multicenter, open-label study of fixed dose naproxen/esomeprazole in adolescent patients with juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA) is an inflammatory arthritis of unknown etiology, which lasts for greater than 6 weeks with onset before 16 years of age. JIA is the most common chronic rheumatic disease in...

Authors: Daniel J. Lovell, Jason A. Dare, Megan Francis-Sedlak, Julie Ball, Brian D. LaMoreaux, Emily Von Scheven, Adam Reinhardt, Rita Jerath, Oral Alpan, Ramesh Gupta, Donald Goldsmith, Andrew Zeft, Henry Naddaf, Beth Gottlieb, Lawrence Jung and Robert J. Holt

Development of practice and consensus-based strategies including a treat-to-target approach for the management of moderate and severe juvenile dermatomyositis in Germany and Austria

Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in childhood and a major cause of morbidity among children with pediatric rheumatic diseases. The management of JDM is very heterogeneous...

Authors: Claas H. Hinze, Prasad T. Oommen, Frank Dressler, Andreas Urban, Frank Weller-Heinemann, Fabian Speth, Elke Lainka, Jürgen Brunner, Heike Fesq, Dirk Foell, Wolfgang Müller-Felber, Ulrich Neudorf, Christoph Rietschel, Tobias Schwarz, Ulrike Schara and Johannes-Peter Haas

The transcription factor CREM drives an inflammatory phenotype of T cells in oligoarticular juvenile idiopathic arthritis

Inflammatory effector T cells trigger inflammation despite increased numbers of Treg cells in the synovial joint of patients suffering from juvenile idiopathic arthritis (JIA). The cAMP response element (CREM)...

Authors: Kim Ohl, Helge Nickel, Halima Moncrieffe, Patricia Klemm, Anja Scheufen, Dirk Föll, Viktor Wixler, Angela Schippers, Norbert Wagner, Lucy R. Wedderburn and Klaus Tenbrock

Current management of juvenile dermatomyositis in Germany and Austria: an online survey of pediatric rheumatologists and pediatric neurologists

Juvenile Dermatomyositis (JDM) is a rare pediatric autoimmune disease with broad variations of the individual course. Data on the optimal management are mostly lacking. Currently treatment decisions are often ...

Authors: Claas H. Hinze, Fabian Speth, Prasad T. Oommen and Johannes-Peter Haas

Reliability of LoSCAT score for activity and tissue damage assessment in a large cohort of patients with Juvenile Localized Scleroderma

To assess reliability of the two indexes of Localized Scleroderma Cutaneous Assessment Tool (LoSCAT), the modified Localized Scleroderma Skin Severity Index (mLoSSI) and the Localized Scleroderma Skin Damage I...

Authors: Anna Agazzi, Gloria Fadanelli, Fabio Vittadello, Francesco Zulian and Giorgia Martini

Early use of alendronate as a protective factor against the development of glucocorticoid-induced bone loss in childhood-onset rheumatic diseases: a cross-sectional study

Bisphosphonates are recommended for use as first-line therapy for the prevention and treatment of glucocorticoid-induced osteoporosis in adults. However, the appropriate usage of bisphosphonates for the preven...

Authors: Yuzaburo Inoue, Kanako Mitsunaga, Takeshi Yamamoto, Koki Chiba, Fumiya Yamaide, Taiji Nakano, Yoshinori Morita, Akiko Yamaide, Shuichi Suzuki, Takayasu Arima, Ken-ichi Yamaguchi, Minako Tomiita, Naoki Shimojo and Yoichi Kohno

What do young people with rheumatic conditions in the UK think about research involvement? A qualitative study

Involving people of all ages in health research is now widely advocated. To date, no studies have explored whether and how young people with chronic rheumatic conditions want to be involved in influencing heal...

Authors: Suzanne Parsons, Wendy Thomson, Katharine Cresswell, Bella Starling and Janet E. McDonagh

Vitamin D and juvenile idiopathic arthritis

Vitamin D has been implicated in the pathogenesis of autoimmune diseases. While the roles of vitamin D in other autoimmune diseases have been investigated, less is known about the role of vitamin D in chronic ...

Authors: Sarah L. Finch, Alan M. Rosenberg and Hassan Vatanparast

Circulating adipokines are associated with Kawasaki disease

The pathogenesis of Kawasaki disease are still not well understood. It was designed to investigate the relationship between adipokines including chemerin, omentin-1, adiponectin and acute Kawasaki disease.

Authors: Xin-yan Zhang, Ting-ting Yang, Xiu-fen Hu, Yu Wen, Feng Fang and Hui-ling Lu

Temporomandibular joint arthritis in juvenile idiopathic arthritis, now what?

Arthritis involving the temporomandibular joint (TMJ) complicates 40 - 96% of cases of juvenile idiopathic arthritis (JIA), potentially leading to devastating changes to form and function. Optimal evaluation a...

Authors: Matthew L. Stoll, Chung H. Kau, Peter D. Waite and Randy Q. Cron

Clinical study of children with cryofibrinogenemia: a retrospective study from a single center

This study aimed to evaluate the demographic, clinical features, laboratory data, pathology and other survey in pediatric patients with cryofibrinogenemia.

Authors: Hsiao-Feng Chou, Yu-Hung Wu, Che-Sheng Ho and Yu-Hsuan Kao

Final adult height of patients with childhood-onset systemic lupus erythematosus: a cross sectional analysis

To compare final height to mid-parental target height among adults with childhood-onset systemic lupus erythematosus (cSLE) versus adult-onset SLE (aSLE), and to evaluate the impact of age at SLE onset on fina...

Authors: Merav Heshin-Bekenstein, Liat Perl, Aimee O. Hersh, Emily von Scheven, Ed Yelin, Laura Trupin, Jinoos Yazdany and Erica F. Lawson

Correction to: A national cohort study on pediatric Behçet’s disease: cross-sectional data from an Italian registry

Following publication of the original article [1], the authors reported that the names of two institutional authors – EUROFEVER and the Paediatric Rheumatology International Trials Organisation (PRINTO) – had ...

Authors: Romina Gallizzi, Caterina Pidone, Luca Cantarini, Martina Finetti, Marco Cattalini, Giovanni Filocamo, Antonella Insalaco, Donato Rigante, Rita Consolini, Maria Cristina Maggio, Adele Civino, Silvana Martino, Alma Nunzia Olivieri, Giovanna Fabio, Serena Pastore, Angela Mauro…

Comparing the importance of quality measurement themes in juvenile idiopathic inflammatory myositis between patients and families and healthcare professionals

A standardized set of quality measures for juvenile idiopathic inflammatory myopathies (JIIM) is not in use. Discordance has been shown between the importance ascribed to quality measures between patients and ...

Authors: Heather O. Tory, Ruy Carrasco, Thomas Griffin, Adam M. Huber, Philip Kahn, Angela Byun Robinson, David Zurakowski and Susan Kim

An international delphi survey for the definition of the variables for the development of new classification criteria for periodic fever aphtous stomatitis pharingitis cervical adenitis (PFAPA)

Diagnosis of Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) is currently based on a set of criteria proposed in 1999 modified from Marshall’s criteria. Nevertheless no validated...

Authors: Federica Vanoni, Silvia Federici, Jordi Antón, Karyl S. Barron, Paul Brogan, Fabrizio De Benedetti, Fatma Dedeoglu, Erkan Demirkaya, Veronique Hentgen, Tilmann Kallinich, Ronald Laxer, Ricardo Russo, Natasa Toplak, Yosef Uziel, Alberto Martini, Nicolino Ruperto…

Adverse pregnancy outcomes in adolescents and young women with systemic lupus erythematosus: a national estimate

Pregnant women with systemic lupus erythematosus (SLE) have increased risk of adverse outcomes including disease flare, spontaneous abortion, preeclampsia/eclampsia, premature birth and maternal death. However...

Authors: Nicole Ling, Erica Lawson and Emily von Scheven

Incidence and risk factors for recurrent Henoch-Schönlein purpura in children from a 16-year nationwide database

The recurrence rate of Henoch-Schönlein purpura (HSP) is 2.7%–30%, with varied average intervals between the first and second episodes. Few studies have explored the incidence and risk factors for recurrent HSP.

Authors: Wei-Te Lei, Po-Li Tsai, Szu-Hung Chu, Yu-Hsuan Kao, Chien-Yu Lin, Li-Ching Fang, Shyh-Dar Shyur, Yu-Wen Lin and Shu-I Wu

MBL2 gene polymorphism rs1800450 and rheumatic fever with and without rheumatic heart disease: an Egyptian pilot study

Rheumatic fever (RF) is the result of an autoimmune response to pharyngitis caused by infection with Streptococcus pyogenes. RF is most prevalent in Africa and the Middle East. Rheumatic heart disease (RHD) is th...

Authors: Maher Hassan Gomaa, Shawkey Sadik Ali, Aya Mohamed Fattouh, Hala Salah Hamza and Mohamed Mohamed Badr

Amendment of the OMERACT ultrasound definitions of joints’ features in healthy children when using the DOPPLER technique

Recently preliminary ultrasonography (US) definitions, in B mode, for normal components of pediatric joints have been developed by the OMERACT US group. The aim of the current study was to include Doppler find...

Authors: P. Collado, D. Windschall, J. Vojinovic, S. Magni-Manzoni, P. Balint, G. A. W. Bruyn, C. Hernandez-Diaz, J. C. Nieto, V. Ravagnani, N. Tzaribachev, A. Iagnocco, M. A. D’Agostino and E. Naredo

IL-6 blockade in systemic juvenile idiopathic arthritis – achievement of inactive disease and remission (data from the German AID-registry)

Systemic juvenile idiopathic arthritis (sJIA) is a complex disease with an autoinflammatory component of unknown etiology related to the innate immune system. A major role in the pathogenesis has been ascribed...

Authors: M. Bielak, E. Husmann, N. Weyandt, J.-P. Haas, B. Hügle, G. Horneff, U. Neudorf, T. Lutz, E. Lilienthal, T. Kallinich, K. Tenbrock, R. Berendes, T. Niehues, H. Wittkowski, E. Weißbarth-Riedel, G. Heubner…

Clinical features of children with enthesitis-related juvenile idiopathic arthritis / juvenile spondyloarthritis followed in a French tertiary care pediatric rheumatology centre

Childhood-onset spondyloarthropathies usually start with enthesitis and peripheral arthritis. However, axial disease may develop afterward. Patients are most often classified, following revised (Edmonton 2011)...

Authors: Maxime Goirand, Sylvain Breton, Frédéric Chevallier, Ngoc-Phoi Duong, Florence Uettwiller, Isabelle Melki, Richard Mouy, Carine Wouters, Brigitte Bader-Meunier, Chantal Job-Deslandre and Pierre Quartier

Impairment of microcirculation and vascular responsiveness in adolescents with primary Raynaud phenomenon

Raynaud’s phenomenon (RP) is a functional vascular disease, presenting with recurrent episodes of ischemia of extremities in response to cold and emotional stress. Investigating cutaneous microcirculation is a...

Authors: Bernadett Mosdósi, Kata Bölcskei and Zsuzsanna Helyes

The safety of live-attenuated vaccines in patients using IL-1 or IL-6 blockade: an international survey

Withholding live-attenuated vaccines in patients using interleukin (IL)-1 or IL-6 blocking agents is recommended by guidelines for both pediatric and adult rheumatic diseases, since there is a risk of infectio...

Authors: Jerold Jeyaratnam, Nienke M. ter Haar, Helen J. Lachmann, Ozgur Kasapcopur, Amanda K. Ombrello, Donato Rigante, Fatma Dedeoglu, Ezgi H. Baris, Sebastiaan J. Vastert, Nico M. Wulffraat and Joost Frenkel

Improvement of medication adherence in adolescents and young adults with SLE using web-based education with and without a social media intervention, a pilot study

Self-management skills, including medication management, are vital to the health of adolescents and young adults with systemic lupus erythematosus (SLE). The purpose of this study was to assess the feasibility...

Authors: Lisabeth V. Scalzi, Christopher S. Hollenbeak, Emily Mascuilli and Nancy Olsen

Development of neoplasms in pediatric patients with rheumatic disease exposed to anti-tumor necrosis factor therapies: a single Centre retrospective study

Anti-TNF (Tumor necrosis factor) therapy is effective in treating pediatric patients with refractory rheumatic disease. There is however a concern that anti-TNF usage may increase the risk of malignancy. Repor...

Authors: Alexandra Okihiro, Rachana Hasija, Lillia Fung, Bonnie Cameron, Brian M. Feldman, Ronald Laxer, Rayfel Schneider, Earl Silverman, Lynn Spiegel, Rae S. M. Yeung and Shirley M. L. Tse

Pediatric rheumatology infusion center: report on therapeutic protocols and infusions given over 4 Years with focus on adverse events over 1 Year

Children with chronic rheumatic disease often require intravenous (IV) therapy. Our center has instituted standardized protocols for use of IV medications in rheumatology patients. Herein, we introduce the the...

Authors: Surabhi S. Vinod, Annelle B. Reed, Jamelle Maxwell, Randy Q. Cron and Matthew L. Stoll

Varicella-zoster-virus vaccination in immunosuppressed children with rheumatic diseases using a pre-vaccination check list

The goal of this study was to apply the varicella zoster virus (VZV) vaccine to patients with pediatric rheumatic diseases (PRD) at risk for severe chickenpox, without interrupting their current immunosuppress...

Authors: Fabian Speth, Claas H. Hinze, Susanne Andel, Thomas Mertens and Johannes-Peter Haas

Clinical predictors of proteinuric remission following an LN flare - evidence from the UK JSLE cohort study

Proteinuria is a well-known risk factor for progression of renal dysfunction in a variety of chronic kidney diseases. In adult-onset Systemic Lupus Erytematosus (SLE) patients with lupus nephritis (LN), protei...

Authors: Eve M. D. Smith, Peng Yin, Andrea L. Jorgensen and Michael W. Beresford