-
Arthritis Care & Research Mar 2024Systemic juvenile idiopathic arthritis-associated lung disease (SJIA-LD) is a life-threatening disease complication. Key questions remain regarding clinical course and...
OBJECTIVE
Systemic juvenile idiopathic arthritis-associated lung disease (SJIA-LD) is a life-threatening disease complication. Key questions remain regarding clinical course and optimal treatment approaches. The objectives of the study were to detail management strategies after SJIA-LD detection, characterize overall disease courses, and measure long-term outcomes.
METHODS
This was a prospective cohort study. Clinical data were abstracted from the electronic medical record, including current clinical status and changes since diagnosis. Serum biomarkers were determined and correlated with presence of LD.
RESULTS
We enrolled 41 patients with SJIA-LD, 85% with at least one episode of macrophage activation syndrome and 41% with adverse reactions to a biologic. Although 93% of patients were alive at last follow-up (median 2.9 years), 37% progressed to requiring chronic oxygen or other ventilator support, and 65% of patients had abnormal overnight oximetry studies, which changed over time. Eighty-four percent of patients carried the HLA-DRB1*15 haplotype, significantly more than patients without LD. Patients with SJIA-LD also showed markedly elevated serum interleukin-18 (IL-18), variable C-X-C motif chemokine ligand 9 (CXCL9), and significantly elevated matrix metalloproteinase 7. Treatment strategies showed variable use of anti-IL-1/6 biologics and addition of other immunomodulatory treatments and lung-directed therapies. We found a broad range of current clinical status independent of time from diagnosis or continued biologic treatment. Multidomain measures of change showed imaging features were the least likely to improve with time.
CONCLUSION
Patients with SJIA-LD had highly varied courses, with lower mortality than previously reported but frequent hypoxia and requirement for respiratory support. Treatment strategies were highly varied, highlighting an urgent need for focused clinical trials.
Topics: Child; Humans; Arthritis, Juvenile; Prospective Studies; Lung; Lung Diseases; Macrophage Activation Syndrome; Disease Progression
PubMed: 37691306
DOI: 10.1002/acr.25234 -
Seminars in Arthritis and Rheumatism Oct 2023To describe clinical characteristics of patients with Still's disease treated with methotrexate (MTX) and to assess drug effectiveness evaluating change in disease...
OBJECTIVES
To describe clinical characteristics of patients with Still's disease treated with methotrexate (MTX) and to assess drug effectiveness evaluating change in disease activity, reduction of inflammatory markers, and glucocorticoid (GC)-sparing effect.
METHODS
Patients with Still's disease treated with MTX were assessed among those included in AIDA Network Still Disease Registry.
RESULTS
In this registry, 171 patients with Still's disease were treated with MTX (males 43.3%, age 37.1 ± 16.0 years). They were mainly characterised by joint features and fever without a prominent multiorgan involvement. MTX was administered with GCs in 68.4% of patients, with other conventional synthetic DMARDs in 6.4%, and with biologic DMARDs in 25.1%. A significant reduction of the modified systemic score was observed, and 38.6% patients were codified as being in clinical remission at the end of follow-up. The concomitant administration of a biologic DMARD resulted a predictor of the clinical remission. Furthermore, a reduction of inflammatory markers and ferritin levels was observed following the administration of MTX. Additionally, a marked reduction of the dosage of concomitant GCs was identified, while 36.7% discontinued such drugs. Male gender appeared as a predictor of GC discontinuation. MTX was discontinued in 12.3% of patients because of adverse effects, and in 12.3% for lack of efficacy.
CONCLUSIONS
Clinical characteristics of patients with Still's disease treated with MTX were described, mainly joint features and fever without a prominent multiorgan involvement. The clinical usefulness of MTX was reported in reducing the disease activity, decreasing the inflammatory markers, and as GC-sparing agent.
Topics: Humans; Male; Young Adult; Adult; Middle Aged; Methotrexate; Arthritis, Juvenile; Antirheumatic Agents; Glucocorticoids; Registries; Fever; Biological Products; Still's Disease, Adult-Onset
PubMed: 37517110
DOI: 10.1016/j.semarthrit.2023.152244 -
Pediatric Rheumatology Online Journal Mar 2024Biomarkers may be useful in monitoring disease activity in juvenile idiopathic arthritis (JIA). With new treatment options and treatment goals in JIA, there is an urgent...
BACKGROUND
Biomarkers may be useful in monitoring disease activity in juvenile idiopathic arthritis (JIA). With new treatment options and treatment goals in JIA, there is an urgent need for more sensitive and responsive biomarkers.
OBJECTIVE
We aimed to investigate the patterns of 92 inflammation-related biomarkers in serum and saliva in a group of Norwegian children and adolescents with JIA and controls and in active and inactive JIA. In addition, we explored whether treatment with tumor necrosis factor inhibitors (TNFi) affected the biomarker levels.
METHODS
This explorative, cross-sectional study comprised a subset of children and adolescents with non-systemic JIA and matched controls from the Norwegian juvenile idiopathic arthritis study (NorJIA Study). The JIA group included individuals with clinically active or inactive JIA. Serum and unstimulated saliva were analyzed using a multiplex assay of 92 inflammation-related biomarkers. Welch's t-test and Mann-Whitney U-test were used to analyze the differences in biomarker levels between JIA and controls and between active and inactive disease.
RESULTS
We included 42 participants with JIA and 30 controls, predominantly females, with a median age of 14 years. Of the 92 biomarkers, 87 were detected in serum, 73 in saliva, and 71 in both biofluids. A pronounced difference between serum and salivary biomarker patterns was found. Most biomarkers had higher levels in serum and lower levels in saliva in JIA versus controls, and in active versus inactive disease. In serum, TNF and S100A12 levels were notably higher in JIA and active disease. The TNF increase was less pronounced when excluding TNFi-treated individuals. In saliva, several biomarkers from the chemokine family were distinctly lower in the JIA group, and levels were even lower in active disease.
CONCLUSION
In this explorative study, the serum and salivary biomarker patterns differed markedly, suggesting that saliva may not be a suitable substitute for serum when assessing systemic inflammation in JIA. Increased TNF levels in serum may not be a reliable biomarker for inflammatory activity in TNFi-treated children and adolescents with JIA. The lower levels of chemokines in saliva in JIA compared to controls and in active compared to inactive disease, warrant further investigation.
Topics: Child; Adolescent; Female; Humans; Male; Arthritis, Juvenile; Cross-Sectional Studies; Saliva; Inflammation; Biomarkers
PubMed: 38461338
DOI: 10.1186/s12969-024-00972-6 -
Human Vaccines & Immunotherapeutics Dec 2023Patients with pediatric rheumatic diseases (PRDs) have higher morbidity and mortality associated with infectious diseases. Vaccination is an effective way to prevent...
Patients with pediatric rheumatic diseases (PRDs) have higher morbidity and mortality associated with infectious diseases. Vaccination is an effective way to prevent infection. This study aimed to understand the vaccination status, vaccination-related attitudes, and adverse reactions in patients with PRDs in one of the largest Pediatric Rheumatic and Immune centers in China. A cross-sectional study using an online questionnaire was conducted among the caregivers of patients with PRDs admitted to the Children's Hospital of Chongqing Medical University. 189 valid questionnaires were collected. The most two common PRDs in this study were juvenile idiopathic arthritis (29.6%) and systemic lupus erythematosus (19.6%). Univariate analysis and multivariate logistic regression were used to identify potential factors associated with vaccination completion among these patients. Univariate analysis suggested that the age of onset, course of the disease, treatment duration, disease duration <1 month, disease duration ≥24 months, treatment duration <1 month, use of biological agents, at least one hospitalization, whether with one-time intravenous human immunoglobulin, caregiver concerns about vaccination before or after illness, and vaccine hesitancy may affect the scheduled vaccination completion by age in patients ( < .05). Multivariate logistic regression analysis showed that the age of onset (OR, 1.013; 95% CI, 1.005-1.022; = .002) and caregiver concerns about vaccination before illness (OR, 0.600; 95% CI, 0.428-0.840; = .003) independently influenced patients' completion of scheduled vaccinations. This study suggests that rheumatic disease and treatment may influence age-appropriate vaccination. Appropriate education for patients and carers may improve vaccination cognition and attitudes.
Topics: Humans; Child; Infant; Cross-Sectional Studies; Vaccination; Rheumatic Diseases; Hospitalization; China
PubMed: 37211623
DOI: 10.1080/21645515.2023.2215108 -
Research Square Nov 2023Children with chronic illnesses, including arthritis, are at increased risk for adverse psychosocial outcomes influenced by social determinants of health (SDOH)....
OBJECTIVE
Children with chronic illnesses, including arthritis, are at increased risk for adverse psychosocial outcomes influenced by social determinants of health (SDOH). Comparing psychosocial outcomes in families affected by juvenile arthritis compared to other chronic illnesses may help identify areas in need of special attention vs areas that may be addressed through adopting other disease examples' care models. We examined child and parent behavioral health outcomes for families with juvenile arthritis compared to diabetes, accounting for SDOH.
METHODS
Secondary data analysis of the National Survey of Children's Health including 365 children (<18yrs) with arthritis and 571 children with diabetes. Psychosocial outcomes were depression, anxiety, ADHD, physical pain, behavioral problems, and treatment for mental health. School outcomes were school engagement, school absence, involvement in clubs/organization, and involvement in organized activities. Parent outcomes were family resilience, emotional support, coping with daily demands of raising a child, job change due to problems with childcare, and parent mental health. SDOH variables were food insecurity, food/cash assistance, unsafe neighborhood, detracting neighborhood elements, parent education, households earning <100% of the federal poverty line. Logistic regression analyses were utilized to examine variation in child and parent outcomes, variation in SDOH, and the role of SDOH.
RESULTS
Children with arthritis experienced significantly more physical pain, anxiety, depression, ADHD, and behavior problems compared to children with diabetes. Children with arthritis were more likely to see a mental health professional and get treatment for problems with emotions/behaviors. When considering SDOH, children with arthritis were still more likely to experience adverse psychosocial outcomes but were no longer more likely to get treatment. Children with arthritis had increased likelihood of school absence and were less involved in organized activities than children with diabetes. Parents of children with arthritis had poorer mental health than parents of children with diabetes. SDOH were more prevalent in children with arthritis than children with diabetes.
CONCLUSIONS
Increased risk for adverse psychosocial outcomes in youth with arthritis compared to youth with diabetes indicates a need to mirror endocrinology models of care in rheumatology clinics. The role of SDOH highlights the need for regular SDOH screening in clinic.
PubMed: 38076886
DOI: 10.21203/rs.3.rs-3610878/v1 -
Pediatric Rheumatology Online Journal Jul 2023Juvenile idiopathic arthritis (JIA) is a common pediatric rheumatic condition and is associated with symptoms such as joint pain that can negatively impact...
BACKGROUND
Juvenile idiopathic arthritis (JIA) is a common pediatric rheumatic condition and is associated with symptoms such as joint pain that can negatively impact health-related quality of life. To effectively manage pain in JIA, young people, their families, and health care providers (HCPs) should be supported to discuss pain management options and make a shared decision. However, pain is often under-recognized, and pain management discussions are not optimal. No studies have explored decision-making needs for pain management in JIA using a shared decision making (SDM) model. We sought to explore families' decision-making needs with respect to pain management among young people with JIA, parents/caregivers, and HCPs.
METHODS
We conducted semi-structured virtual or face-to-face individual interviews with young people with JIA 8-18 years of age, parents/caregivers and HCPs using a qualitative descriptive study design. We recruited participants online across Canada and the United States, from a hospital and from a quality improvement network. We used interview guides based on the Ottawa Decision Support Framework to assess decision-making needs. We audiotaped, transcribed verbatim and analyzed interviews using thematic analysis.
RESULTS
A total of 12 young people (n = 6 children and n = 6 adolescents), 13 parents/caregivers and 11 HCPs participated in interviews. Pediatric HCPs were comprised of rheumatologists (n = 4), physical therapists (n = 3), rheumatology nurses (n = 2) and occupational therapists (n = 2). The following themes were identified: (1) need to assess pain in an accurate manner; (2) need to address pain in pediatric rheumatology consultations; (3) need for information on pain management options, especially nonpharmacological approaches; (4) importance of effectiveness, safety and ease of use of treatments; (5) need to discuss young people/families' values and preferences for pain management options; and the (6) need for decision support. Themes were similar for young people, parents/caregivers and HCPs, although their respective importance varied.
CONCLUSIONS
Findings suggest a need for evidence-based information and communication about pain management options, which would be addressed by decision support interventions and HCP training in pain and SDM. Work is underway to develop such interventions and implement them into practice to improve pain management in JIA and in turn lead to better health outcomes.
Topics: Adolescent; Child; Humans; Arthritis, Juvenile; Pain; Pain Management; Qualitative Research; Quality of Life; Decision Making, Shared
PubMed: 37491246
DOI: 10.1186/s12969-023-00849-0 -
Revista Paulista de Pediatria : Orgao... 2023To conduct a bibliographic review on tuberculosis (TB) disease in children and adolescents with rheumatic diseases, being managed with biologic therapy. (Review)
Review
OBJECTIVE
To conduct a bibliographic review on tuberculosis (TB) disease in children and adolescents with rheumatic diseases, being managed with biologic therapy.
DATA SOURCE
An integrative review with a search in the U.S. National Library of Medicine and the National Institutes of Health (PubMed) using the following descriptors and Boolean operators: (["tuberculosis"] AND (["children"] OR ["adolescent"]) AND ["rheumatic diseases"] AND (["tumor necrosis factor-alpha"] OR ["etanercept"] OR ["adalimumab"] OR ["infliximab"] OR ["biological drugs"] OR ["rituximab"] OR ["belimumab"] OR ["tocilizumab"] OR ["canakinumab"] OR ["golimumab"] OR ["secukinumab"] OR ["ustekinumab"] OR ["tofacitinib"] OR ["baricitinib"] OR ["anakinra"] OR ["rilonacept"] OR ["abatacept"]), between January 2010 and October 2021.
DATA SYNTHESIS
Thirty-seven articles were included, with the total number of 36,198 patients. There were 81 cases of latent tuberculosis infection (LTBI), 80 cases of pulmonary tuberculosis (PTB), and four of extrapulmonary tuberculosis (EPTB). The main rheumatic disease was juvenile idiopathic arthritis. Among LTBI cases, most were diagnosed at screening and none progressed to TB disease during follow-up. Of the TB cases using biologics, most used tumor necrosis factor-alpha inhibitors (anti-TNFα) drugs. There was only one death.
CONCLUSIONS
The study revealed a low rate of active TB in pediatric patients using biologic therapy. Screening for LTBI before initiating biologics should be done in all patients, and treatment, in cases of positive screening, plays a critical role in preventing progression to TB disease.
Topics: United States; Humans; Child; Adolescent; Antirheumatic Agents; Biological Factors; Tuberculosis; Rheumatic Diseases; Latent Tuberculosis; Biological Products; Tumor Necrosis Factors
PubMed: 37436237
DOI: 10.1590/1984-0462/2024/42/2022084 -
Clinical Pediatric Endocrinology : Case... 2024Chronic inflammatory conditions, such as juvenile idiopathic arthritis, are associated with growth failure. Growth failure appears to be correlated with both the effects... (Review)
Review
Chronic inflammatory conditions, such as juvenile idiopathic arthritis, are associated with growth failure. Growth failure appears to be correlated with both the effects of inflammation and negative effects of glucocorticoids (used as therapeutic option) on the growth hormone axis and locally on the growth plate and bone metabolism. In the last decade, the introduction of biologics has changed the disease course regarding consequences and outcomes. Anyway in some cases, treatment with biologics has failed in restoring normal growth in patients with juvenile idiopathic arthritis; in contrast, several studies have reported improved height velocity and growth rate in patients with juvenile idiopathic arthritis treated with growth hormone. This study aimed to evaluate the impact of growth hormone treatment on the growth and pubertal development in juvenile idiopathic arthritis patients through a narrative review of the literature over the last four decades.
PubMed: 38299178
DOI: 10.1297/cpe.2023-0036 -
Pediatric Radiology Apr 2024In recent years, imaging has become increasingly important to confirm diagnosis, monitor disease activity, and predict disease course and outcome in children with... (Review)
Review
In recent years, imaging has become increasingly important to confirm diagnosis, monitor disease activity, and predict disease course and outcome in children with juvenile idiopathic arthritis (JIA). Over the past few decades, great efforts have been made to improve the quality of diagnostic imaging and to reach a consensus on which methods and scoring systems to use. However, there are still some critical issues, and the diagnosis, course, and management of JIA are closely related to clinical assessment. This review discusses the main indications for conventional radiography (XR), musculoskeletal ultrasound (US), and magnetic resonance imaging (MRI), while trying to maintain a clinical perspective. The diagnostic-therapeutic timing at which one or the other method should be used, depending on the disease/patient phenotype, will be assessed, considering the main advantages and disadvantages of each imaging modality according to the currently available literature. Some brief clinical case scenarios on the most frequently and severely involved joints in JIA are also presented.
Topics: Child; Humans; Arthritis, Juvenile; Ultrasonography; Magnetic Resonance Imaging
PubMed: 38015293
DOI: 10.1007/s00247-023-05815-2 -
Frontiers in Immunology 2023Juvenile idiopathic arthritis (JIA), a clinically variable disease characterized by autoimmune arthritis, affects children, and its immunopathology remains elusive....
INTRODUCTION
Juvenile idiopathic arthritis (JIA), a clinically variable disease characterized by autoimmune arthritis, affects children, and its immunopathology remains elusive. Alterations in neutrophil biology play an important role in this disease. In the present study, we aimed to explore the features of low-density neutrophils (LDNs) in patients with JIA.
METHODS
Gene expression of peripheral blood mononuclear cells (PBMCs) from children with distinct subtypes of JIA was analyzed by NanoString Immunology panel. Presence of LDNs was ascertained by flow cytometry and the release of neutrophil-associated products were analyzed by LUMINEX.
RESULTS
LDNs were detected in patients' peripheral blood mononuclear cells (PBMCs) after density gradient centrifugation. Transcriptomic analysis of JIA PBMCs revealed that genes related to neutrophil degranulation were markedly upregulated. The number of LDNs and level of their degranulation products increased in patients' PBMCs and correlated with serum calprotectin, but not with disease activity, sedimentation rate and C-reactive protein (CRP) levels. The phenotypes of LDNs varied from those of normal-density neutrophils and healthy donor LDNs. Phenotypical analysis revealed LDNs are immature and primed population with decreased suppressive capacity. A negative correlation between surface proteins CD62L, CD66b, and CD11b and the number of inflamed joints/JADAS was established.
CONCLUSION
Our results describe LDNs as primed, degranulated, immature cells with impaired suppressive activities. This work thus contributes to the increasing body of evidence that LDNs in JIA are altered and their role in the disease immunopathogenesis and possible clinical associations should be investigated further.
Topics: Child; Humans; Neutrophils; Arthritis, Juvenile; Leukocytes, Mononuclear; Neutrophil Activation; Flow Cytometry
PubMed: 37915575
DOI: 10.3389/fimmu.2023.1229520