-
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 -
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 -
Scandinavian Journal of Psychology Dec 2022A bulk of studies showed an association between stressful events and juvenile idiopathic arthritis (JIA) but failed to identify specific psychological tendencies that...
A bulk of studies showed an association between stressful events and juvenile idiopathic arthritis (JIA) but failed to identify specific psychological tendencies that contribute to the patients' vulnerability to stress. The purpose of this paper is to identify psychological tendencies specific to JIA that would unravel characteristic sources of stress. The study is based on the cognitive orientation model of health, which enables us to identify these kinds of tendencies in terms of four belief types (beliefs about self, general beliefs, beliefs about norms, and goals) that refer to specific themes. This is a case-control-cohort study that included a sample of 36 patients (mean age = 12.44 years, SD = 2.97, 21 females) and 41 matched controls (mean age = 13.15 years, SD = 2.01, 22 females). The JIA cognitive-orientation questionnaire was administered, and relevant medical parameters were recorded. The belief types differentiated between the two groups, and the patients were characterized using six themes. Examples of the themes are being over-sensitive, striving for success, and not fulfilling duties well. The themes differentiated between the participants' groups with an accuracy of 89.1%. The likelihood of the patients being characterized by the themes is 3.24-9.35 times more than the controls. The psychological tendencies of JIA were discussed as generators of stress (e.g., being over-sensitive) and cognitive conflicts (e.g., the contradiction between striving for success versus not fulfilling duties well). Also, the suggested reflections of these tendencies in the health workers' and patients' relationships, such as egalitarian interaction, and non-formal communication style, were described.
Topics: Child; Female; Humans; Adolescent; Arthritis, Juvenile; Cohort Studies; Surveys and Questionnaires; Case-Control Studies
PubMed: 35689406
DOI: 10.1111/sjop.12839 -
Anales de Pediatria Oct 2020
Topics: Arthritis, Juvenile; Humans; Turner Syndrome
PubMed: 34092340
DOI: 10.1016/j.anpede.2019.12.011 -
Annals of the Rheumatic Diseases Dec 2020Juvenile idiopathic arthritis (JIA) is an autoimmune disease and a common cause of chronic disability in children. Diagnosis of JIA is based purely on clinical symptoms,...
OBJECTIVES
Juvenile idiopathic arthritis (JIA) is an autoimmune disease and a common cause of chronic disability in children. Diagnosis of JIA is based purely on clinical symptoms, which can be variable, leading to diagnosis and treatment delays. Despite JIA having substantial heritability, the construction of genomic risk scores (GRSs) to aid or expedite diagnosis has not been assessed. Here, we generate GRSs for JIA and its subtypes and evaluate their performance.
METHODS
We examined three case/control cohorts (UK, US-based and Australia) with genome-wide single nucleotide polymorphism (SNP) genotypes. We trained GRSs for JIA and its subtypes using lasso-penalised linear models in cross-validation on the UK cohort, and externally tested it in the other cohorts.
RESULTS
The JIA GRS alone achieved cross-validated area under the receiver operating characteristic curve (AUC)=0.670 in the UK cohort and externally-validated AUCs of 0.657 and 0.671 in the US-based and Australian cohorts, respectively. In logistic regression of case/control status, the corresponding odds ratios (ORs) per standard deviation (SD) of GRS were 1.831 (1.685 to 1.991) and 2.008 (1.731 to 2.345), and were unattenuated by adjustment for sex or the top 10 genetic principal components. Extending our analysis to JIA subtypes revealed that the enthesitis-related JIA had both the longest time-to-referral and the subtype GRS with the strongest predictive capacity overall across data sets: AUCs 0.82 in UK; 0.84 in Australian; and 0.70 in US-based. The particularly common oligoarthritis JIA also had a GRS that outperformed those for JIA overall, with AUCs of 0.72, 0.74 and 0.77, respectively.
CONCLUSIONS
A GRS for JIA has potential to augment clinical JIA diagnosis protocols, prioritising higher-risk individuals for follow-up and treatment. Consistent with JIA heterogeneity, subtype-specific GRSs showed particularly high performance for enthesitis-related and oligoarthritis JIA.
Topics: Adolescent; Arthritis, Juvenile; Child; Cohort Studies; Female; Genetic Predisposition to Disease; Humans; Machine Learning; Male; Polymorphism, Single Nucleotide; Risk Factors
PubMed: 32887683
DOI: 10.1136/annrheumdis-2020-217421 -
Pediatric Rheumatology Online Journal Dec 2022Children with chronic diseases are reported to have increased risk of psychiatric comorbidity. Few studies have investigated this risk in juvenile idiopathic arthritis...
The risk of depression and anxiety is not increased in individuals with juvenile idiopathic arthritis - results from the south-Swedish juvenile idiopathic arthritis cohort.
BACKGROUND
Children with chronic diseases are reported to have increased risk of psychiatric comorbidity. Few studies have investigated this risk in juvenile idiopathic arthritis (JIA), with conflicting results. We performed a population-based, longitudinal cohort study of the risk of depression and anxiety in south-Swedish patients with juvenile arthritis.
METHODS
The south-Swedish JIA cohort (n = 640), a population-based cohort with validated JIA diagnosis 1980 - 2010 and comparators, a reference group of 3200 individuals free from JIA, matched for sex, year of birth and residential region, was used. Data on comorbid diagnosis with depression or anxiety were obtained from the Skåne Healthcare Register, containing all healthcare contacts in the region, from 1998 to 2019. We used Cox proportional models for the calculation of hazard ratios.
RESULTS
During the study period, 1998 to 2019, 93 (14.5%) of the individuals in the JIA group were diagnosed with depression, and 111 (17.3%) with anxiety. Corresponding numbers among the references was 474 (14.8%) with depression and 557 (17.4%) with anxiety. Hazard ratio for depression was 1.1 (95% CI 0.9 - 1.5) in females and 0.8 (95% CI 0.5 - 1.4) in males, and for anxiety 1.2 (95% CI 0.9 - 1.5) in females and 0.6 (95% CI 0.4 - 1.1) in males. There were no statistically significant hazard ratios when analyzing subgroups of JIA patients with long disease duration or treatment with disease-modifying antirheumatic drugs.
CONCLUSIONS
Individuals with JIA do not have any statistically increased risk of being diagnosed with depression or anxiety compared to matched references.
Topics: Child; Male; Female; Humans; Arthritis, Juvenile; Longitudinal Studies; Antirheumatic Agents; Proportional Hazards Models; Comorbidity
PubMed: 36494819
DOI: 10.1186/s12969-022-00765-9 -
Scientific Reports Jun 2023Systemic juvenile idiopathic arthritis (SJIA) is a chronic inflammatory disease of childhood with elevated serum IL-6 levels. As an inhibitor of IL-6R, tocilizumab (TCZ)...
Systemic juvenile idiopathic arthritis (SJIA) is a chronic inflammatory disease of childhood with elevated serum IL-6 levels. As an inhibitor of IL-6R, tocilizumab (TCZ) has been approved to treat SJIA patients. TCZ-induced hypofibrinogenemia has been only reported in adult cases and limited small case series with rheumatoid arthritis or giant cell arteritis. Here, we describe the incidence of TCZ-induced hypofibrinogenemia in SJIA patients and its possible influence on bleeding risk. SJIA patients with TCZ treatment in Shenzhen Children's hospital were retrospectively reviewed. Only those with the data on serum fibrinogen levels were included. Data on clinical manifestations, laboratory parameters, management, and sJADAS10-ESR score were collected. Laboratory data were extracted following the start of TCZ therapy at 2, 4, 8, 12, and 24 weeks thereafter. Seventeen SJIA patients with TCZ treatment were included. Thirteen (76.47%, 13/17) had hypofibrinogenemia. The lowest serum fibrinogen levels were even below 1.5 g/L in seven (41.17%, 7/17) patients. Among four patients without MTX treatment, two had obvious hypofibrinogenemia. Although five patients had already stopped steroid treatment 24 weeks after TCZ treatment, three of them still had hypofibrinogenemia. Only P14 had mild nasal mucosal bleeding occasionally. Coagulation tests were regularly performed in eight patients, of these, six had hypofibrinogenemia, which occurred following one to four doses of TCZ; continuation of TCZ treatment hadn't further aggravated hypofibrinogenemia. Serum fibrinogen levels were not decreased consistently with the improvement of sJADAS10-ESR score in more than half of these eight patients. Factor XIII was detected in six patients and none was identified with Factor XIII deficiency. TCZ alone may induce hypofibrinogenemia in SJIA patients. Continuation of TCZ treatment may be safe for most SJIA patients. But for SJIA patients with indications of surgery or complicated with MAS, the risk of hemorrhage should be regularly evaluated during TCZ treatment. The association between TCZ-induced hypofibrinogenemia and factor XIII deficiency remains uncertain.Trial registration: Not applicable; this was a retrospective study.
Topics: Child; Adult; Humans; Arthritis, Juvenile; Retrospective Studies; Afibrinogenemia; Factor XIII Deficiency; Disseminated Intravascular Coagulation; Fibrinogen; Treatment Outcome
PubMed: 37270663
DOI: 10.1038/s41598-023-36246-6 -
Rheumatology International Feb 2022The study was aimed to review a rare coexistence of type 1 diabetes (T1D) and juvenile idiopathic arthritis (JIA) regarding different clinical approaches to the... (Review)
Review
The study was aimed to review a rare coexistence of type 1 diabetes (T1D) and juvenile idiopathic arthritis (JIA) regarding different clinical approaches to the management and treatment options. Medical complications of the two autoimmune disorders in children and adolescents have been evaluated, particularly in those treated with glucocorticosteroids (GCS) and insulin. A review of the literature regarding reports on concomitant T1D and JIA was conducted using resources available in Medline, Google Scholar, and Web of Science databases, with a specific focus on the combination of T1D and JIA in a pediatric population. The review was extended by our analysis of two patients treated in a single center for this comorbidity. Eligible reports of four cases were found, and including our two original records, a total of six pediatric patients (5 females) were analyzed, of which three had also other autoimmune diseases (thyroiditis, coeliac disease, autoimmune hepatitis), whereas four had been treated with a long-term GCS, and two were receiving biological therapy (etanercept or adalimumab). Only one of them had good metabolic control of diabetes. Diabetes in childhood may coexist with other autoimmune diseases, including rheumatologic conditions. Hyperglycemia can worsen JIA therapy by induction and maintaining inflammation. Using modern diabetes technologies (like personal insulin pumps and continuous glucose monitoring) helps to minimize the deteriorating effect of JIA exacerbations and the rheumatoid treatment on metabolic control of diabetes.
Topics: Adolescent; Arthritis, Juvenile; Diabetes Mellitus, Type 1; Female; Humans; Infant
PubMed: 34999914
DOI: 10.1007/s00296-021-05083-z -
Arthritis & Rheumatology (Hoboken, N.J.) Nov 2019
Topics: Arthritis, Juvenile; Humans; Lung Diseases
PubMed: 31390168
DOI: 10.1002/art.41071 -
Annals of the Rheumatic Diseases Apr 2023In childhood arthritis, collectively known as Juvenile idiopathic arthritis (JIA), the rapid rise of available licensed biological and targeted small molecule treatments... (Review)
Review
In childhood arthritis, collectively known as Juvenile idiopathic arthritis (JIA), the rapid rise of available licensed biological and targeted small molecule treatments in recent years has led to improved outcomes. However, real-world data from multiple countries and registries show that despite a large number of available drugs, many children and young people continue to suffer flares and experience significant periods of time with active disease for many years. More than 50% of young people with JIA require ongoing immune suppression well into adult life, and they may have to try multiple different treatments in that time. There are currently no validated tools with which to select specific treatments, nor biomarkers of response to assist in such choices, therefore, current management uses essentially a trial-and-error approach. A further consequence of recent progress is a reducing pool of available children or young people who are eligible for new trials. In this review we consider how progress towards a molecular based approach to defining treatment targets and informing trial design in JIA, combined with novel approaches to clinical trials, could provide strategies to maximise discovery and progress, in order to move towards precision medicine for children with arthritis.
Topics: Child; Adult; Humans; Adolescent; Arthritis, Juvenile; Precision Medicine; Registries
PubMed: 36600186
DOI: 10.1136/ard-2022-222553