Articles

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

Contradictory and weak evidence on the effectiveness of anti-emetics for MTX-intolerance in JIA-patients

Authors: Casper G. Schoemaker, E. H. Pieter van Dijkhuizen and Sebastiaan J. Vastert

Is palindromic rheumatism amongst children a benign disease?

Palindromic rheumatism is an idiopathic, periodic arthritis characterized by multiple, transient, recurring episodes. Palindromic rheumatism is well-characterized in adults, but has never been reported in pedi...

Authors: Yonatan Butbul-Aviel, Yosef Uziel, Nofar Hezkelo, Riva Brik and Gil Amarilyo

Successful treatment of methotrexate intolerance in juvenile idiopathic arthritis using eye movement desensitization and reprocessing – treatment protocol and preliminary results

Methotrexate (MTX), commonly used in juvenile idiopathic arthritis (JIA), frequently has to be discontinued due to intolerance with anticipatory and associative gastrointestinal adverse effects. Eye Movement D...

Authors: Lea Höfel, Bruno Eppler, Magdalena Storf, Elizabeth Schnöbel-Müller, Johannes-Peter Haas and Boris Hügle

Patient’s experiences with the care for juvenile idiopathic arthritis across Europe

To assess the views of juvenile idiopathic arthritis (JIA) patients and their parents on the care and treatment they receive in referral pediatric rheumatology centers throughout Europe.

Authors: E. H. Pieter. van Dijkhuizen, Tsipi Egert, Yona Egert, Wendy Costello, Casper Schoemaker, Marlous Fernhout, Mirjam Kepic, Alberto Martini, Silvia Scala, Ingrid Rotstein-Grein, Sebastiaan J. Vastert and Nico M. Wulffraat

Application of the international league against rheumatism classification criteria for systemic juvenile idiopathic arthritis as a prognostic factor in patients with adults-onset Still’s disease

Adult-onset Still’s disease (AOSD) is an adult form of systemic juvenile idiopathic arthritis (JIA) that differs from the latter in its classification. This study evaluated the concordance between the Internat...

Authors: Ji Won Yang, Eunyoung Lee, Ji-Yeon Seo, Ju-Yang Jung, Chang-Hee Suh and Hyoun-Ah Kim

Defining the normal appearance of the temporomandibular joints by magnetic resonance imaging with contrast: a comparative study of children with and without juvenile idiopathic arthritis

Up to 80% of children with juvenile idiopathic arthritis (JIA) develop arthritis involving their temporomandibular joint (TMJ). Recent studies have questioned the sensitivity of an abnormal MRI in the diagnosi...

Authors: Matthew L. Stoll, Saurabh Guleria, Melissa L. Mannion, Daniel W. Young, Stuart A. Royal, Randy Q. Cron and Yoginder N. Vaid

Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in Germany

Systemic juvenile idiopathic arthritis (SJIA) is an autoinflammatory disease associated with chronic arthritis. Early diagnosis and effective therapy of SJIA is desirable, so that complications are avoided. Th...

Authors: Claas H. Hinze, Dirk Holzinger, Elke Lainka, Johannes-Peter Haas, Fabian Speth, Tilmann Kallinich, Nikolaus Rieber, Markus Hufnagel, Annette F. Jansson, Christian Hedrich, Hanna Winowski, Thomas Berger, Ivan Foeldvari, Gerd Ganser, Anton Hospach, Hans-Iko Huppertz…

Pigmented villonodular synovitis in pediatric population: review of literature and a case report

Pigmented villonodular synovitis (PVNS) is a rare proliferative process in children that mostly affects the knee joint.

Authors: Mohsen Karami, Mehryar Soleimani and Reza Shiari

Coexistent sickle-cell anemia and autoimmune disease in eight children: pitfalls and challenges

Patients with sickle cell disease (SCD) present a defective activation of the alternate complement pathway that increases the risk of infection and is thought to predispose to autoimmune disease (AID). However...

Authors: Valerie Li-Thiao-Te, Florence Uettwiller, Pierre Quartier, Florence Lacaille, Brigitte Bader-Meunier, Valentine Brousse and Mariane de Montalembert

Homocysteine, folate, hs-C-reactive protein, tumor necrosis factor alpha and inflammatory proteins: are these biomarkers related to nutritional status and cardiovascular risk in childhood-onset systemic lupus erythematosus?

Childhood-onset systemic lupus erythematosus (c-SLE) is a chronic autoimmune disease which increases cardiovascular risk factors (CRF) such as elevated homocysteine, TNF-α, and hs-C reactive protein.

Authors: Roberta Garcia Salomão, Luciana Martins de Carvalho, Clarice Izumi, Érika Silva Czernisz, José César Rosa, Sonir Roberto Rauber Antonini, Ana Carolina Bueno, Maria Olímpia Ribeiro do Vale Almada, Carolina de Almeida Coelho-Landell, Alceu Afonso Jordão, Virgínia Paes Leme Ferriani and Jacqueline Pontes Monteiro

Prior to extension, Transcriptomes of fibroblast-like Synoviocytes from extended and Polyarticular juvenile idiopathic arthritis are indistinguishable

Our intent was to identify differences between the transcriptome of fibroblast-like synoviocytes (FLS) in oligoarticular juvenile idiopathic arthritis (JIA) before extension when compared to persistent subtype...

Authors: AnneMarie C. Brescia, Megan M. Simonds, Suzanne M. McCahan, Kathleen E. Sullivan and Carlos D. Rose

Patient characteristics associated with response to NSAID monotherapy in children with systemic juvenile idiopathic arthritis

Systemic juvenile idiopathic arthritis (sJIA) is an auto-inflammatory disease characterized by fever, arthritis, and ≥1 of rash, generalized lymphadenopathy, hepato/splenomegaly, and serositis. Non-steroidal a...

Authors: Anjali Sura, Christopher Failing, Julie Sturza, Jasmine Stannard and Meredith Riebschleger

Cone beam computed tomography for the assessment of linear scleroderma of the face

To date, standardized methods for assessing the disease progression of linear scleroderma of the face (LSF) are lacking.

Authors: C. Di Giovanni, S. Puggina, A. Meneghel, F. Vittadello, G. Martini and F. Zulian

“Next generation sequencing identifies mutations in GNPTG gene as a cause of familial form of scleroderma-like disease”

Authors: Afolake T. Arowolo, Henry A. Adeola and Nonhlanhla P. Khumalo

Dealing with Chronic Non-Bacterial Osteomyelitis: a practical approach

Chronic Non-Bacterial Osteomyelitis (CNO) is an inflammatory disorder that primarily affects children. Although underestimated, its incidence is rare. For these reasons, no diagnostic and no therapeutic guidel...

Authors: Andrea Taddio, Giovanna Ferrara, Antonella Insalaco, Manuela Pardeo, Massimo Gregori, Martina Finetti, Serena Pastore, Alberto Tommasini, Alessandro Ventura and Marco Gattorno

Pharmacokinetic and safety profile of tofacitinib in children with polyarticular course juvenile idiopathic arthritis: results of a phase 1, open-label, multicenter study

Juvenile idiopathic arthritis (JIA) is the most common pediatric rheumatic disease and a leading cause of childhood disability. The objective of this study was to characterize the PK, safety, and taste accepta...

Authors: Nicolino Ruperto, Hermine I. Brunner, Zbigniew Zuber, Nikolay Tzaribachev, Daniel J. Kingsbury, Ivan Foeldvari, Gerd Horneff, Elzbieta Smolewska, Richard K. Vehe, Anasuya Hazra, Rong Wang, Charles A. Mebus, Christine Alvey, Manisha Lamba, Sriram Krishnaswami, Thomas C. Stock…

Mental health care for youth with rheumatologic diseases – bridging the gap

Youth with rheumatologic diseases have a high prevalence of comorbid mental health disorders. Individuals with comorbid mental health disorders are at increased risk for adverse outcomes related to mental heal...

Authors: Alaina M. Davis, Tamar B. Rubinstein, Martha Rodriguez and Andrea M. Knight

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

Behçet’s disease is a rare multi-systemic inflammatory disease with unknown etiology which involves principally oral and genital mucosa, skin and eyes. Average age at onset of the disease is about 25-30 years,...

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…