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Rev Prat 2019 Feb;69(2):188-194

Rhumatologie pédiatrique, hôpital Necker- Enfants malades, Paris, France.

Juvenile idiopathic arthritis. Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory rheumatic disease in childhood. It comprises a heterogeneous group of disorders characterized by joint inflammation, extraarticular features including uveitis and, in some cases laboratory markers. Read More

Child Care Health Dev 2019 Apr 14. Epub 2019 Apr 14.

School of Public Health and Health Systems, University of Waterloo, Waterloo, Ontario, Canada.

Background: Evidence suggests that physical and mental illness are strongly correlated in children. This study examined patterns of the chronicity of multimorbidity (co-occurring physical and mental illness); estimated homotypic continuity; and, modelled factors associated with chronicity in children newly diagnosed with a chronic physical illness.

Methods: Children aged 6-16 years diagnosed with one of asthma, diabetes, epilepsy, food allergy, or juvenile arthritis were recruited from two children's hospitals and followed for six months. Read More

Pediatr Rheumatol Online J 2019 Apr 11;17(1):15. Epub 2019 Apr 11.

Department of Lifetime Clinical Immunology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8510, Japan.

Background: Although there are many reports on Juvenile Idiopathic arthritis-associated uveitis (JIA-U) from various countries, especially from Europe and North America, there are few reports from Asia. Our aim was to investigate the epidemiology, characteristics and predictors of JIA-U in Japan.

Methods: Data were retrospectively collected on 726 patients with JIA from medical records as of April 2016 at 15 medical centers specialized in pediatric rheumatic diseases. Read More

Arthritis Care Res (Hoboken) 2019 Apr 11. Epub 2019 Apr 11.

Section of Orthodontics, Aarhus University, Denmark and Department of Oral and Maxillofacial Surgery, Aarhus University Hospital, Aarhus, Denmark.

Objectives: To estimate the cumulative incidence of arthritis-induced orofacial symptoms, dysfunctions, and dentofacial deformities in growing individuals with juvenile idiopathic arthritis (JIA) in a 36 month regional cohort study, and to identify predictors for the development of arthritis-induced dentofacial deformities.

Methods: Data was retrieved from the Aarhus JIA TMJ cohort register, which contains standardized, longitudinal, observational data regarding orofacial conditions in patients with JIA (n=1040). This regional cohort represents the majority of all subjects with JIA from the western part of Denmark between 1990 and 2016, regardless of temporomandibular joint (TMJ) arthritis status. Read More

Br J Dermatol 2019 Apr 10. Epub 2019 Apr 10.

Centre of Evidence Based Dermatology, University of Nottingham, Nottingham, UK.

Psoriasis in children can be a challenging diagnosis: the clinical presentation is often more subtle, may occur in covered sites and can be an unexpected diagnosis as psoriasis if often thought to occur at older ages. Poor recognition and delayed diagnosis of psoriasis in children can lead to inadequate treatment and lack of monitoring for comorbidities including juvenile psoriatic arthritis. Diagnostic criteria would help both clinical practice and clinical research, but to date none are available. Read More

Front Genet 2019 27;10:181. Epub 2019 Mar 27.

Department of Cell Biology, 2011 Collaborative Innovation Center of Tianjin for Medical Epigenetics, Tianjin Key Laboratory of Medical Epigenetics, Tianjin Medical University, Tianjin, China.

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease among children which could cause severe disability. Genomic studies have discovered substantial number of risk loci for JIA, however, the mechanism of how these loci affect JIA development is not fully understood. Neutrophil is an important cell type involved in autoimmune diseases. Read More

Case Rep Orthop 2019 6;2019:4593129. Epub 2019 Mar 6.

Department of Orthopaedic Surgery, University of California at Davis, 4860 Y Street, Suite 3800, Sacramento, CA 95829, USA.

We report a novel case of a pediatric patient with bilateral hip destruction from untreated Juvenile idiopathic arthritis (JIA). She was presented at the age of 9 with hip pain associated with bilateral acetabular dysplasia and a dislocated left femoral head. Only 1. Read More

Pediatr Rheumatol Online J 2019 Apr 8;17(1):14. Epub 2019 Apr 8.

Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway.

Background: Poor cardiorespiratory fitness is previously reported in patients with juvenile idiopathic arthritis (JIA) measured both by maximal and submaximal exercise tests, but a submaximal exercise test with acceptable measurement properties is currently lacking for both clinical and research purposes in this patient population. The objectives of this study were to evaluate the measurement properties and performance of a submaximal treadmill test in patients with JIA, and to compare the results with those obtained in controls.

Methods: Fifty-nine patients (50 girls), aged 10-16 years, with oligo- (n = 30) and polyarticular (n = 29) JIA, and 59 age- and sex-matched controls performed an eight-minute submaximal treadmill test for estimating peak oxygen uptake (VO) followed by a maximal treadmill test measuring VO directly. Read More

J AAPOS 2019 Apr 5. Epub 2019 Apr 5.

Vitreo-retina, Trauma and Uvea Services; Dr. Rajendra Prasad Center for Ophthalmic Sciences; All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.

Juvenile idiopathic arthritis (JIA) is a group of disorders occurring in children with arthritis of more than 6 weeks' duration. JIA may have varied systemic and ocular presentations, which can pose a diagnostic challenge. Chronic uveitis with insidious onset is the most common type of ocular presentation. Read More

Indian Pediatr 2019 Mar;56(3):242-243

Department of Pediatric and Adolescent Endocrinology, Institute of Pediatrics, Jagiellonian University Medical College, and Children's University Hospital, Krakow, Poland.

Background: The most common cause of hypothalamic-pituitary-adrenal axis suppression is systemic glucocorticoids administration.

Case Characteristics: A 14-year-old boy with juvenile idiopathic arthritis receiving repeated intra-articular steroids for last 3 years developed fever, fatigue, nausea and abdominal pain. Stimulation with low-dose Synathen revealed low adrenal reserve, suggesting secondary adrenal insufficiency. Read More

Cytokine 2019 Apr 2;119:182-187. Epub 2019 Apr 2.

Department of Pediatrics, Graduate School of Medical Sciences, Kanazawa University, Japan.

To clarify cytokines involved in the development of systemic juvenile idiopathic arthritis (s-JIA) associated macrophage activation syndrome (MAS) and to identify the serum biomarkers for the diagnosis of s-JIA associated MAS, we employed an antibody array that simultaneously detects 174 cytokines. Fifteen s-JIA patients including 5 patients receiving tocilizumab (TCZ) were analyzed. The levels of five cytokines were significantly elevated in MAS phase compared to those in the active phase of s-JIA. Read More

Pediatrics 2019 Apr 4. Epub 2019 Apr 4.

Department of Rheumatology and Immunology, Guangdong Second Provincial General Hospital, Guangzhou, China; and

Systemic juvenile idiopathic arthritis (sJIA) is an aggressive form of childhood arthritis accompanied by persistent systemic inflammation. Patients with sJIA often exhibit poor response to conventional disease-modifying antirheumatic drugs, and chronic glucocorticoid use is associated with significant adverse effects. Although biologics used to target interleukin 1 and interleukin 6 are efficacious, the long-term commitment to frequent injections or infusions remains a challenge in young children. Read More

J Arthroplasty 2019 Mar 12. Epub 2019 Mar 12.

Adult Reconstruction and Joint Replacement Service, Hospital for Special Surgery, New York, NY.

Background: The purpose of this study was to identify reasons for revision of total hip arthroplasty (THA) in patients who underwent primary THA at or before the age of 35 years. We hypothesized that the reasons for revision in younger patients would be different from the general older population of patients undergoing THA because of the differences in diagnoses, complexity of deformities, and differences in activity level.

Methods: Data for 108 hips in 82 patients who underwent primary THA at our institution before the age of 35 years from 1982-2007 and subsequently underwent revision THA were reviewed. Read More

Psychiatr Danub 2019 Mar;31(Suppl 1):44-49

Zagreb University Hospital Centre, Department of Ophthalmology, Kišpatićeva 12, 10000 Zagreb, Croatia,

JIA is the most common rheumatic disease of childhood and JIA-U is its most frequent and most devastating extraarticular manifestation. This form of uveitis is usually asymptomatic, chronic anterior uveitis, often accompained with complications. JIA-U is the main cause of vision loss and even blidness in childhood. Read More

Int J Rheum Dis 2019 Apr 3;22(4):750-751. Epub 2019 Apr 3.

Department of Clinical Immunology and Rheumatology, Christian Medical College & Hospital, Vellore, India.

Br J Ophthalmol 2019 Apr 2. Epub 2019 Apr 2.

The First Affiliated Hospital of Chongqing Medical University, Chongqing Key Laboratory of Ophthalmology and Chongqing Eye Institute, Chongqing, P. R. China

Background: Idiopathic paediatric uveitis (IPU) and juvenile idiopathic arthritis associated uveitis (JIA-U) are the two most common entities in paediatric uveitis. This study addressed the possible association of IPU and JIA-U with genes that had been shown earlier to be associated with juvenile idiopathic arthritis.

Methods: We carried out a case-control association study involving 286 IPU, 134 JIA-U patients and 743 healthy individuals. Read More

Cochrane Database Syst Rev 2019 Apr 2;4:CD011118. Epub 2019 Apr 2.

Cochrane Pain, Palliative and Supportive Care Group, Pain Research Unit, Churchill Hospital, Oxford, UK.

Background: This is the first update of a review published in 2015, Issue 1. Chronic pain is common during childhood and adolescence and is associated with negative outcomes, such as increased severity of pain, reduced function, and low mood. Psychological therapies, traditionally delivered face-to-face with a therapist, are efficacious at reducing pain intensity and disability. Read More

Immunol Med 2018 Mar 3;41(1):43-45. Epub 2018 Apr 3.

a Department of Pediatrics , Yamaguchi University Graduate School of Medicine , Ube , Japan.

Acute rheumatic fever (ARF), caused by group A β-hemolytic streptococcus infection, is characterized by inflammation affecting several organs. There are few reports on magnetic resonance imaging (MRI) findings in patients with ARF. An 8-year-old Japanese boy presented with a prolonged fever of unknown cause and swelling of his right hand. Read More

Indian J Pediatr 2019 Apr 1. Epub 2019 Apr 1.

Pediatric Nephrology Unit, Department of Pediatrics, Institute of Child Health, Kolkata, West Bengal, India.

Objectives: To assess the efficacy of cyclosporine (CsA) in patients of oral steroid unresponsive or steroid dependent systemic juvenile idiopathic arthritis (sJIA); to evaluate the optimum dosage and blood level of CsA to achieve and maintain remission and to observe for side-effects on prolonged usage.

Methods: This prospective observational study was conducted on children with steroid dependent /refractory sJIA admitted at the Institute of Child Health, Kolkata from July 2009 through November 2014. A total of 82 sJIA was diagnosed; 15 were steroid dependent /refractory and were included as candidates for cyclosporine therapy. Read More

World Allergy Organ J 2019 14;12(3):100019. Epub 2019 Mar 14.

Department of Dermatology and Allergy, Charité - Universitätsmedizin Berlin, Germany.

Background: Systemic autoinflammatory diseases (SAIDs) are rare debilitating disorders of which there is limited awareness and a significant delay in diagnosis. There is no uniform approach in the diagnosis and treatment of these disorders and the real life state of SAID patient care is poorly characterized. The aim of this study was to obtain data on the epidemiology, state of care and the perception of physicians who are involved in the care of SAID patients. Read More

World Allergy Organ J 2019 22;12(3):100018. Epub 2019 Mar 22.

Division of Allergy Immunology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Background: X-linked agammaglobulinemia is an inherited immunodeficiency recognized since 1952. In spite of seven decades of experience, there is still a limited understanding of regional differences in presentation and complications. This study was designed by the Primary Immunodeficiencies Committee of the World Allergy Organization to better understand regional needs, challenges and unique patient features. Read More

Front Immunol 2019 14;10:450. Epub 2019 Mar 14.

Department of Experimental and Clinical Medicine and DENOTHE Center, University of Florence, Florence, Italy.

In the last years much attention has focused on the Th17 and Th1 phenotypes and on their pathogenic role in juvenile idiopathic arthritis, investigating how the cytokines produced by T helper cells act on resident cells on the synovia and which signal transduction pathways regulate Th17 cells proliferation and plasticity. In this context, an important milestone was represented by the identification of the non-classic Th1 phenotype, developed from the shift of Th17 cells. The cytokine TNF-α, beyond its well-known proinflammatory activity is involved in this process and this is one of the reasons why the TNF-α inhibitors are widely used in the treatment of juvenile idiopathic arthritis patients. Read More

Hum Reprod 2019 Mar 30. Epub 2019 Mar 30.

Virus, Lifestyle and Genes, Danish Cancer Society Research Center, Copenhagen, Denmark.

Study Question: Is the risk of juvenile idiopathic arthritis (JIA) increased in children conceived after fertility treatment, and is an observed association caused by specific types of fertility treatment or by factors associated with the underlying infertility?

Summary Answer: The risk of JIA in children conceived after fertility treatment (any and specific types of fertility treatment) was not convincingly affected when compared with children born to fertile women.

What Is Known Already: It has been suggested that fertility treatment may affect the development of the immune system and thereby increase the risk of developing autoimmune diseases, including JIA.

Study Design, Size, Duration: This retrospective population-based cohort study included all live-born children in Denmark between 1 January 1996 and 31 December 2012 (n = 1 084 184). Read More

BMC Med Genet 2019 Mar 29;20(1):53. Epub 2019 Mar 29.

Metabolic Disease Unit, Edmond and Lily Safra Children's Hospital, Sheba Medical Center, 52621, Tel-Hashomer, Israel.

Background: Progressive pseudorheumatoid dysplasia (PPRD) is a rare autosomal-recessive, non-inflammatory arthropathy, shown to be caused by mutations in the WNT1-inducible signaling pathway protein 3 (WISP3) gene. Although several hundred cases were reported worldwide, the diagnosis remains challenging. Subsequently, the syndrome is often unrecognized and misdiagnosed (for instance, as Juvenile Idiopathic Arthritis), leading to unnecessary procedures and treatments. Read More

Klin Monbl Augenheilkd 2019 Apr 27;236(4):407-409. Epub 2019 Mar 27.

Ophthalmology, University of Lausanne, Hopital Ophtalmique Jules Gonin, Lausanne, Switzerland.

Sci Rep 2019 Mar 26;9(1):5165. Epub 2019 Mar 26.

Department of Orthopaedic Surgery, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan.

Juvenile tissue healing is capable of extensive scarless healing that is distinct from the scar-forming process of the adult healing response. Although many growth factors can be found in the juvenile healing process, the molecular mechanisms of juvenile tissue healing are poorly understood. Here we show that juvenile mice deficient in the chemokine receptor CCR7 exhibit diminished large-scale healing potential, whereas CCR7-depleted adult mice undergo normal scar-forming healing similar to wild type mice. Read More

Pediatr Phys Ther 2019 04;31(2):232

J Scleroderma Relat Disord 2019 Feb 18;4(1):17-27. Epub 2018 Jul 18.

Rheumatology Unit, University Hospital of Cagliari.

The Scleroderma Clinical Trials Consortium (SCTC) represents many of the clinical researchers in the world who are interested in improving the efficiency of clinical trials in Systemic Sclerosis (SSc). The SCTC has established 11 working groups (WGs) to develop and validate better ways of measuring and recording multiple aspects of this heterogeneous disease. These include groups working on arthritis, disease damage, disease activity, cardiac disease, juvenile SSc, the gastrointestinal tract, vascular component, calcinosis, scleroderma renal crisis, interstitial lung disease, and skin measurement. Read More

Adv Rheumatol 2019 Mar 22;59(1):13. Epub 2019 Mar 22.

Pediatric Rheumatology Unit, Children's Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Av. Dr. Eneas Carvalho Aguiar, São Paulo, SP, 05403-000, Brazil.

Objective: To evaluate sexual function female adolescents and young adults with juvenile idiopathic arthritis (JIA) and healthy controls.

Methods: After exclusion, 21 female adolescent and young JIA patients and 25 healthy controls were selected for this study. Sexual function was assessed by the Sexual Quotient Questionnaire for Females (SQQ-F) score, which is a validated tool and adapted for Brazilian Portuguese language. Read More

Indian J Ophthalmol 2019 Apr;67(4):490-495

Department of Ophthalmology, Advanced Eye Centre, Post Graduate Institute of Medical Education and Research, Sector 12, Chandigarh, India.

Purpose: To evaluate the long-term outcomes of cataract surgery in children with uveitis.

Methods: Retrospective, noncomparative review of medical records of children (≤16 years) with uveitic cataract who had undergone cataract surgery between January 2001 and December 2014 at a tertiary care center was done. The main outcome measures were visual acuity and postoperative complications. Read More

Arch Med Sci 2019 Mar 5;15(2):531-536. Epub 2018 Jul 5.

Arthritis Research Center, Rahmat Noor Clinic, Rawalpindi, Pakistan.

Introduction: Multifunctional pro-inflammatory cytokine tumor necrosis factor-α (TNF-α) has been implicated in a variety of inflammatory diseases including rheumatoid arthritis (RA). TNF-α polymorphisms are mostly located in its promoter region and play a significant role in disease susceptibility and severity. We therefore sought to investigate TNFA -863C/A (rs1800630) polymorphism association with RA activity in our Pakistani study group. Read More

Pediatr Rheumatol Online J 2019 Mar 20;17(1):11. Epub 2019 Mar 20.

Department of Pediatric Anesthesiology, UPMC Children's Hospital of Pittsburgh, 4401 Penn Avenue Pittsburgh, Pittsburgh, PA, 15224, USA.

Background: Intra-articular corticosteroid injections (IACI) are frequently used in the treatment of juvenile idiopathic arthritis. There is a paucity of evidence-based research describing methods of pain and anxiety control for this procedure. IACI were mostly performed under general anesthesia for children younger than 13 years old in our institution as of 2014. Read More

BMC Rheumatol 2019 28;3. Epub 2019 Feb 28.

1Clinic of Rheumatology, University of Magdeburg, Sophie-von-Boetticher-Strasse 1, 39245 Vogelsang-Gommern, Germany.

Background: Signs and symptoms establish the diagnosis of adult onset Still's disease (AOSD) as well as of systemic onset juvenile idiopathic arthritis (sJIA). The published data regarding the importance of IL-18 as a marker for diagnosis and disease activity so far are conflicting. The aim of this study was to clarify the role of IL-18 as a diagnostic and disease activity marker in AOSD and sJIA. Read More

BMC Rheumatol 2018 27;2. Epub 2018 Feb 27.

5Department of Women's and Children's, Institute of Translational Medicine, University of Liverpool, Liverpool, UK.

Background: Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. Children with JIA are at risk of intraocular inflammation (uveitis). In the initial stages of mild-moderate inflammation uveitis is asymptomatic. Read More

Pediatr Neonatol 2019 Feb 22. Epub 2019 Feb 22.

Biostatistics and Clinical Trials Center, Novosibirsk, Russia.

Background: Both the steroid- and NSAID-sparing effects of biologics in juvenile idiopathic arthritis (JIA) treatment are key aspects of the dynamics of patient's condition. The proper selection of biologics enables maximum treatment effectiveness and reduction of the dosage of concomitant therapy. Our aim was to study the dynamics of concomitant therapy during etanercept (ETA) and methotrexate (MTX) treatment in patients with JIA. Read More

J Oral Maxillofac Surg 2019 Feb 21. Epub 2019 Feb 21.

Associate Professor, Division of Oral and Maxillofacial Surgery, Department of Surgery, Emory University School of Medicine; and Associate Chief, Oral and Maxillofacial Surgery, Children's Healthcare of Atlanta, Atlanta, GA.

Purpose: Juvenile idiopathic arthritis (JIA) frequently affects the temporomandibular joints and may cause dentofacial deformity and dysfunction. The adverse effects of JIA on dentofacial growth, morphology, and function may be due to erosion of the existing mandibular condyle(s), the inhibitory effect of the arthritis on the growing mandible, or both. No algorithm exists for management of JIA-induced skeletal and dental abnormalities; treatment varies widely. Read More

J Autoimmun 2019 Mar 15. Epub 2019 Mar 15.

Department of Ophthalmology and Ophtha-Lab at St. Franziskus-Hospital, Münster, Germany; Department of Ophthalmology, University of Duisburg-Essen, Essen, Germany.

Gene and protein expression profiles of iris biopsies, aqueous humor (AqH), and sera in patients with juvenile idiopathic arthritis-associated uveitis (JIAU) in comparison to control patients with primary open-angle glaucoma (POAG) and HLA-B27-positive acute anterior uveitis (AAU) were investigated. Via RNA Sequencing (RNA-Seq) and mass spectrometry-based protein expression analyses 136 genes and 56 proteins could be identified as being significantly differentially expressed (DE) between the JIAU and POAG group. Gene expression of different immunoglobulin (Ig) components as well as of the B cell-associated factors ID3, ID1, and EBF1 was significantly upregulated in the JIAU group as compared to POAG patients. Read More

Int J Rheum Dis 2019 Mar 18. Epub 2019 Mar 18.

Department of Clinical Immunology, Jawaharlal Institute of Postgraduate Medical Education & Research, Puducherry, India.

Aim: The aim of the study was to assess the distribution of human leukocyte antigen (HLA)-B*27 subtypes and its correlation with disease phenotypes in children with enthesitis-related arthritis variant of juvenile idiopathic arthritis (JIA-ERA).

Method: One hundred and sixty patients (132 males, 28 females) satisfying the International League Against Rheumatism (ILAR) classification criteria for JIA-ERA were assessed and relevant demographic, clinical and radiographic data were documented. HLA-B*27 typing was done for all the patients and B*27 positive samples were subjected to high-resolution gene sequencing. Read More

J Autoimmun 2019 Mar 15. Epub 2019 Mar 15.

Department of Orthopaedic Surgery, Musculoskeletal Research Center, Washington University School of Medicine, St. Louis, MO, USA; Shriners Hospitals for Children, St. Louis, MO, USA. Electronic address:

Cytokine storm syndrome (CSS) is a life-threatening condition characterized by excessive activation of T cells and uncontrolled inflammation, mostly described in patients with familial hemophagocytic lymphohistiocytosis and certain systemic auto-inflammatory diseases, such as systemic juvenile idiopathic arthritis (sJIA). Defects in T cell cytotoxicity as a mechanism for uncontrolled inflammation following viral infections fail to represent the whole spectrum of CSS. Evidence implicates dysregulated innate immune responses, especially activation of monocytes and macrophages, in patients with CSS. Read More

J Rheumatol 2019 Mar 15. Epub 2019 Mar 15.

From the Unger-Vetlesens Institute, Lovisenberg Diaconal Hospital, Oslo, Norway; Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway; Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway; Section of Psychosomatics and CL-Child Psychiatry, Department of ClinicalNeurosciences for Children, Division of Paediatric and Adolescent Medicine, Oslo; University Hospital, Rikshospitalet, Oslo, Norway; Department of Patient Safety and Research, Lovisenberg Diaconal Hospital, Oslo, Norway; Department of Nursing Science, Institute of Health and Society, Faculty of Medicine, University of Oslo, Oslo, Norway. The project was supported by the Norwegian Foundation for Health and Rehabilitation. Address correspondence to Anita Tollisen, Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Postboks 4950, Nydalen, 0424 Oslo, Norway. Email:

Objective: To describe the longitudinal health status from childhood to adulthood in patients with juvenile idiopathic arthritis (JIA), compare outcomes after 19 years with those of controls and identify early predictors of physical functioning, pain and physical health-related quality of life (HRQOL).

Methods: Between 1995-2003, 96 JIA patients (mean 6.1±4. Read More

Arthritis Care Res (Hoboken) 2019 Mar 15. Epub 2019 Mar 15.

NIHR Manchester Biomedical Research Centre, Central Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK.

Objective: In paediatric research, investigators rely on proxy reports of outcome, such as the proxy-completed Childhood Health Assessment Questionnaire (P-CHAQ), to assess function in JIA. As children mature, they may self-complete the adult Health Assessment Questionnaire (HAQ) or the un-validated adolescent-specific CHAQ (A-CHAQ). It is unclear how these measures compare and whether they are directly interchangeable. Read More

Jpn J Radiol 2019 Mar 14. Epub 2019 Mar 14.

Department of Rheumatology, The Hospital for Sick Children, Department of Medical Imaging, University of Toronto, Toronto, ON, Canada.

Mixed connective tissue disease (MCTD) is a rare disease in children and adolescents which overlaps features of juvenile idiopathic arthritis, polymyositis/dermatomyositis, systemic lupus erythematosus, and systemic sclerosis. We have provided an image-based approach for evaluation of MCTD in children and adolescents, outlying the most frequent imaging findings. This approach would aid imagers and clinicians to consider the diagnosis of this rare entity and be able to make an accurate list of differential diagnosis for complex rheumatologic diseases such as MCTD, thus facilitating the ultimate goal of early diagnosis and optimal management of affected children. Read More

Expert Rev Clin Immunol 2019 Mar 15:1-10. Epub 2019 Mar 15.

a Department of Ophthalmology, University Medical Center Utrecht , Utrecht University , Utrecht , The Netherlands.

Introduction: Uveitis is a sight-threatening eye inflammation and common manifestation of juvenile idiopathic arthritis (JIA). New biomarkers that can predict uveitis are needed to alleviate personalized clinical screening. In this review, we outline clinical and molecular risk factors for uveitis and discuss their putative biology and value for clinical practice. Read More

Clin Exp Rheumatol 2019 Mar 7. Epub 2019 Mar 7.

Department of Rheumatology and Joint and Bone Research Unit, Hospital Universitario Fundación Jiménez Díaz, Madrid, Spain.

Objectives: Subclinical synovitis is often detected by musculoskeletal ultrasound (MSUS) in juvenile idiopathic arthritis (JIA) patients in clinical remission. The main objective of this prospective, observational, longitudinal, multicentre study was to evaluate the predictive value of MSUS-detected subclinical synovitis in relation to flares at 12 months following TNFi tapering in a JIA population in stable clinical remission.

Methods: We included 56 JIA patients in stable remission undergoing TNFi therapy tapered at baseline and in some cases at 6 months. Read More

Sci Rep 2019 Mar 14;9(1):4579. Epub 2019 Mar 14.

Department of Immunology, Hospital Clínic-IDIBAPS, Barcelona, Spain.

Juvenile idiopathic arthritis (JIA) is a complex rheumatic disease with both autoimmune and autoinflammatory components. Recently, familial cases of systemic-onset JIA have been attributed to mutations in LACC1/FAMIN. We describe three affected siblings from a Moroccan consanguineous family with an early-onset chronic, symmetric and erosive arthritis previously diagnosed as rheumatoid factor (RF)-negative polyarticular JIA. Read More

J Clin Med 2019 Mar 12;8(3). Epub 2019 Mar 12.

Department of Pediatrics, Yonsei University College of Medicine, Yonsei-ro 50, Seodaemun-gu, C.P.O. Box 8044, Seoul 03722, Korea.

The 1858T allele in the protein tyrosine phosphatase non-receptor type 22 (PTPN22) locus shows one of the strongest and most consistent genetic associations with autoimmune diseases. We synthesized all meta-analyses reporting a genetic association of the PTPN22 1858T C/T polymorphism with autoimmune diseases. This work examined their validity to discover false positive results under Bayesian methods. Read More

J Fam Pract 2019 Mar;68(2):E8-E13

Department of Pediatric Rheumatology, University of Mississippi Medical Center, Jackson, MS, USA.

Beyond NSAIDs and disease-modifying antirheumatic drugs are now biologic agents and anti-interleukin drugs that can augment therapy. Read More

J Immunol Res 2019 4;2019:3140204. Epub 2019 Feb 4.

Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical, and Health Sciences, Kanazawa University, Kanazawa, Japan.

To investigate whether serum leucine-rich 2-glycoprotein (LRG) levels are useful as a marker of disease activity in systemic juvenile idiopathic arthritis (s-JIA), we determined serum LRG levels in fifty-nine s-JIA patients, 15 with other subtypes of JIA, 7 with Kawasaki disease (KD), 7 with influenza A infection (flu), 7 with enterohemorrhagic (EHEC) infection, and 20 healthy controls (HC). Results were compared with the clinical features of s-JIA and serum cytokine levels including interleukin- (IL-) 6, IL-18, and soluble tumor necrosis factor receptors I and II. Serum LRG levels in active s-JIA were higher compared to those in other subtypes of JIA, EHEC, flu patients, and HC. Read More

Ann Rheum Dis 2019 May 12;78(5):617-628. Epub 2019 Mar 12.

VIB Center for Brain and Disease Research, Leuven, Belgium

Objectives: Juvenile idiopathic arthritis (JIA) is the most common class of childhood rheumatic diseases, with distinct disease subsets that may have diverging pathophysiological origins. Both adaptive and innate immune processes have been proposed as primary drivers, which may account for the observed clinical heterogeneity, but few high-depth studies have been performed.

Methods: Here we profiled the adaptive immune system of 85 patients with JIA and 43 age-matched controls with indepth flow cytometry and machine learning approaches. Read More