Articles

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

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

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

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

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…

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

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

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

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

“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

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

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…

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…

Correction to: P92 chronic arthritis as the only manifestation of FMF in Armenian children

Familial Mediterranean Fever (FMF) is the most common inherited auto inflammatory disease, characterized by recurrent, self–limited attacks of fever and aseptic polyserositis.FMF is widespread in Armenia and t...

Authors: Gayane Khloyan, Gayane Amaryan and Rotraud Saurenmann

Anatomy of the sacroiliac joints in children and adolescents by computed tomography

Diagnosing sacroiliitis by magnetic resonance imaging (MRI) in children/adolescents can be difficult due to the growth-related changes. This study analyzed the normal osseous anatomy of the sacroiliac joints (...

Authors: Anna Zejden and Anne Grethe Jurik

Functional resonance magnetic imaging (fMRI) in adolescents with idiopathic musculoskeletal pain: a paradigm of experimental pain

Studies on functional magnetic resonance imaging (fMRI) have shown that adults with musculoskeletal pain syndromes tolerate smaller amount of pressure (pain) as well as differences in brain activation patterns...

Authors: Juliana Molina, Edson Amaro Jr, Liana Guerra Sanches da Rocha, Liliana Jorge, Flavia Heloisa Santos and Claudio A. Len

Patients with juvenile idiopathic arthritis in clinical remission with positive power Doppler signal in joint ultrasonography have an increased rate of clinical flare: a prospective study

Ultrasonography (US) studies carried out on joints of juvenile idiopathic arthritis (JIA) patients in clinical remission demonstrate the presence of subclinical synovitis. The significance of subclinical synov...

Authors: Vanessa Bugni Miotto e Silva, Sônia de Aguiar Vilela Mitraud, Rita Nely Vilar Furtado, Jamil Natour, Claudio Arnaldo Len and Maria Teresa de Sande e Lemos Ramos Ascensão Terreri

Risk of tuberculosis among Alabama children and adolescents treated with tumor necrosis factor inhibitors: a retrospective study

Tumor Necrosis Factor inhibitors (TNFi) have dramatically improved the outlook for patients with inflammatory arthritides and bowel disease (IBD), but are associated with increased infection risks, including t...

Authors: Matthew L. Stoll, James Aaron Grubbs, Timothy Beukelman, Melissa L. Mannion, Traci W. Jester, Randy Q. Cron and Marilyn J. Crain

Protocols on classification, monitoring and therapy in children’s rheumatology (PRO-KIND): results of the working group Polyarticular juvenile idiopathic arthritis

Several effective pharmacologic treatment options for polyarticual juvenile idiopathic arthritis (JIA) have emerged but initial treatment is heterogeneous in Germany. Therefore, the German Society of Pediatric...

Authors: Gerd Horneff, Ariane Klein, Gerd Ganser, Michaela Sailer-Höck, Annette Günther, Ivan Foeldvari and Frank Weller-Heinemann

Effective induction therapy for anti-SRP associated myositis in childhood: A small case series and review of the literature

Anti-Signal Recognition Particle associated myopathy is a clinically and histopathologically distinct subgroup of Juvenile Idiopathic Inflammatory Myositis, which is under-recognised in children and fails to r...

Authors: E. L. Binns, E. Moraitis, S. Maillard, S. Tansley, N. McHugh, T. S. Jacques, L. R. Wedderburn, C. Pilkington, S. A. Yasin and K. Nistala

H syndrome: 5 new cases from the United States with novel features and responses to therapy

H Syndrome is an autosomal recessive disorder characterized by cutaneous hyperpigmentation, hypertrichosis, and induration with numerous systemic manifestations. The syndrome is caused by mutations in SLC29A3, a ...

Authors: Jessica L. Bloom, Clara Lin, Lisa Imundo, Stephen Guthery, Shelly Stepenaskie, Csaba Galambos, Amy Lowichik and John F. Bohnsack

Short term effectiveness and experiences of a peer guided web-based self-management intervention for young adults with juvenile idiopathic arthritis

A web-based self-management intervention guided by peer-trainers was developed to support young adults’ self-management in coping with Juvenile Idiopathic Arthritis (JIA). To investigate its effectiveness, a r...

Authors: Judy Ammerlaan, Harmieke van Os-Medendorp, Nienke de Boer-Nijhof, Lieske Scholtus, Aike A. Kruize, Philomine van Pelt, Berent Prakken and Hans Bijlsma

Effects of age and gender on reference levels of biomarkers comprising the pediatric Renal Activity Index for Lupus Nephritis (p-RAIL)

Systemic Lupus Erythematosus (SLE) is a multisystem autoimmune disease that disproportionately effects women and children of minorities. Renal involvement (lupus nephritis, or LN) occurs in up to 80% of childr...

Authors: Michael R. Bennett, Qing Ma, Jun Ying, Prasad Devarajan and Hermine Brunner

Readout-segmented multi-shot diffusion-weighted MRI of the knee joint in patients with juvenile idiopathic arthritis

Diffusion-weighted MRI has been proposed as a new technique for imaging synovitis without intravenous contrast application. We investigated diagnostic utility of multi-shot readout-segmented diffusion-weighted...

Authors: Alexander Sauer, Mengxia Li, Annette Holl-Wieden, Thomas Pabst and Henning Neubauer

Next Generation Sequencing identifies mutations in GNPTG gene as a cause of familial form of scleroderma-like disease

Scleroderma is a multisystem disease, characterized by fibrosis of skin and internal organs, immune dysregulation, and vasculopathy. The etiology of the disease remains unknown, but it is likely multifactorial...

Authors: Abdelali Zrhidri, Saadia Amasdl, Jaber Lyahyai, Hanane Elouardi, Bouchra Chkirate, Laure Raymond, Grégory Egéa, Mohamed Taoudi, Said El Mouatassim and Abdelaziz Sefiani

Assessment, classification and treatment of calcinosis as a complication of juvenile dermatomyositis: a survey of pediatric rheumatologists by the childhood arthritis and rheumatology research alliance (CARRA)

There is no standardized approach to the management of JDM-associated calcinosis and its phenotypes. Current knowledge of treatment outcomes is confined to small series and case reports. We describe physician ...

Authors: A. B. Orandi, K. W. Baszis, V. R. Dharnidharka, A. M. Huber and M. F. Hoeltzel

Assessment of transition readiness in adolescents and young adults with chronic health conditions

Transition from pediatric to adult health care is a vulnerable period for adolescents and young adults. Challenges include paucity of validated measures to assess patients’ transition readiness. We evaluated t...

Authors: Paul T. Jensen, Gabrielle V. Paul, Stephanie LaCount, Juan Peng, Charles H. Spencer, Gloria C. Higgins, Brendan Boyle, Manmohan Kamboj, Christopher Smallwood and Stacy P. Ardoin

Comics as an educational tool for children with juvenile idiopathic arthritis

This study examined whether the comic book Neta and the Medikidz Explain JIA would improve disease-related knowledge and treatment adherence among patients with juvenile idiopathic arthritis (JIA).

Authors: Amir Mendelson, Noa Rabinowicz, Yonit Reis, Gil Amarilyo, Liora Harel, Philip J Hashkes and Yosef Uziel

Proceedings of the 24^th Paediatric Rheumatology European Society Congress: Part three

Authors: Erato Atsali, Dimitra Kassara, Pelagia Katsimbri, Sorina Boiu, Dimitrios T. Boumpas, Vana Papaevangelou, Olena A. Oshlyanska, Ludmila I. Omelchenko, Tatiana A. Ljudvik, Katerina Bouchalova, Marcel Schüller, Jana Franova, Jarmila Skotakova, Marie Macku, Antoni Fellas, Fiona Hawke…

Proceedings of the 24^th Paediatric Rheumatology European Society Congress: Part one

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Proceedings of the 24^th Paediatric Rheumatology European Society Congress: Part two

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