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Scientific Reports Oct 2023Uveitis is one of the most common manifestations of juvenile idiopathic arthritis (JIA). Currently, JIA is associated with decreased gut microbiota diversity. Studies...
Uveitis is one of the most common manifestations of juvenile idiopathic arthritis (JIA). Currently, JIA is associated with decreased gut microbiota diversity. Studies confirm that perinatal events can cause aberrant microbial colonization. The objective of this study is to determine if JIA is associated with perinatal events with a secondary focus on these variables to the development of JIA-uveitis. 369 patients with strabismus (n = 200) or JIA (n = 196) were included in the study. Completed surveys (JIA 37; strabismus 18) collected data about birth route, pregnancy and labor complications, JIA medications, and the presence of eye disorders. Analysis indicates that there is no relationship between JIA development and the perinatal events investigated. Similarly, no significance was found between JIA-uveitis and birth route or labor complications. Pregnancy complications, namely gestational diabetes (GD), were statistically higher in the JIA group with uveitis compared to JIA without uveitis. The data from this survey study showed that JIA-uveitis was highly associated with pregnancy complications, particularly with GD. However, no statistically significant association was found between JIA and route of delivery, labor complications, or pregnancy complications. Further studies are needed to understand the ways that GD interrelates with the development of uveitis in JIA patients.
Topics: Humans; Female; Arthritis, Juvenile; Uveitis; Strabismus; Obstetric Labor Complications; Pregnancy Complications
PubMed: 37845273
DOI: 10.1038/s41598-023-44208-1 -
The Journal of Pediatrics Dec 2022To develop and validate a diagnostic prediction model that can distinguish between juvenile idiopathic arthritis (JIA) and chronic musculoskeletal pain syndrome (CMPS)...
OBJECTIVE
To develop and validate a diagnostic prediction model that can distinguish between juvenile idiopathic arthritis (JIA) and chronic musculoskeletal pain syndrome (CMPS) based on patient-reported outcomes.
STUDY DESIGN
This retrospective cohort study evaluated whether the Juvenile Arthritis Multidimensional Assessment Report (JAMAR) performs well in distinguishing JIA from CMPS. We analyzed JAMARs completed by 287 patients at their first visit to the pediatric rheumatology department of Wilhelmina Children's Hospital in Utrecht, The Netherlands. Relevant JAMAR items for predicting a diagnosis of JIA were selected in a penalized multivariable model suitable for clinical application. The model was subsequently validated with new data from the same center.
RESULTS
A total of 196 JAMARs (97 JIA, 99 CMPS) were collected in the model development data, and 91 JAMARs (48 JIA, 43 CMPS) were collected in the validation data. Variables in the prediction model that were strongest associated with a diagnosis of JIA instead of CMPS were asymmetric pain/swelling in the shoulder (OR, 2.34), difficulty with self-care (OR, 2.41), skin rash (OR, 2.07), and asymmetric/pain swelling in the knee (OR, 2.29). Calibration and discrimination (area under the receiver operating characteristic curve, 0.83; 95% CI, 0.74-0.92) of the model in the validation data were good.
CONCLUSIONS
Several items from the JAMAR questionnaire can potentially distinguish JIA from CMPS in patients with corresponding symptoms. We present an easy-to-use, adjusted, and validated model to separate these 2 diagnoses early at presentation based on patient-reported outcomes to facilitate proper referral and treatment.
Topics: Child; Humans; Arthritis, Juvenile; Disability Evaluation; Translating; Psychometrics; Musculoskeletal Pain; Retrospective Studies; Quality of Life; Reproducibility of Results; Cultural Characteristics; Patients; Parents; Age of Onset; Predictive Value of Tests; Prognosis; Case-Control Studies
PubMed: 35460700
DOI: 10.1016/j.jpeds.2022.04.029 -
Zhonghua Nei Ke Za Zhi Feb 2022The common clinical subtypes of juvenile idiopathic arthritis (JIA) include systemic onset juvenile idiopathic arthritis (SOJIA), oligoarthritis/polyarthritis juvenile...
The common clinical subtypes of juvenile idiopathic arthritis (JIA) include systemic onset juvenile idiopathic arthritis (SOJIA), oligoarthritis/polyarthritis juvenile idiopathic arthritis and juvenile spondyloarthritis. Juvenile idiopathic arthritis has no specific diagnostic index, and needs to be differentiated from infectious diseases and malignant diseases. The onset of SOJIA is rapid, the disease progresses rapidly, and it is easy to be complicated with macrophage activation syndrome (MAS) which is life-threatening. The experience of pediatric rheumatologists in dealing with JIA is still insufficient, and the standardized diagnosis and treatment level of this disease needs to be further improved. Based on the experience and guidelines of diagnosis and treatment in China and abroad, we formulated this diagnosis and treatment standard, aiming at standardizing the diagnosis and treatment of the subtypes of JIA and MAS, so as to reduce the incidence of disability and serious complications and improve the prognosis.
Topics: Arthritis, Juvenile; Child; Humans; Incidence; Macrophage Activation Syndrome; Prognosis; Spondylitis, Ankylosing
PubMed: 35090249
DOI: 10.3760/cma.j.cn112138-20210929-00666 -
Clinical Immunology (Orlando, Fla.) May 2020Approximately 5% of children with juvenile idiopathic arthritis (JIA) are diagnosed with the psoriatic form of the disease. In recent years, there has been substantial... (Review)
Review
Approximately 5% of children with juvenile idiopathic arthritis (JIA) are diagnosed with the psoriatic form of the disease. In recent years, there has been substantial scholarship demonstrating both heterogeneity within the disease as well as similarities with other forms of JIA, culminating in a recent proposal for the categorization of JIA that excluded the psoriatic form altogether. The purpose of the review is to summarize the clinical, epidemiologic, and genetic features of psoriatic JIA (PsJIA), comparing it with other categories of JIA including spondyloarthritis. We conclude that there are sufficient unique clinical and genetic features within PsJIA as well as similarities with its adult counterpart that warrant including it within the JIA paradigm.
Topics: Adult; Age of Onset; Arthritis, Juvenile; Arthritis, Psoriatic; Child; Comorbidity; Humans; Models, Immunological; Spondylarthritis
PubMed: 32229291
DOI: 10.1016/j.clim.2020.108396 -
Arthritis Care & Research Oct 2023Although interleukin-1 (IL-1)/IL-6 inhibitors are effective therapies for systemic juvenile idiopathic arthritis (JIA), some patients develop eosinophilia and lung...
OBJECTIVE
Although interleukin-1 (IL-1)/IL-6 inhibitors are effective therapies for systemic juvenile idiopathic arthritis (JIA), some patients develop eosinophilia and lung disease during treatment. This study was undertaken to retrospectively evaluate incidence and risk factors for eosinophilia and describe lung disease outcomes in IL-1/IL-6 inhibitor-exposed patients with systemic JIA.
METHODS
Among JIA patients at our institution exposed to interleukin-1 (IL-1)/IL-6 inhibitors (1995-2022), we compared incidence rate of eosinophilia in systemic JIA compared to other JIA, stratified by medication class (IL-1/IL-6 inhibitors, other cytokine inhibitors, methotrexate). We used Cox models to identify predictors of eosinophilia during IL-1/IL-6 inhibitor use and summarized treatment changes and outcomes after eosinophilia, including lung disease. HLA typing was performed on a clinical or research basis.
RESULTS
There were 264 new medication exposures in 75 patients with systemic JIA and 41 patients with other JIA. A total of 49% of patients with systemic JIA with HLA typing (n = 45) were positive for HLA-DRB1*15 alleles. Eosinophilia was common during IL-1/IL-6 inhibitor use and did not differ by systemic JIA compared to other JIA (0.08 and 0.07 per person-year, respectively; P = 0.30). Among systemic JIA patients, pretreatment macrophage activation syndrome (MAS) was associated with a higher rate of subsequent eosinophilia on biologic therapy (unadjusted hazard ratio 3.2 [95% confidence interval 1.2-8.3]). A total of 4 of 5 patients who switched therapy within 10 weeks of eosinophilia experienced disease flare compared to none of the patients who continued the original therapy. A total of 8 of 25 patients with pulmonary evaluations had lung disease, and all had severe manifestations of systemic JIA (MAS, intensive care unit stay). One death was attributed to systemic JIA-lung disease.
CONCLUSION
Eosinophilia is common in JIA patients using IL-1/IL-6 inhibitors. Severe disease may be associated with eosinophilia and lung disease in systemic JIA.
Topics: Humans; Child; Arthritis, Juvenile; Incidence; Retrospective Studies; Interleukin-6 Inhibitors; Lung Diseases; Eosinophilia; Risk Factors; Interleukin-1; Biological Products
PubMed: 37038961
DOI: 10.1002/acr.25129 -
Pediatric Rheumatology Online Journal Jul 2023Children and adolescents with juvenile idiopathic arthritis (JIA) may suffer from disability and disease-related damage. This study aimed to investigate the prevalence...
BACKGROUND
Children and adolescents with juvenile idiopathic arthritis (JIA) may suffer from disability and disease-related damage. This study aimed to investigate the prevalence of disability and damage, and identify the factors associated with articular and extra-articular damage in children and adolescents with JIA in a resource-restricted setting in Thailand.
METHODS
This cross-sectional study enrolled JIA patients during June 2019-June 2021. Disability was assessed using the Child Health Assessment Questionnaire (CHAQ) and Steinbrocker classification criteria. Damage was evaluated using the Juvenile Arthritis Damage Index (JADI) and the modified-JADI (mJADI) tools.
RESULTS
There were 101 patients (50.5% female) with median age of 11.8 years. Median disease duration was 32.7 months. Enthesitis-related arthritis (ERA) was the most common subtype (33.7%), followed by systemic JIA (25.7%). Thirty-three (32.7%) patients had delayed diagnosis ≥ 6 months. Moderate to severe disability was found in 20 (19.8%) patients. Patients with Steinbrocker functional classification > class I were seen in 17.9%. Thirty-seven (36.6%) patients had articular damage. Extra-articular complications were observed in 24.8%. Growth failure and striae were the most common complications in 7.8%. Leg-length discrepancy was documented in 5.0%. Ocular damage was found in 1 patient with ERA. Multivariable logistic regression analysis revealed Steinbrocker functional classification > class I (aOR: 18.1, 95% CI: 3.9-84.6; p < 0.001), delayed diagnosis ≥ 6 months (aOR: 8.5, 95%CI: 2.7-27.0; p < 0.001), and ERA (aOR: 5.7, 95%CI: 1.8-18.3; p = 0.004) as independent predictors of articular damage. Systemic corticosteroids use was the independent predictor of extra-articular damage (aOR: 3.8, 95%CI: 1.3-11.1; p = 0.013).
CONCLUSIONS
Disability and disease-related damage was identified in one-fifth and one-third of JIA patients. Early detection and treatment are essential for preventing permanent damage.
Topics: Humans; Adolescent; Child; Female; Male; Arthritis, Juvenile; Cross-Sectional Studies; Southeast Asian People; Thailand; Face
PubMed: 37430274
DOI: 10.1186/s12969-023-00852-5 -
Modern Rheumatology Sep 2021The aim of this study is to describe clinical features of patients with oligoarticular juvenile idiopathic arthritis (JIA) who achieved inactive disease at 3rd month and...
OBJECTIVE
The aim of this study is to describe clinical features of patients with oligoarticular juvenile idiopathic arthritis (JIA) who achieved inactive disease at 3rd month and also to determine the predictors of relapse and extended course.
METHODS
In the cohort study, 88 patients with oligoarticular JIA were retrospectively analyzed. The demographic data, clinical features, medications, relapse rates were recorded. Juvenile Arthritis Disease Activity Score (JADAS) and American College of Rheumatology Pediatric criteria were used to measure disease activity and treatment response at 3, 6 and 12 months.
RESULTS
Fifty-nine (67%) patients were females and the mean age at diagnosis was 7.9 ± 4.3 years. The odds of achieving inactive disease (JADAS ≤1) at 3rd month were increased by a lower JADAS27 score at admission. Forty-one (48.8%) of 84 patients relapsed. Ankle involvement at onset, high JADAS27 score at admission, increased ESR at admission and presence of synovial hypertrophy in imaging were risk factors for occurrence of relapse.
CONCLUSION
Our results show that a significant proportion of oligoarticular JIA patients relapse after inactive period. JADAS is a useful tool to guide the treatment decisions of patients who may be at risk of high disease activity and relapse.
Topics: Antirheumatic Agents; Arthritis, Juvenile; Child; Child, Preschool; Cohort Studies; Female; Humans; Male; Recurrence; Retrospective Studies; Treatment Outcome
PubMed: 33050742
DOI: 10.1080/14397595.2020.1836788 -
The Journal of Clinical Investigation Nov 2023Systemic autoimmune and autoinflammatory diseases are characterized by genetic and cellular heterogeneity. While current single-cell genomics methods provide insights...
Systemic autoimmune and autoinflammatory diseases are characterized by genetic and cellular heterogeneity. While current single-cell genomics methods provide insights into known disease subtypes, these analysis methods do not readily reveal novel cell-type perturbation programs shared among distinct patient subsets. Here, we performed single-cell RNA-Seq of PBMCs of patients with systemic juvenile idiopathic arthritis (SJIA) with diverse clinical manifestations, including macrophage activation syndrome (MAS) and lung disease (LD). We introduced two new computational frameworks called UDON and SATAY-UDON, which define patient subtypes based on their underlying disrupted cellular programs as well as associated biomarkers or clinical features. Among twelve independently identified subtypes, this analysis uncovered what we believe to be a novel complement and interferon activation program identified in SJIA-LD monocytes. Extending these analyses to adult and pediatric lupus patients found new but also shared disease programs with SJIA, including interferon and complement activation. Finally, supervised comparison of these programs in a compiled single-cell pan-immune atlas of over 1,000 healthy donors found a handful of normal healthy donors with evidence of early inflammatory activation in subsets of monocytes and platelets, nominating possible biomarkers for early disease detection. Thus, integrative pan-immune single-cell analysis resolved what we believe to be new conserved gene programs underlying inflammatory disease pathogenesis and associated complications.
Topics: Adult; Humans; Child; Arthritis, Juvenile; Biomarkers; Lung Diseases; Interferons; Genomics
PubMed: 37733441
DOI: 10.1172/JCI166741 -
The Journal of Rheumatology Jul 2022Data on uveitis in juvenile psoriatic arthritis (JPsA), a category of juvenile idiopathic arthritis (JIA), are scarce. We describe prevalence and risk factors for...
OBJECTIVE
Data on uveitis in juvenile psoriatic arthritis (JPsA), a category of juvenile idiopathic arthritis (JIA), are scarce. We describe prevalence and risk factors for JPsA-associated uveitis (JPsA-U).
METHODS
Cross-sectional data from the German National Pediatric Rheumatological Database (2002-2014) were used to characterize JPsA-U and assess risk factors for the development of uveitis.
RESULTS
Uveitis developed in 6.6% of 1862 patients with JPsA. Patients with JPsA-U were more frequently female (73.0 vs 62.9%, = 0.03), antinuclear antibody (ANA) positive (60.3 vs 37.0%, < 0.001), younger at JPsA onset (5.3 ± 4.1 vs 9.3 ± 4.4 yrs, < 0.001), and treated with disease-modifying antirheumatic drugs (DMARDs) significantly more frequently compared with JPsA patients without uveitis. On a multivariable analysis of a subgroup of 655 patients enrolled in the study ≤ 1 year after arthritis onset, mean clinical Juvenile Arthritis Disease Activity Score for 10 joints during study documentation was significantly associated with uveitis development. Children with early onset of JPsA (aged < 5 yrs vs ≥ 5 yrs) were significantly more frequently ANA positive (48.4% vs 35.7%, < 0.001), affected by uveitis (17.3% vs 3.8%, < 0.001), and treated with DMARDs (52.9% vs 43.8%, < 0.001), but less often affected by skin disease (55.3% vs 61.0%, = 0.03).
CONCLUSION
The characteristics of patients with JPsA developing uveitis are similar to those of patients with uveitis in other JIA categories, such as oligoarticular JIA. Children with early-onset JPsA are at a higher risk for ocular involvement. Our data support the notion of a major clinical difference between those patients with early vs late onset of JPsA.
Topics: Antirheumatic Agents; Arthritis, Juvenile; Child; Cross-Sectional Studies; Female; Germany; Humans; Risk Factors; Uveitis
PubMed: 35034000
DOI: 10.3899/jrheum.210755 -
Arthritis Care & Research Apr 2023Children with well-controlled juvenile idiopathic arthritis (JIA) frequently experience flares after medication discontinuation, but the outcomes of these flares have... (Observational Study)
Observational Study
Disease Recapture Rates After Medication Discontinuation and Flare in Juvenile Idiopathic Arthritis: An Observational Study Within the Childhood Arthritis and Rheumatology Research Alliance Registry.
OBJECTIVE
Children with well-controlled juvenile idiopathic arthritis (JIA) frequently experience flares after medication discontinuation, but the outcomes of these flares have not been well described. The objective of this study was to characterize the rates and predictors of disease recapture among children with JIA who restarted medication to treat disease flare.
METHODS
Children with JIA who discontinued conventional synthetic or biologic disease-modifying antirheumatic drugs for well-controlled disease but subsequently experienced a flare and restarted medication were identified from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) registry. The primary outcome was inactive disease (ID) (physician global assessment <1 and active joint count = 0) 6 months after flare.
RESULTS
A total of 333 patients had complete data for ID at 6 months after flare. The recapture rate for the cohort was 55%, ranging from 47% (persistent oligoarthritis) to 69% (systemic arthritis) (P = 0.4). Approximately 67% of children achieved ID by 12 months. In the multivariable model, history and reinitiation of biologic drugs were associated with increased odds of successful recapture (odds ratio [OR] 4.79 [95% confidence interval (95% CI) 1.22-18.78] and OR 2.74 [95% CI 1.62-4.63], respectively). Number of joints with limited range of motion was associated with decreased odds (OR 0.83 per 1 joint increase [95% CI 0.72-0.95]).
CONCLUSION
Approximately half of JIA flares post-discontinuation were recaptured within 6 months, but rates of recapture varied across JIA categories. These findings inform shared decision-making for patients, families, and clinicians regarding the risks and benefits of medication discontinuation. Better understanding of biologic predictors of successful recapture in JIA are needed.
Topics: Humans; Child; Arthritis, Juvenile; Rheumatology; Antirheumatic Agents; Biological Products; Registries; Treatment Outcome
PubMed: 35921198
DOI: 10.1002/acr.24994