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

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

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

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

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

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

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

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

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…

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

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

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