Journals Pediatric Rheumatology Online ...

Pediatric Rheumatology Online Journal
Kati Räsänen, Kati Markula-Patjas, Saija Kantanen, Kalle Sipilä, Timo A Lakka, Pekka Arikoski, Eija Piippo-Savolainen
BACKGROUND: In recent years, biologic drug therapies have altered the course of juvenile idiopathic arthritis (JIA) possibly also improving the patients' physical fitness. However, studies measuring both cardiorespiratory and muscular fitness in children with JIA are sparse and have failed to show consistent results. Our aim was to assess both cardiorespiratory and neuromuscular fitness and contributing factors in children and adolescents with JIA in the era of biologic drug therapies...
March 17, 2023: Pediatric Rheumatology Online Journal
Tianyu Zhang, Zhongxun Yu, Sihao Gao, Yuelun Zhang, Changyan Wang, Shan Jian, Lin Wang, Lijuan Gou, Ji Li, Mingsheng Ma, Hongmei Song
BACKGROUND: Cytomegalovirus (CMV) plays an important role in the pathogenesis of systemic lupus erythematosus (SLE). However, it is not clear whether the anti-CMV treatment has an impact on the prognosis of SLE patients with CMV infection. We aimed to analyze the clinical characteristics and prognosis of CMV infection in pediatric SLE (pSLE) and to evaluate the effect of anti-CMV treatment on pSLE outcome. METHODS: A retrospective study including 146 pSLE from 2012 to 2021 was conducted...
March 16, 2023: Pediatric Rheumatology Online Journal
Aurélie Chausset, Céline Lambert, Alexandre Belot, Etienne Merlin, Elvira Cannizzaro, Isabelle Kone-Paut, Claire Ballot, Valérie Devauchelle, Sylvaine Poignant, Raffaella Carlomagno, Anne Lohse, Catherine Barbier, Véronique Despert, Aurélia Carbasse, Laetitia Sparsa, Eva Adank, Federica Vanoni, Héloise Reumaux, Pascal Pillet, Daniela Kaiser, Michael Hofer, Caroline Freychet, Anne-Marie Schott
BACKGROUND: Despite guidelines, poor access to appropriate care for juvenile idiopathic arthritis (JIA) patients remains a global issue. Prompt referral to a pediatric rheumatology (PR) center and effective care is known to be critical for changing the natural history of the disease and improving long-term prognosis. This project assesses socio-economic factors of delayed referral to a pediatric rheumatologist (PRst) for JIA patients in France and Switzerland within the Juvenile Inflammatory Rheumatism (JIR) Cohort...
March 14, 2023: Pediatric Rheumatology Online Journal
Qiaoqian Zeng, Haimei Liu, Guomin Li, Yifan Li, Wanzhen Guan, Tao Zhang, Yinv Gong, Xiaomei Zhang, Qianying Lv, Bingbing Wu, Hong Xu, Li Sun
BACKGROUND: Blau syndrome is a rare autoinflammatory disease caused by autosomal dominant mutations in the CARD15/NOD2 gene. Vascular involvement is a rare phenotype in Blau syndrome patients. In this study, we aimed to describe a 20-year- old Chinese girl with Blau syndrome complicated by renal arteritis. In addition, we summarized a literature review of published cases of vascular involvement in patients with Blau syndrome. CASE PRESENTATION: We describe a 20-year-old girl who was initially misdiagnosed with juvenile idiopathic arthritis (JIA) almost 15 years prior...
March 13, 2023: Pediatric Rheumatology Online Journal
Xiangrui Sheng, Xiaohui Song, Yue Xiong, Tian Ren, Xin Chang, Jian Wu, Jing Cao, Tao Cheng, Mingjun Wang
OBJECTIVE: The relationship between anti-SSA/RO antibodies and pregnancy has been reported previously, and we aim to visualize the rates of maternal and infant outcomes with anti-SSA/RO. METHODS: We systematically searched records from Pubmed, Cochrane, Embase, and Web of Science databases, pooled incidence rates of adverse outcomes of pregnancy, and 95% confidence intervals (CIs) were performed with RStudio. RESULTS: A total of 890 records comprising 1675 patients and 1920 pregnancies were searched from the electronic databases...
March 4, 2023: Pediatric Rheumatology Online Journal
D A Al-Julandani, N K Bagri, N Tsang, S Clarke, A Upadhyay, C Guly, A V Ramanan
BACKGROUND: Adalimumab in combination with other disease-modifying antirheumatic drugs (DMARD) such as methotrexate has a proven efficacy in the management of paediatric non-infectious uveitis. However, many children experience significant intolerance to methotrexate while on this combination, leaving a dilemma for clinicians for choosing the subsequent therapeutic roadmap. Continuation of adalimumab monotherapy might be an alternative feasible option under such settings. This study aims to investigate the efficacy of adalimumab monotherapy in paediatric non-infectious uveitis...
March 2, 2023: Pediatric Rheumatology Online Journal
Gerd Horneff, Kirsten Minden, Catherine Rolland, Ana C Hernandez Daly, Cecilia Borlenghi, Nicolino Ruperto
OBJECTIVE: A systematic literature review was conducted to summarize efficacy and safety data from studies that evaluated tumor necrosis factor inhibitors in patients with juvenile idiopathic arthritis (JIA). METHODS: Relevant publications were identified via online searches (cutoff: March 16, 2021). After screening search results, outcome data were extracted if the treatment arm included ≥ 30 patients. Outcomes were described narratively, with efficacy assessed by JIA-American College of Rheumatology (ACR) response criteria and safety assessed by the incidence of serious adverse events (SAEs) per 100 patient-years (100PY)...
February 24, 2023: Pediatric Rheumatology Online Journal
Joeri W van Straalen, Laurie Baas, Gabriella Giancane, Lyudmila Grebenkina, Jurgen Brunner, Gabriel Vega-Cornejo, Vyacheslav G Chasnyk, Liora Harel, Simone Appenzeller, Elisabeth Gervais, Sytze de Roock, Nico M Wulffraat, Nicolino Ruperto, Joost F Swart
BACKGROUND: Little is known about the association between juvenile idiopathic arthritis (JIA) and autoimmune thyroid disease (AITD) and therefore there are no indications for AITD screening in this population, which is possible using standard blood tests. The objective of this study is to determine the prevalence and predictors of symptomatic AITD in JIA patients from the international Pharmachild registry. METHODS: Occurrence of AITD was determined from adverse event forms and comorbidity reports...
February 21, 2023: Pediatric Rheumatology Online Journal
Kathleen J Imbach, Nicole J Treadway, Vaishali Prahalad, Astrid Kosters, Dalia Arafat, Meixue Duan, Talia Gergely, Lori A Ponder, Shanmuganathan Chandrakasan, Eliver E B Ghosn, Sampath Prahalad, Greg Gibson
BACKGROUND: Juvenile Idiopathic Arthritis (JIA) is an autoimmune disease with a heterogenous clinical presentation and unpredictable response to available therapies. This personalized transcriptomics study sought proof-of-concept for single-cell RNA sequencing to characterize patient-specific immune profiles. METHODS: Whole blood samples from six untreated children, newly diagnosed with JIA, and two healthy controls were cultured for 24 h with or without ex vivo TNF stimulation and subjected to scRNAseq to examine cellular populations and transcript expression in PBMCs...
February 15, 2023: Pediatric Rheumatology Online Journal
Giorgia Martini, Alessandra Meneghel, Michele Fastiggi, Filippo Dell'Apa, Fabio Vittadello, Francesco Zulian
OBJECTIVE: To evaluate the efficacy of levofolinic acid (LVF) administered 48 h before methotrexate (MTX) in reducing gastrointestinal side effects without interference with drug efficacy. METHODS: A prospective observational study was performed including patients with Juvenile Idiopathic Arthritis (JIA) reporting significant gastrointestinal discomfort after MTX despite taking a dose of LVF 48 h after MTX. Patients with anticipatory symptoms were excluded...
February 15, 2023: Pediatric Rheumatology Online Journal
Moustafa Ali Saad, Hamdy Ahmed, Rasmia Elgohary, Hala Ibrahem El Gendy
BACKGROUND: IgG4-related disease (IgG4-RD) is a progressive and sometimes fatal disease that rarely affects pediatric age group. It may affect the orbits, lacrimal and salivary glands, pancreas, kidneys, peritoneum and other organs. Lung and pleura are not commonly reported in IgG4-RD. We here present a rare case of pediatric IgG4-RD with rare involvement of pericardium, pleura and lungs. CASE PRESENTATION: A 13-year-old girl presented with intrathoracic IgG4-RD with pleuropericardial involvement...
February 13, 2023: Pediatric Rheumatology Online Journal
Jia Liu, Ranran Zhang, Zhi Yi, Yi Lin, Hong Chang, Qiuye Zhang
BACKGROUND: Cryopyrin-associated periodic syndrome (CAPS), a rare genetic autoimmune disease, is composed of familial cold autoinflammatory syndrome (FCAs), Muckle-Wells syndrome (MWS), and neonatal onset multisystem inflammatory disease (NOMID). MWS is caused by dominantly inherited or de novo gain-of-function mutations in the NOD-like receptor 3 (NLRP3) gene. At present, there is no report about the variation of R262W in China. CASE PRESENTATION: We reported a 3-year-old Chinese boy who had recurrent fever without obvious inducement, bilateral conjunctival congestion, and urticarial-like rash...
February 10, 2023: Pediatric Rheumatology Online Journal
K Khawaja, R Kalas, N Almasri
BACKGROUND: Juvenile Idiopathic Arthritis is a chronic inflammatory disease that affects 1 in 1000 children worldwide. Our population in the United Arab Emirates is diverse. The objective of this study is to describe the subtype frequency, demographic features and treatments received and outcome of our patients. METHODS: Patients with the diagnosis of Juvenile Arthritis identified through the hospital electronic medical records system (EMR), which was implemented for all medical documentation in January 2011...
February 7, 2023: Pediatric Rheumatology Online Journal
Bethany Gillies Whiteside, Hannah Titheradge, Eslam Al-Abadi
No abstract text is available yet for this article.
February 6, 2023: Pediatric Rheumatology Online Journal
Yanli Guo, Yuying Fang, Tonghao Zhang, Yuting Pan, Panpan Wang, Zhidan Fan, Haiguo Yu
BACKGROUND: Axial involvement in children with enthesitis-related arthritis (ERA) has characteristics that differ from those of peripheral involvement. This study characterized their clinical characteristics and treatment. METHODS: Patients with ERA at the Children's Hospital of Nanjing Medical University between January 2018 and December 2020 were included. The ERA cohort was divided into two based on the presence or absence of axial joint involvement. Demographic characteristics, clinical features, and treatments were described and compared...
February 6, 2023: Pediatric Rheumatology Online Journal
Yinv Gong, Haimei Liu, Guomin Li, Tao Zhang, Yifan Li, Wanzhen Guan, Qiaoqian Zeng, Qianying Lv, Xiaomei Zhang, Wen Yao, Yu Shi, Hong Xu, Li Sun
OBJECTIVES: To characterize the clinical features and outcomes of childhood-onset primary Sjögren's syndrome (pSS). METHODS: Patients less than 18 years old who were diagnosed with pSS by paediatric rheumatologists were included, and all patients were applied the 2002 American-European Consensus Group (ACEG) criteria, the 2016 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria for pSS, or the 1999 proposed juvenile pSS criteria...
January 27, 2023: Pediatric Rheumatology Online Journal
Zixuan Shen, Jiayun Ling, Xiaona Zhu, Jun Yang, Tingyan He
BACKGROUND: Kikuchi-Fujimoto disease (KFD) is typically a benign, self-limiting inflammatory disease. However, some patients may have a prolonged or recurrent disease course, or present with life-threatening complications such as macrophage activation syndrome (MAS). In this study, we aimed to describe the incidence and clinical features of MAS in KFD and to access potential laboratory markers for the diagnosis of KFD-associated MAS. METHODS: Patients with KFD were retrospectively enrolled from January 2015 to November 2021 at Shenzhen Children's Hospital...
January 25, 2023: Pediatric Rheumatology Online Journal
Maria Backström, Hanna Vuorimaa, Maarit Tarkiainen, Eliisa Löyttyniemi, Liisa Kröger, Kristiina Aalto, Katariina Rebane, Kati Markula-Patjas, Merja Malin, Sirja Sard, Paula Keskitalo, Katja Korkatti, Minna-Maija Grönlund, Milja Möttönen, Heini Pohjankoski, Maiju Hietanen, Johanna Kärki, Paula Vähäsalo
BACKGROUND: In a chronic pain-causing disease such as juvenile idiopathic arthritis, the quality of coping with pain is crucial. Parents have a substantial influence on their children's pain-coping strategies. This study aimed to develop scales for assessing parents' strategies for coping with their children's pain and a shorter improved scale for children usable in clinical practice. METHODS: The number of items in the Finnish version of the pain-coping questionnaire for children was reduced from 39 to 20...
January 25, 2023: Pediatric Rheumatology Online Journal
İlknur Bağrul, Serdar Ceylaner, Yasemin Tasci Yildiz, Serife Tuncez, Elif Arslanoglu Aydin, Esra Bağlan, Semanur Ozdel, Mehmet Bülbül
BACKGROUND: Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome, caused by biallelic pathogenic mutations in the proteoglycan 4 (PRG4) gene, is characterized by early-onset camptodactyly, noninflammatory arthropathy, coxa vara deformity, and rarely, pericardial effusion. This syndrome can mimic juvenile idiopathic arthritis. CACP syndrome is caused by mutations in the proteoglycan 4 (PRG4) gene. To date, only 36 pathogenic mutations have been reported in this gene, but none have been reported from Azerbaijan...
January 24, 2023: Pediatric Rheumatology Online Journal
Lien De Somer, Brigitte Bader-Meunier, Sylvain Breton, Sara Brachi, Carine Wouters, Francesco Zulian
BACKGROUND: Dry synovitis (DS) is a rare entity as only a few cases have been reported to date. We describe the clinical features, radiological manifestations and course of DS in comparison with rheumatoid factor negative polyarticular juvenile idiopathic arthritis (RFneg-polyJIA). METHODS: We performed a multicenter retrospective collection of data of DS patients who presented with progressive joint limitations without palpable synovitis, absence of elevated acute phase reactants, negative ANA and RF, and imaging showing joint and/or osteochondral involvement...
January 23, 2023: Pediatric Rheumatology Online Journal
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