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Arthritis & Rheumatology (Hoboken, N.J.) Nov 2019Systemic juvenile idiopathic arthritis (JIA) is associated with a recently recognized, albeit poorly defined and characterized, lung disease (LD). The objective of this...
OBJECTIVE
Systemic juvenile idiopathic arthritis (JIA) is associated with a recently recognized, albeit poorly defined and characterized, lung disease (LD). The objective of this study was to describe the clinical characteristics, risk factors, and histopathologic and immunologic features of this novel inflammatory LD associated with systemic JIA (designated SJIA-LD).
METHODS
Clinical data collected since 2010 were abstracted from the medical records of patients with systemic JIA from the Cincinnati Children's Hospital Medical Center. Epidemiologic, cellular, biochemical, genomic, and transcriptional profiling analyses were performed.
RESULTS
Eighteen patients with SJIA-LD were identified. Radiographic findings included diffuse ground-glass opacities, subpleural reticulation, interlobular septal thickening, and lymphadenopathy. Pathologic findings included patchy, but extensive, lymphoplasmacytic infiltrates and mixed features of pulmonary alveolar proteinosis (PAP) and endogenous lipoid pneumonia. Compared to systemic JIA patients without LD, those with SJIA-LD were younger at the diagnosis of systemic JIA (odds ratio [OR] 6.5, P = 0.007), more often had prior episodes of macrophage activation syndrome (MAS) (OR 14.5, P < 0.001), had a greater frequency of adverse reactions to biologic therapy (OR 13.6, P < 0.001), and had higher serum levels of interleukin-18 (IL-18) (median 27,612 pg/ml versus 5,413 pg/ml; P = 0.047). Patients with SJIA-LD lacked genetic, serologic, or functional evidence of granulocyte-macrophage colony-stimulating factor pathway dysfunction, a feature that is typical of familial or autoimmune PAP. Moreover, bronchoalveolar lavage (BAL) fluid from patients with SJIA-LD rarely demonstrated proteinaceous material and had less lipid-laden macrophages than that seen in patients with primary PAP (mean 10.5% in patients with SJIA-LD versus 66.1% in patients with primary PAP; P < 0.001). BAL fluid from patients with SJIA-LD contained elevated levels of IL-18 and the interferon-γ-induced chemokines CXCL9 and CXCL10. Transcriptional profiling of the lung tissue from patients with SJIA-LD identified up-regulated type II interferon and T cell activation networks. This signature was also present in SJIA-LD human lung tissue sections that lacked substantial histopathologic findings, suggesting that this activation signature may precede and drive the lung pathology in SJIA-LD.
CONCLUSION
Pulmonary disease is increasingly detected in children with systemic JIA, particularly in association with MAS. This entity has distinct clinical and immunologic features and represents an uncharacterized inflammatory LD.
Topics: Age Distribution; Arthritis, Juvenile; Bronchoalveolar Lavage Fluid; Chemokine CXCL10; Chemokine CXCL9; Child; Child, Preschool; Female; Granulocyte-Macrophage Colony-Stimulating Factor; Humans; Infant; Interferon-gamma; Interleukin-18; Lung; Lung Diseases; Macrophage Activation Syndrome; Male; Pulmonary Alveolar Proteinosis; T-Lymphocytes; Tomography, X-Ray Computed; Transcriptome; Up-Regulation
PubMed: 31379071
DOI: 10.1002/art.41073 -
La Tunisie Medicale Jun 2023Juvenile idiopathic arthritis (JIA), as a chronic condition, is associated with significant disease- and treatment-related morbidity, thus impacting children's quality... (Review)
Review
Juvenile idiopathic arthritis (JIA), as a chronic condition, is associated with significant disease- and treatment-related morbidity, thus impacting children's quality of life. In order to optimize JIA management and to ensure the best possible care and outcome for children with rheumatic diseases, dedicated disease activity and damage assessment tools are essential. In recent years, there has been a concerted and important international effort to develop and validate disease activity and outcome instruments specific to JIA. This update aims to describe the main outcome measures currently used in JIA patients. These outcome measures include composite disease activity score, measures of physical function, measures of health related quality of life, clinical measures of damage and the assessment of Parent and child reported outcomes (PCROs).
Topics: Child; Humans; Arthritis, Juvenile; Quality of Life; Outcome Assessment, Health Care; Severity of Illness Index
PubMed: 38372552
DOI: No ID Found -
International Journal of Environmental... Jun 2022Objective: This study intended to examine the effects of Pilates exercise on pain, cardiorespiratory fitness, functional ability, and quality of life in children with... (Randomized Controlled Trial)
Randomized Controlled Trial
Impact of Clinical Pilates Exercise on Pain, Cardiorespiratory Fitness, Functional Ability, and Quality of Life in Children with Polyarticular Juvenile Idiopathic Arthritis.
Objective: This study intended to examine the effects of Pilates exercise on pain, cardiorespiratory fitness, functional ability, and quality of life in children with polyarticular juvenile idiopathic arthritis. Methods: Forty children with polyarticular JIA aged 10−14 years old were randomly allocated into two groups: the control group (n = 20) received conventional physical therapy (CPT), and the experimental group (n = 20) received clinical Pilates exercises combined with CPT. Patients in both groups received their program three times/week for 3 months. Pain, cardiorespiratory fitness, functional ability, and quality of life were assessed through the visual analogue scale, cardiopulmonary exercise test, 6 min walk test, and PedsQL scale, respectively, just before and after treatment. Results: Pain (p = 0.001), cardiorespiratory markers (all p < 0.05), functional ability (p = 0.002), and overall quality of life (p = 0.007) improved significantly in the experimental groups compared to the control group. Conclusion: Incorporating Pilates exercises into CPT is likely more effective for decreasing pain intensity, improving cardiorespiratory fitness, augmenting functional ability, and promoting quality of life in children with JIA than CPT alone.
Topics: Adolescent; Arthritis, Juvenile; Cardiorespiratory Fitness; Child; Exercise Movement Techniques; Exercise Therapy; Humans; Pain; Quality of Life
PubMed: 35805451
DOI: 10.3390/ijerph19137793 -
Jornal de Pediatria 2022In this article, the authors aimed to review the different tools used in the monitoring of enthesitis-related arthritis. (Review)
Review
OBJECTIVES
In this article, the authors aimed to review the different tools used in the monitoring of enthesitis-related arthritis.
SOURCES
The authors performed a literature review on PubMed, Google Scholar, and Scopus databases. The dataset included the original research and the reviews including patients with enthesitis-related arthritis or juvenile spondylarthritis up to October 2020.
SUMMARY OF FINDING
Enthesitis-related arthritis is a category of juvenile idiopathic arthritis. It is characterized by the presence of enthesitis, peripheral arthritis, as well as axial involvement. The only validated tool for disease activity measurement in juvenile idiopathic arthritis is the Disease Activity Score: It has proven its reliability and sensitivity. Nevertheless, due to an absence of validated evaluation tools, the extent of functional impairment, as well as the children and parents' perception of the disease, could not be objectively perceived. Despite the great progress in the field of imaging modalities, the role they play in the evaluation of disease activity is still controversial. This is partially due to the lack of validated scoring systems.
CONCLUSIONS
Further work is still required to standardize the monitoring strategy and validate the outcome measures in enthesitis-related arthritis.
Topics: Arthritis, Juvenile; Child; Humans; Reproducibility of Results; Spondylarthritis
PubMed: 34597529
DOI: 10.1016/j.jped.2021.08.002 -
Japanese Journal of Radiology Nov 2023Juvenile idiopathic arthritis (JIA) is a collective term for pediatric inflammatory arthritis of unknown etiology, which presents diverse clinical and imaging findings.... (Review)
Review
Juvenile idiopathic arthritis (JIA) is a collective term for pediatric inflammatory arthritis of unknown etiology, which presents diverse clinical and imaging findings. The pathogenesis is complex; however, most cases stem from an autoimmune mechanism. Herein we provide a short review of imaging findings of JIA. Imaging assessment begins with plain radiography demonstrating joint swelling, periarticular osteopenia, and juxtaarticular bone erosion. Bone erosion occurs later in JIA. Instead, aberrant epimetaphyseal growth often gives the first clue to the diagnosis. US and MRI can demonstrate the details of the synovium, cartilage, and subchondral bone. JIA is subdivided into oligoarthritis, polyarthritis (rheumatoid factor-negative and positive), psoriatic arthritis, enthesitis-related arthritis, and systemic JIA. Awareness of the different clinical characteristics, pathogenic background, and prognosis of each subtype facilitates a more advanced, imaging-based diagnosis. Unlike the other types, systemic JIA is an autoinflammatory disease accompanied by inflammatory cytokinemia and systemic symptoms stemming from aberrant activation of the innate immunity. Other autoinflammatory diseases, both monogenic (e.g., NOMID/CINCA) and multifactorial (e.g., CRMO), are also discussed.
Topics: Child; Humans; Arthritis, Juvenile; Radiography; Magnetic Resonance Imaging; Hereditary Autoinflammatory Diseases
PubMed: 37329408
DOI: 10.1007/s11604-023-01447-6 -
Journal of Orthopaedic Surgery (Hong... 2020There is limited literature to guide shoulder surgeons in the management of juvenile idiopathic arthritis (JIA). We aim to help clinicians to formulate an approach to... (Review)
Review
There is limited literature to guide shoulder surgeons in the management of juvenile idiopathic arthritis (JIA). We aim to help clinicians to formulate an approach to the surgical management of the condition through a review of the available literature on arthroplasty in JIA, general considerations when operating on patients with inflammatory arthropathy and recommendations based on the authors' experience. Four articles report formal data on arthroplasty in JIA with favourable improvements in post-operative pain and function scores after the long-term follow-up. Significant heterogeneity in treatment and a lack of standardisation in quantitative outcomes highlights the need for further larger scale and higher quality research. The aim of this study is to review the evidence and provide information on preoperative evaluation of surgical candidates, operative techniques, choice of implant design and to evaluate functional outcomes in patients who undergo shoulder arthroplasty.
Topics: Arthritis, Juvenile; Arthroplasty, Replacement, Shoulder; Humans; Range of Motion, Articular; Shoulder Joint; Treatment Outcome
PubMed: 31916484
DOI: 10.1177/2309499019890615 -
Pediatric Rheumatology Online Journal May 2023To determine the clinical and laboratory differences between leukemic arthritis (LA) and juvenile idiopathic arthritis (JIA) at the onset of the disease.
OBJECTIVES
To determine the clinical and laboratory differences between leukemic arthritis (LA) and juvenile idiopathic arthritis (JIA) at the onset of the disease.
MATERIAL AND METHODS
Patients under 16 years of age, both genders, who presented for the first time to the pediatric rheumatology service with a diagnosis of probable JIA, with arthritis and without peripheral blood blasts, in which the final diagnosis was acute lymphoblastic leukemia (ALL) or JIA. The clinical and laboratory characteristics of the patients were compared, chi-square and relative risk were used for categorical variables, and the Mann-Whitney U and T-test for the comparison of means between groups. A binary logistic regression model was developed to differentiate leukemic arthritis from JIA.
RESULTS
A total of 76 patients, 14 with LA and 62 with JIA, were analyzed. The mean age at diagnosis was lower in the leukemic arthritis group, the female gender prevailed in the JIA group, and the time to onset of symptoms was lower in the leukemic arthritis group. Patients with leukemic arthritis showed increased pain intensity, fever, weight loss, nocturnal diaphoresis, lymph node enlargement, hepatosplenomegaly, and pain that did not improve with analgesic administration. Laboratory parameters with statistical significance were the presence of anemia, leukopenia, and neutropenia. The platelet count was significant but in a low normal value, compared to the JIA. A binary logistic regression model was developed to differentiate leukemic arthritis from JIA. The probability associated with the statistic (Chi-square) was 0.000, and the Cox and Snell R2 and Nagelkerke R2 values were 0.615 and 1, respectively. The developed model correctly classified 100% of the cases.
CONCLUSIONS
The diagnosis of acute lymphoblastic leukemia should be ruled out in patients who present with arthritis and hematological alterations, mainly leukopenia and neutropenia, with joint pain disproportionate to the degree of arthritis, predominantly at night and that does not improve with the use of analgesics, fever, lymph nodes, and hepatosplenomegaly. Criteria are suggested to differentiate both diseases.
Topics: Child; Humans; Female; Male; Arthritis, Juvenile; Pain; Arthralgia; Neutropenia; Thrombocytopenia; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Hepatomegaly
PubMed: 37259088
DOI: 10.1186/s12969-023-00836-5 -
Arthritis Research & Therapy Mar 2023Macrophage activation syndrome (MAS) is a life-threatening complication of pediatric rheumatic diseases, occurring most commonly in children with systemic juvenile... (Review)
Review
Macrophage activation syndrome (MAS) is a life-threatening complication of pediatric rheumatic diseases, occurring most commonly in children with systemic juvenile idiopathic arthritis (SJIA). Despite several classes of currently available treatment options for SJIA, including biologic agents targeting IL-1 or IL-6, there remain severe cases suffering from refractory disease and recurrent MAS. The phenotype of MAS is similar to hemophagocytic lymphohistiocytosis (HLH), but the underlying pathophysiology of MAS complicating SJIA or other disorders has not been fully clarified. These facts make it challenging to develop and utilize animal models to study MAS. To date, there is no "perfect" model replicating MAS, but several models do demonstrate aspects of SJIA and/or MAS. In this review, we examine the proposed animal models of SJIA and MAS, focusing on how they reflect these disorders, what we have learned from the models, and potential future research questions. As we better understand the key features of each, animal models can be powerful tools to further define the pathophysiology of SJIA and MAS, and develop new treatment targets and strategies.
Topics: Animals; Mice; Macrophage Activation Syndrome; Arthritis, Juvenile; Lymphohistiocytosis, Hemophagocytic; Biological Factors; Disease Models, Animal
PubMed: 36964620
DOI: 10.1186/s13075-023-03032-8 -
Deutsches Arzteblatt International Aug 2020
Topics: Arthralgia; Arthritis, Juvenile; Child; Humans; Pain
PubMed: 33161946
DOI: 10.3238/arztebl.2020.0599b -
BMJ Open Ophthalmology Jan 2023To facilitate the integration of eye care into universal health coverage, the WHO is developing a Package of Eye Care Interventions (PECI). Development of the PECI... (Review)
Review
To facilitate the integration of eye care into universal health coverage, the WHO is developing a Package of Eye Care Interventions (PECI). Development of the PECI involves the identification of evidence-based interventions from relevant clinical practice guidelines (CPGs) for uveitis.A systematic review of CPGs published on uveitis between 2010 and March 2020 was conducted. CPGs passing title and abstract and full-text screening were evaluated using the Appraisal of Guidelines for Research and Evaluation II (AGREE II) tool and data on recommended interventions extracted using a standard data extraction sheet.Of 56 CPGs identified as potentially relevant from the systematic literature search, 3 CPGs underwent data extraction following the screening stages and appraisal with the AGREE II tool. These CPGs covered screening for, monitoring and treating juvenile idiopathic arthritis (JIA)-associated uveitis, the use of adalimumab and dexamethasone in treating non-infectious uveitis, and a top-level summary of assessment, differential diagnosis and referral recommendations for uveitis, aimed at primary care practitioners. Many of the recommendations were based on expert opinion, though some incorporated clinical study and randomised controlled trial data.There is currently sparse coverage of the spectrum of disease caused by uveitis within CPGs. This may partially be due to the large number of conditions with diverse causes and clinical presentations covered by the umbrella term uveitis, which makes numerous sets of guidelines necessary. The limited pool of CPGs to select from has implications for clinicians seeking guidance on clinical care strategies for uveitis.
Topics: Humans; Uveitis; Adalimumab; Arthritis, Juvenile
PubMed: 37278434
DOI: 10.1136/bmjophth-2022-001091