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Annals of the Rheumatic Diseases Oct 2023Haemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are life-threatening systemic hyperinflammatory syndromes that can develop in most...
The 2022 EULAR/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS).
OBJECTIVE
Haemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are life-threatening systemic hyperinflammatory syndromes that can develop in most inflammatory contexts. They can progress rapidly, and early identification and management are critical for preventing organ failure and mortality. This effort aimed to develop evidence-based and consensus-based points to consider to assist clinicians in optimising decision-making in the of diagnosis, treatment and monitoring of HLH/MAS.
METHODS
A multinational, multidisciplinary task force of physician experts, including adult and paediatric rheumatologists, haematologist/oncologists, immunologists, infectious disease specialists, intensivists, allied healthcare professionals and patients/parents, formulated relevant research questions and conducted a systematic literature review (SLR). Delphi methodology, informed by SLR results and questionnaires of experts, was used to generate statements aimed at assisting early decision-making and optimising the initial care of patients with HLH/MAS.
RESULTS
The task force developed 6 overarching statements and 24 specific points to consider relevant to early recognition of HLH/MAS, diagnostic approaches, initial management and monitoring of HLH/MAS. Major themes included the simultaneous need for prompt syndrome recognition, systematic evaluation of underlying contributors, early intervention targeting both hyperinflammation and likely contributors, careful monitoring for progression/complications and expert multidisciplinary assistance.
CONCLUSION
These 2022 EULAR/American College of Rheumatology points to consider provide up-to-date guidance, based on the best available published data and expert opinion. They are meant to help guide the initial evaluation, management and monitoring of patients with HLH/MAS in order to halt disease progression and prevent life-threatening immunopathology.
Topics: Child; Adult; Humans; United States; Lymphohistiocytosis, Hemophagocytic; Macrophage Activation Syndrome; Rheumatology; Consensus
PubMed: 37487610
DOI: 10.1136/ard-2023-224123 -
The Journal of Clinical Pediatric... Sep 2023Although periodontal diseases have been widely reported in patients with juvenile idiopathic arthritis (JIA), their association with JIA remains controversial. This... (Meta-Analysis)
Meta-Analysis
Although periodontal diseases have been widely reported in patients with juvenile idiopathic arthritis (JIA), their association with JIA remains controversial. This systematic review and meta-analysis aimed to evaluate the association between JIA and periodontal diseases to facilitate oral health management and periodontal disease prevention in JIA patients. We conducted a comprehensive search of Web of Science, Cochrane Library, PubMed, Embase, Chinese Scientific and Technological Journal (VIP) database, Wan Fang Data, China National Knowledge Infrastructure (CNKI), and China Biomedical Literature Database (CBM) up to 30 September 2022, without publication dates or language restrictions. Two authors independently evaluated observational studies for inclusion, and the quality of the included studies was assessed using the Newcastle Ottawa Scale (NOS) and the Agency for Healthcare Research and Quality (AHRQ). Continuous variables are presented as mean difference (MD) and 95% confidence interval (CI). Parameters of the simplified oral hygiene index (OHI-S), plaque index (PI), gingival index (GI), clinical attachment loss (CAL), and probing depth (PD) were considered as outcome measures and were compared between JIA patients and healthy controls. The initial search comprised 15 studies with a total of 1537 individuals. The meta-analysis showed the parameters of OHI-S (MD = 0.12, 95% CI: 0.04-0.19, = 0.002), PI (MD = 2.08, 95% CI: 1.67-2.50, < 0.00001), GI (MD = 0.50, 95% CI: 0.17-0.82, = 0.003), CAL (MD = 0.22, 95% CI: 0.01-0.43, = 0.04), and PD (MD = 1.42, 95% CI: 0.08-2.77, = 0.04) in JIA patients were significantly higher than those of healthy controls. All of the included studies were of high quality. This systematic review and meta-analysis showed a possible association between JIA and periodontal diseases. Therefore, it is recommended to continuously pay attention to the periodontal health of JIA patients and fully explore the underlying mechanism.
Topics: United States; Humans; Arthritis, Juvenile; Periodontal Diseases; Administration, Oral; Databases, Factual; Oral Health
PubMed: 37732432
DOI: 10.22514/jocpd.2023.050 -
Rheumatology (Oxford, England) Nov 2023JIA is the most common type of arthritis in children and adolescents, causing joint damage, chronic pain and disability. Deconditioning is also prevalent in patients... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
JIA is the most common type of arthritis in children and adolescents, causing joint damage, chronic pain and disability. Deconditioning is also prevalent in patients with JIA due to both inactivity and the disease progression, resulting in reduced cardiorespiratory fitness (CRF). We aimed to evaluate CRF of patients with JIA compared with healthy controls.
METHODS
This is a systematic review and meta-analysis of studies using cardiopulmonary exercise testing (CPET) to examine differences in determinants of CRF between patients with JIA vs healthy controls. The primary outcome was peak oxygen uptake (VO2peak). Literature search involved PubMed, Web of Science and Scopus databases, manual search of article references and grey literature. Quality assessment was undertaken with Newcastle-Ottawa Scale.
RESULTS
From 480 literature records initially retrieved, eight studies (538 participants) were included in final meta-analysis. VO2peak was significantly lower in patients with JIA compared with controls [weighted mean difference (WMD): -5.95 ml/kg/min (95% CI -9.26, -2.65)]. Exercise duration and VO2peak (% predicted) were found to be significantly impaired in patients with JIA compared with controls [standardized mean difference: -0.67 (95% CI -1.04, -0.29) and WMD: -11.31% (95% CI -20.09, -2.53), respectively], while no significant differences were found in maximum heart rate.
CONCLUSION
VO2peak and other CPET variables were lower in patients with JIA compared with controls, indicating reduced CRF in the former. Overall, exercise programs for patients with JIA should be promoted as part of their treatment to improve physical fitness and reduce muscle atrophy.
PROSPERO REGISTRATION
CRD42022380833.
Topics: Child; Adolescent; Humans; Exercise Test; Cardiorespiratory Fitness; Arthritis, Juvenile; Oxygen Consumption; Exercise
PubMed: 37280055
DOI: 10.1093/rheumatology/kead272 -
Lipids in Health and Disease Aug 2023The purpose of this study was to comprehensively evaluate the lipid profiles in patients with juvenile idiopathic arthritis (JIA). (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
The purpose of this study was to comprehensively evaluate the lipid profiles in patients with juvenile idiopathic arthritis (JIA).
METHODS
The literature and relevant reviews were searched for published clinical studies on the relationship between JIA and blood lipid levels. The Newcastle-Ottawa scale (NOS) was applied to evaluate the risk and methodological value of the included case‒control and cohort studies. Standardized mean differences (SMDs) and 95% confidence intervals were derived for all variables with adequate unprocessed data. This meta-analysis followed the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) guidelines.
RESULTS
In total, 16 studies were incorporated through screening. The analysis findings revealed that the levels of very low-density lipoprotein cholesterol [SMD=-0.411, 95% CI (-0.774~-0.048), P = 0.026], high-density lipoprotein cholesterol [SMD=-0.528, 95% CI (-0.976~-0.079), P = 0.021], and apolipoprotein A1 [SMD=-1.050, 95% CI (-1.452~-0.647), P = 0.000] in JIA patients were statistically lower than those observed in healthy controls. The level of low-density lipoprotein cholesterol [SMD = 0.202, 95% CI (0.003 ~ 0.400), P = 0.046] was significantly higher in JIA patients than in healthy controls. In JIA patients, body mass index [SMD=-0.189, 95% CI (-0.690 ~ 0.311), P = 0.459], high-density lipoprotein [SMD =-1.235, 95% CI (-2.845 ~ 0.374), P = 0.133), low-density lipoprotein [SMD = 0.616, 95% CI (-0.813 ~ 2.046), P = 0.398), triglycerides (SMD = 0.278, 95% CI (-0.182 ~ 0.738), P = 0.236], total cholesterol [SMD=-0.073, 95% CI (-0.438 ~ 0.293), P = 0.696] and apolipoprotein B levels [SMD = 0.226, 95% CI (-0.133 ~ 0.585), P = 0.217] were not significantly different from those in healthy controls.
CONCLUSIONS
The outcomes of this meta-analysis suggest that dyslipidemia is common in JIA patients compared to healthy controls. Patients with JIA have a significantly increased risk of atherosclerosis and cardiovascular disease later in life.
Topics: Humans; Arthritis, Juvenile; Apolipoproteins B; Cholesterol, HDL; Cholesterol, LDL; Lipoproteins, HDL
PubMed: 37626321
DOI: 10.1186/s12944-023-01885-1 -
Journal of Oral Rehabilitation Oct 2023The objectives of this systematic review were to evaluate the correlation between Ultrasound (US) and Magnetic Resonance Imaging (MRI) in patients with JIA and to... (Review)
Review
OBJECTIVES
The objectives of this systematic review were to evaluate the correlation between Ultrasound (US) and Magnetic Resonance Imaging (MRI) in patients with JIA and to investigate the association with Temporomandibular Disorders (TMD).
MATERIALS AND METHODS
The protocol was registered in PROSPERO (CRD42022312734). Databases Medline, Embase, Cochrane Central Register of Controlled Trials, Scopus, Web of Science, Latin American and Caribbean Health Sciences Literature were searched. Eligibility criteria were patients with JIA subjected to diagnostic evaluation using US and MRI. No language restrictions were applied. After duplicate study selection, data extraction and risk of bias assessment according to Cochrane were conducted. Data extraction of patients was conducted by two independent authors.
RESULTS
Five observational studies were included with 217 participants (153 females and 64 males; mean age 11.3 years). The quality of the studies was overall satisfactory. The correlation between US and MRI in children with JIA was 'moderate' in acute arthritis while the chronic arthritis correlated positively in two studies.
CONCLUSIONS
Even if MRI remains the more accurate imaging modality for the detection of TMJ of patients with JIA, US may be useful to early detect pathological conditions and to address the patient with JIA and putative TMJ involvement to a more accurate diagnosis with MRI and consequent appropriate treatment management.
CLINICAL RELEVANCE
MRI should be deemed necessary only secondary to less-invasive assessments with US just to confirm the diagnosis or to increase sensitivity, accuracy of positive predictive values detected.
Topics: Male; Child; Female; Humans; Arthritis, Juvenile; Temporomandibular Joint; Temporomandibular Joint Disorders; Magnetic Resonance Imaging; Ultrasonography
PubMed: 37301975
DOI: 10.1111/joor.13529 -
Current Rheumatology Reports Feb 2024We performed a systematic review of the literature on the epidemiology, pathogenesis, clinical and laboratory characterization, and treatment of calcinosis in patients... (Review)
Review
PURPOSE OF REVIEW
We performed a systematic review of the literature on the epidemiology, pathogenesis, clinical and laboratory characterization, and treatment of calcinosis in patients with juvenile dermatomyositis (JDM). A qualitative systematic review was conducted from January 1975 to April 2023 according to the PRISMA protocol using three electronic databases: PubMed, Web of Science, and Scopus. Studies were analyzed based on the following eligibility criteria: at least one combination of the terms described in the search strategy appeared in the title, written in English, Portuguese, or Spanish, and addressed the epidemiology, pathogenesis, diagnosis, and treatment of calcinosis in juvenile dermatomyositis. Systematic or scoping reviews, letters, clinical images, book chapters, abstracts, inflammatory myopathy in other connective tissue diseases, idiopathic inflammatory myopathies in adults, and purely qualitative studies were excluded.
RECENT FINDINGS
Seventy-five studies were included. According to the literature, calcinosis is common in women, around five years old, with three years of disease in association with osteoarticular, cutaneous, pulmonary manifestations, and fever. The pathogenesis is still unknown, but the participation of interleukin 1 and 6, tumor necrosis factor alpha, and innate immunity dysregulation seem to be involved. Common autoantibodies are anti-NXP-2, anti-MDA-5, and anti-Mi-2, and their treatment remains controversial. Prospective, randomized, controlled studies are needed to evaluate treatment protocols and map the natural history of this serious complication. Calcinosis seems to be more common in White female children with muscle weakness, fever, arthritis, severe pulmonary, and skin involvement with anti-NXP-2, anti-MDA-5, and anti-Mi-2 autoantibodies. The multitargets and aggressive treatment is recommended.
Topics: Child; Adult; Humans; Female; Child, Preschool; Dermatomyositis; Prospective Studies; Autoantibodies; Myositis; Calcinosis
PubMed: 38060107
DOI: 10.1007/s11926-023-01126-5 -
Pharmacological Research Sep 2023To evaluate efficacy and safety of total glucosides of paeony in the treatment of 5 types of inflammatory arthritis METHODS: Databases such as Pubmed, Cochran Library,... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
To evaluate efficacy and safety of total glucosides of paeony in the treatment of 5 types of inflammatory arthritis METHODS: Databases such as Pubmed, Cochran Library, Embase were searched to collect RCTs about TGP in the treatment of inflammatory arthritis. Then, the RCTs were assessed for risk of bias and RCT data were extracted. Finally, RevMan 5.4 was used for the meta-analysis.
RESULTS
A total of 63 RCTs were finally included, involving 5293 participants and 5 types of types of inflammatory arthritis: rheumatoid arthritis (RA), ankylosing spondylitis (AS), osteoarthritis (OA), juvenile idiopathic arthritis (JIA), psoriatic arthritis. For AS, TGP may improve AS disease activity score (ASDAS), decrease erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), tumor necrosis factor (TNF)- α and interleukin (IL)- 6; for RA, TGP may improve disease activity of 28 joints (DAS28), decrease ESR, CRP, rheumatoid factor (RF), TNF-α and IL-6; for psoriatic arthritis, TGP may improve psoriasis area and severity index (PASI) and decrease ESR; for OA, TGP may improve visual analogue scale (VAS) and decrease nitric oxide (NO); for JIA, TGP may increase total efficiency rate, decrease ESR, CRP and TNF-α. For safety, RCTs showed that the addition of TGP did not increase adverse events, and may even reduce adverse events.
CONCLUSION
TGP may improve symptoms and inflammation levels in patients with inflammatory arthritis. However, due to the low quality and small number of RCTs, large-sample, multi-center clinical trials are still needed for revision or validation.
Topics: Humans; Glucosides; Tumor Necrosis Factor-alpha; Paeonia; Arthritis, Psoriatic; Arthritis, Rheumatoid
PubMed: 37402434
DOI: 10.1016/j.phrs.2023.106842 -
The Cochrane Database of Systematic... Feb 2024Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood. Methotrexate has broad immunomodulatory properties and is the most commonly used... (Review)
Review
BACKGROUND
Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood. Methotrexate has broad immunomodulatory properties and is the most commonly used disease-modifying antirheumatic drug (DMARD). This is an update of a 2001 Cochrane review. It supports a living guideline for children and young people with JIA.
OBJECTIVES
To assess the benefits and harms of methotrexate for children and young people with juvenile idiopathic arthritis.
SEARCH METHODS
The Australian JIA Living Guideline Working Group created a registry of all randomised controlled trials (RCTs) of JIA by searching CENTRAL, MEDLINE, Embase, and trials registries. The date of the most recent search of online databases was 1 February 2023.
SELECTION CRITERIA
We searched for RCTs that compared methotrexate with placebo, no treatment, or another DMARD (with or without concomitant therapies) in children and young people (aged up to 18 years) with JIA.
DATA COLLECTION AND ANALYSIS
We used standard Cochrane methods. The main comparison was methotrexate versus placebo. Our outcomes were treatment response, sustained clinically inactive disease, function, pain, participant global assessment of well-being, serious adverse events, and withdrawals due to adverse events. We used GRADE to assess the certainty of evidence for each outcome.
MAIN RESULTS
We identified three new trials in this update, bringing the total number of included RCTs to five (575 participants). Three trials evaluated oral methotrexate versus placebo, one evaluated methotrexate plus intra-articular glucocorticoid (IAGC) therapy versus IAGC therapy alone, and one evaluated methotrexate versus leflunomide. Doses of methotrexate ranged from 5 mg/m/week to 15 mg/m/week in four trials, and participants in the methotrexate group of the remaining trial received 0.5 mg/kg/week. Trial size varied from 31 to 226 participants. The average age of participants ranged from four to 10 years. Most participants were females and most had nonsystemic JIA. The study that evaluated methotrexate plus IAGC therapy versus IAGC therapy alone recruited children and young people with the oligoarticular disease subtype of JIA. Two placebo-controlled trials and the trial of methotrexate versus leflunomide were adequately randomised and blinded, and likely not susceptible to important biases. One placebo-controlled trial may have been susceptible to selection bias due to lack of adequate reporting of randomisation methods. The trial investigating the addition of methotrexate to IAGC therapy was susceptible to performance and detection biases. Methotrexate versus placebo Methotrexate compared with placebo may increase the number of children and young people who achieve treatment response up to six months (absolute difference of 163 more per 1000 people; risk ratio (RR) 1.67, 95% confidence interval (CI) 1.21 to 2.31; I = 0%; 3 trials, 328 participants; low-certainty evidence). However, methotrexate compared with placebo may have little or no effect on pain as measured on an increasing scale of 0 to 100 (mean difference (MD) -1.10 points, 95% CI -9.09 to 6.88; 1 trial, 114 participants), improvement in participant global assessment of well-being (absolute difference of 92 more per 1000 people; RR 1.23, 95% CI 0.88 to 1.72; 1 trial, 176 participants), occurrence of serious adverse events (absolute difference of 5 fewer per 1000 people; RR 0.63, 95% CI 0.04 to 8.97; 3 trials, 328 participants), and withdrawals due to adverse events (RR 3.46, 95% CI 0.60 to 19.79; 3 trials, 328 participants) up to six months. We could not estimate the absolute difference for withdrawals due to adverse events because there were no withdrawals in the placebo group. All outcomes were reported within six months of randomisation. We downgraded the certainty of the evidence to low for all outcomes due to indirectness (suboptimal dosing of methotrexate and diverse outcome measures) and imprecision (few participants and low event rates). No trials reported function or the number of participants with sustained clinically inactive disease. Serious adverse events included liver derangement, abdominal pain, and inadvertent overdose. Methotrexate plus intra-articular corticosteroid therapy versus intra-articular corticosteroid therapy alone Methotrexate plus IAGC therapy compared with IAGC therapy alone may have little or no effect on the probability of sustained clinically inactive disease or the rate of withdrawals due to adverse events up to 12 months in children and young people with the oligoarticular subtype of JIA (low-certainty evidence). We could not calculate the absolute difference in withdrawals due to adverse events because there were no withdrawals in the control group. We are uncertain if there is any difference between the interventions in the risk of severe adverse events, because none were reported. The study did not report treatment response, function, pain, or participant global assessment of well-being. Methotrexate versus an alternative disease-modifying antirheumatic drug Methotrexate compared with leflunomide may have little or no effect on the probability of treatment response or on function, participant global assessment of well-being, risk of serious adverse events, and rate of withdrawals due to adverse events up to four months. We downgraded the certainty of the evidence for all outcomes to low due to imprecision. The study did not report pain or sustained clinically inactive disease.
AUTHORS' CONCLUSIONS
Oral methotrexate (5 mg/m/week to 15 mg/m/week) compared with placebo may increase the number of children and young people achieving treatment response but may have little or no effect on pain or participant global assessment of well-being. Oral methotrexate plus IAGC injections compared to IAGC injections alone may have little or no effect on the likelihood of sustained clinically inactive disease among children and young people with oligoarticular JIA. Similarly, methotrexate compared with leflunomide may have little or no effect on treatment response, function, and participant global assessment of well-being. Serious adverse events due to methotrexate appear to be rare. We will update this review as new evidence becomes available to inform the living guideline.
Topics: Child; Female; Humans; Adolescent; Aged; Child, Preschool; Male; Methotrexate; Arthritis, Juvenile; Leflunomide; Australia; Antirheumatic Agents; Glucocorticoids; Pain
PubMed: 38334147
DOI: 10.1002/14651858.CD003129.pub2 -
Archives of Dermatological Research Oct 2023Atopic dermatitis (AD) is a highly pruritic, inflammatory skin disease with a strong immune component. Rheumatoid arthritis (RA) is a systemic autoimmune disease that... (Meta-Analysis)
Meta-Analysis Review
Atopic dermatitis (AD) is a highly pruritic, inflammatory skin disease with a strong immune component. Rheumatoid arthritis (RA) is a systemic autoimmune disease that causes synovitis and destruction of small joints. Researchers have attempted to quantify an association between both diseases with mixed conclusions. This systematic review and meta-analysis will study the association between AD and RA. Additionally, we conducted a systematic review between AD and other arthritic conditions including osteoarthritis (OA), psoriatic arthritis (PsA), and juvenile idiopathic arthritis (JIA). Medline, Web of Science, Cochrane, and EMBASE databases were searched for relevant studies from inception to March 2021. Observational studies examining relationships between AD and arthritic conditions were selected. 2539 studies were screened; nine were found suitable for quantitative analysis, all of which examined AD and RA. All studies had low risk of bias as determined by the Newcastle-Ottawa Scale. Patients with RA did not have significantly increased odds of comorbid AD. These findings were consistent across multiple study designs. However, patients with AD had significantly increased odds of comorbid RA. There were not enough studies identified to perform quantitative analysis between AD and other arthritic conditions. Two studies, one on JIA and one PsA, found no association with AD. Two studies on AD and OA had conflicting results. The present study provides definitive evidence of increased odds of comorbid RA in AD patients. There were no such increased odds of comorbid AD in RA patients. No such association was found between AD and PsA, OA or JIA.
Topics: Humans; Dermatitis, Atopic; Arthritis, Psoriatic; Arthritis, Rheumatoid; Risk; Databases, Factual
PubMed: 37043009
DOI: 10.1007/s00403-023-02619-0 -
European Journal of Orthopaedic Surgery... Oct 2023The prevalence of juvenile idiopathic arthritis (JIA) is estimated to be 16-150 per 100,000 children worldwide. The hip joint may be involved in over 50% of children... (Review)
Review
PURPOSE
The prevalence of juvenile idiopathic arthritis (JIA) is estimated to be 16-150 per 100,000 children worldwide. The hip joint may be involved in over 50% of children leading to significant morbidity which may require surgical intervention in the form of arthroplasty. The literature lacks a concise overview of the outcomes, including complication and implant survival of total hip arthroplasty (THA) in juvenile idiopathic arthritis (JIA). The aim of this study is to systematically analyze the literature and report the outcomes of THA in JIA.
METHODS
Search was conducted in the online databases PubMed, Embase and Cochrane database. It included all original studies which evaluated clinical and/or radiological outcomes of THA in JIA with a minimum sample size of 5 patients and published in English. The level of evidence of the included studies was graded according to the Oxford Centre for Evidence Based Medicine. The Institute of Health Economics checklist was used to assess the quality of the studies included.
RESULTS
The nine studies included were retrospective in nature with all being Level IV according to Oxford Centre for Evidence Based Medicine. 475 hips in 304 patients with majority of them being females (241/304, 79.2%) were included in this review. All the studies reported the outcome objectively using various scores. The proportion of revision surgeries (92/378), either femoral or acetabular, noted was 22% (95% CI 10-33%). The proportion of acetabular revisions (72/378) was 16% (95% CI 8-25%) as compared to 4% (95% CI 1-6%) for femoral revisions (20/378). There was no difference in survivorship when cemented and uncemented implants were compared.
CONCLUSION
JIA patients with advanced hip disease represent a unique population with need for extra-long implant longevity. THA in patients of JIA leads to improved pain relief as well as mobility but the conversion of the same outcomes to functional activity is not proportionally improved. The current trend is the use of uncemented and ceramic-on-ceramic implants. Acetabular implants require earlier revision as compared to femoral implants. Age at surgery can be delayed by early institution of methotrexate which indirectly improves implant survival.
LEVEL OF EVIDENCE
IV.
Topics: Child; Female; Humans; Male; Arthroplasty, Replacement, Hip; Arthritis, Juvenile; Hip Prosthesis; Retrospective Studies; Prosthesis Failure; Prosthesis Design; Reoperation; Treatment Outcome
PubMed: 36947313
DOI: 10.1007/s00590-023-03525-x