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Systemic juvenile idiopathic arthritis

Peter A Nigrovic, Timothy Beukelman, George Tomlinson, Brian M Feldman, Laura E Schanberg, Yukiko Kimura
BACKGROUND: Systemic juvenile idiopathic arthritis is a rare febrile arthritis of childhood characterized by a potentially severe course, including prolonged glucocorticoid exposure, growth failure, destructive arthritis, and life-threatening macrophage activation syndrome. Early cytokine-blocking biologic therapy may improve long-term outcomes, although some systemic juvenile idiopathic arthritis patients respond well to non-biologic treatment, leaving optimal management undefined. Consequently, treatment of new-onset systemic juvenile idiopathic arthritis by expert clinicians varies widely...
March 1, 2018: Clinical Trials: Journal of the Society for Clinical Trials
Ann Marie Szymanski, Michael J Ombrello
The intersection of granulomatosis and autoinflammatory disease is a rare occurrence that can be generally subdivided into purely granulomatous phenotypes and disease spectra that are inclusive of granulomatous features. NOD2 (nucleotide binding oligomerization domain containing 2)-related disease, which includes Blau syndrome and early onset sarcoidosis, is the prototypic example of granulomatous inflammation in the context of monogenic autoinflammation. Granulomatous inflammation has also been observed in two related autoinflammatory diseases caused by mutations in PLCG2 (phospholipase C gamma 2)...
March 12, 2018: International Immunology
Eugen Feist, Pierre Quartier, Bruno Fautrel, Rayfel Schneider, Paolo Sfriso, Petros Efthimiou, Luca Cantarini, Karine Lheritier, Karolynn Leon, Chetan S Karyekar, Antonio Speziale
OBJECTIVES: To describe the efficacy, safety, and exposure-response relationship of canakinumab in a subgroup of patients with systemic juvenile idiopathic arthritis (SJIA) aged ≥16 years, representative of adult-onset Still's disease (AOSD) patients, and to compare this subgroup with those of children and young adolescents with SJIA by pooling clinical data collected during the development programme of canakinumab. METHODS: Safety and efficacy data on canakinumab-treated patients were pooled from 4 SJIA studies (NCT00426218, NCT00886769, NCT00889863, and NCT00891046)...
March 2, 2018: Clinical and Experimental Rheumatology
L L Tao, X P Zhu, X M Tang
No abstract text is available yet for this article.
March 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Andrea Rubbert-Roth, Daniel E Furst, Jan Michael Nebesky, Angela Jin, Erhan Berber
Tocilizumab (TCZ) is the first humanized anti-interleukin-6 (IL-6) receptor monoclonal antibody approved for the treatment of patients with rheumatoid arthritis (RA), Castleman's disease, polyarticular and systemic juvenile idiopathic arthritis, and, most recently, giant cell arteritis as well as for the treatment of chimeric antigen receptor T cell therapy-induced cytokine release syndrome. The global clinical development program for TCZ provides a wealth of clinical data on intravenous TCZ, and more recent studies in patients with RA have provided evidence characterizing the role of intravenous TCZ as monotherapy in early disease and led to the introduction of a subcutaneous formulation of TCZ...
March 3, 2018: Rheumatology and Therapy
Amélie Leurs, David Launay, Louis Terriou, Pierre-Yves Hatron, Pierre Quartier, Eric Hachulla
No abstract text is available yet for this article.
February 27, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
Katerina Theodoropoulou, Sophie Georgin-Lavialle
Still's disease is a rare multifactorial disease associated with systemic inflammation. Systemic-onset juvenile idiopathic arthritis and adult-onset Still's disease are both pediatric and respectively adult forms of the disease with a cut-off age of 16 years. The disease is characterized by the following features : hectic fever > 39° C, arthralgia or arthritis, rash, neutrophilia and systemic inflammation. The prognosis of the disease is functional and vital. The evolution over time is variable : regression, evolution by relapses with regression at term and chronic joint evolution...
February 14, 2018: Revue Médicale Suisse
Catherine G Anelli, Claudio A Len, Maria T Terreri, Gleice C S Russo, Andreas O Reiff
OBJECTIVE: To translate and validate the Brazilian Portuguese version of the Transition Readiness Assessment Questionnaire in a population of adolescents and young adults with chronic rheumatologic disorders. This questionnaire evaluates the patient's readiness for making the transition from the pediatric health service to adult care. METHODS: The four-phase methodology for the translation and validation of generic questionnaires was followed, including translation, back-translation, pilot testing and clinical validation of the final tool...
February 16, 2018: Jornal de Pediatria
Brandi E Stevens, Kathryn S Torok, Suzanne C Li, Nicole Hershey, Megan Curran, Gloria C Higgins, Katharine F Moore, C Egla Rabinovich, Samuel Dodson, Anne M Stevens
OBJECTIVE: To investigate clinical manifestations of juvenile systemic sclerosis (jSSc), including disease characteristics and patient quality of life, through the multinational Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry. METHODS: Subjects with jSSc were prospectively enrolled between 2010 and 2013. Diagnosis of jSSc was determined by the enrolling pediatric rheumatologist, with disease onset required prior to age 18. Collected data included demographics, disease characteristics, medication exposure, and quality of life metrics...
February 18, 2018: Arthritis Care & Research
Iris Deitch, Radgonde Amer, Oren Tomkins-Netzer, Zohar Habot-Wilner, Ronit Friling, Ron Neumann, Michal Kramer
PURPOSE: This study aimed to report the clinical outcome of children with uveitis treated with anti-tumor necrosis factor alpha (TNF-α) agents. METHODS: This included a retrospective cohort study. Children with uveitis treated with infliximab or adalimumab in 2008-2014 at five dedicated uveitis clinics were identified by database search. Their medical records were reviewed for demographic data, clinical presentation, ocular complications, and visual outcome. Systemic side effects and the steroid-sparing effect of treatment were documented...
February 18, 2018: Graefe's Archive for Clinical and Experimental Ophthalmology
Nienke M Ter Haar, Tamar Tak, Michal Mokry, Rianne C Scholman, Jenny M Meerding, Wilco de Jager, Anouk Verwoerd, Dirk Foell, Thomas Vogl, Johannes Roth, Pieter H C Leliefeld, Jorg van Loosdregt, Leo Koenderman, Sebastiaan J Vastert, Sytze de Roock
OBJECTIVE: Neutrophils are the most abundant innate immune cells in the blood, however little is known about their role in (acquired) chronic autoinflammatory diseases. We investigated neutrophils in systemic onset Juvenile Idiopathic Arthritis (sJIA), a prototypical multifactorial autoinflammatory disease, characterized by arthritis and severe systemic inflammation. METHODS: We analyzed 50 sJIA patients at disease onset and during remission on recombinant IL-1 receptor antagonist (rIL-1RA, anakinra)...
February 9, 2018: Arthritis & Rheumatology
Samira Esmaeili Reykande, Arezou Rezaei, Maryam Sadr, Mahsima Shabani, Farnaz Najmi Varzaneh, Vahid Ziaee, Nima Rezaei
Interferon regulatory factor 5 (IRF5) is a member of IRF family which induce signaling pathways and are involved in modulation of cell growth, differentiation, apoptosis, and immune system activity. Juvenile idiopathic arthritis (JIA) is an auto-inflammatory syndrome where the inflammatory markers are believed to play a fundamental role in its pathogenesis. In this study, we aimed to assess the association of IRF5 gene polymorphisms with susceptibility of JIA in Iranian population. Three IRF5 single-nucleotide polymorphisms (rs10954213 A/G, rs2004640 G/T, and rs3807306 G/T) were genotyped using TaqMan assays in 55 patients with JIA and 63 matched healthy individuals...
February 8, 2018: Clinical Rheumatology
Grant S Schulert, Scott W Canna
Hyperferritinemia and pronounced hemophagocytosis help distinguish a subset of patients with a particularly inflammatory and deadly systemic inflammatory response syndrome. Two clinically similar disorders typify these hyperferritinemic syndromes: hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS). HLH is canonically associated with a complete disturbance of perforin/granzyme-mediated cytotoxicity, whereas MAS occurs in the context of the related rheumatic diseases systemic juvenile idiopathic arthritis (SJIA) and adult-onset Still's disease (AOSD), with associated IL-1 family cytokine activation...
February 6, 2018: International Immunology
Grant S Schulert, Mingce Zhang, Ammar Husami, Ndate Fall, Hermine Brunner, Kejian Zhang, Randy Q Cron, Alexei A Grom
OBJECTIVE: Macrophage activation syndrome (MAS) is a life-threatening complication of systemic juvenile idiopathic arthritis (SJIA), and has pathologic similarity to hemophagocytic lymphohistiocytosis (HLH). Intronic variants in UNC13D are found in patients with familial HLH-3 (FHL3), but the role of non-coding variants in MAS is unknown. The objective of this study was to identify deep intronic UNC13D variants in patients with MAS. METHODS: A custom enrichment library was constructed to sequence approximately 1 MB flanking UNC13D in 24 patients with SJIA, recurrent MAS, and negative prior genetic (exon/coding) testing...
February 6, 2018: Arthritis & Rheumatology
Piotr Buda, Piotr Gietka, Janusz B Książyk, Maciej Machaczka
Introduction: Secondary hemophagocytic lymphohistiocytosis (sHLH) is a life-threatening syndrome of severe hyperinflammation which is often triggered by infection or autoimmune disease (macrophage activation syndrome - MAS). The aim of our study was to assess the frequency of sHLH/MAS in children treated in our institution and to compare the effectiveness of various therapeutic interventions. Material and methods: Between 2005 and 2013, 24 children (age: 1-17 years) were consecutively treated for sHLH/MAS...
January 2018: Archives of Medical Science: AMS
Surupa Basu, Biplab Maji, Santanu Barman, Apurba Ghosh
Hemophagocytic lymphohistiocytosis (HLH) is an inflammatory condition that may run a rapid fatal course and calls for prompt diagnosis. Early intervention with steroids and other immunosuppressive drugs can contain the disease process and favours positive outcome. Ferritin ≥500 ng/ml is a HLH diagnostic criterion. We evaluated the diagnostic potential of admission ferritin, in children with HLH. Pediatric patients of a referral teaching hospital from Feb 2010-Oct 2013 having been investigated for ferritin on admission were included...
January 2018: Indian Journal of Clinical Biochemistry: IJCB
Ji Won Yang, Eunyoung Lee, Ji-Yeon Seo, Ju-Yang Jung, Chang-Hee Suh, Hyoun-Ah Kim
BACKGROUND: 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 International League Against Rheumatism (ILAR) criteria for systemic JIA and the Yamaguchi criteria and then compared their possible prognostic value in patients with AOSD. METHODS: In a retrospective review of 169 adults with suspected AOSD, patients were classified according to the Yamaguchi or ILAR criteria...
January 25, 2018: Pediatric Rheumatology Online Journal
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, Kirsten Mönkemöller, Ulrich Neudorf, Elisabeth Weißbarth-Riedel, Helmut Wittkowski, Gerd Horneff, Dirk Foell
BACKGROUND: 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. The PRO-KIND initiative of the German Society for Pediatric Rheumatology (GKJR) aims to define consensus-based strategies to harmonize diagnostic and therapeutic approaches in Germany. METHODS: We analyzed data on patients diagnosed with SJIA from 3 national registries in Germany...
January 22, 2018: Pediatric Rheumatology Online Journal
Toshihiro Matsui, Takumi Matsumoto, Fumio Hirano, Fumika Tokunaga, Keisuke Okamoto, Shigeto Tohma, Tomohiro Morio, Hitoshi Kohsaka, Masaaki Mori
OBJECTIVES: To understand the current status of adult rheumatology care for patients who had previously had juvenile idiopathic arthritis (JIA) (excluding systemic JIA), and to identify issues interfering with the transition from pediatric to adult care in Japan. METHODS: Questionnaire-based survey among 30 adult rheumatologists. RESULTS: Eighty-seven percent of adult rheumatologists responded that they had provided medical care to adults who had had JIA; 44% of them had felt hesitation or anxiety when providing such care...
January 18, 2018: Modern Rheumatology
Thuy Do, Rachel Tan, Mark Bennett, Mario Medvedovic, Alexei A Grom, Nan Shen, Sherry Thornton, Grant S Schulert
Systemic juvenile idiopathic arthritis (SJIA) is a severe childhood arthropathy with features of autoinflammation. Monocytes and macrophages in SJIA have a complex phenotype with both pro- and anti-inflammatory properties that combine features of several well characterized in vitro conditions used to activate macrophages. An important anti-inflammatory phenotype is expression of CD163, a scavenger receptor that sequesters toxic pro-inflammatory complexes that is highly expressed in both active SJIA and macrophage activation syndrome (MAS)...
January 2018: Journal of Leukocyte Biology
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