7 research outputs found
Ultrasound Features across Subtypes of Juvenile Idiopathic Arthritis
No full textObjective: The aim of this study was to evaluate musculoskeletal ultrasound (MSUS) features across categories of juvenile idiopathic arthritis (JIA). Methods: In this cross-sectional study, all patients were subjected to full history taking, clinical examination including disease assessment parameters and laboratory investigations. In addition, all children were examined by both grayscale (GS) and power Doppler (PD) MSUS images. Results: By MSUS, the number of joints with synovial effusion was 697 of a total 2400 examined joints (29%) and joints with synovial thickening counted 673 (28%). The number of joints with positive PD signals was 446 (18.6%). There was a significant difference among JIA subtypes as regards different MSUS features. Moreover, there was a discrepancy regarding synovial effusion (p = 0.018), hypertrophy scores (p = 0.013), and the total US severity score (p = 0.026). This divergence was attributed to the significant difference between systemic juvenile idiopathic arthritis (SJIA) and other categories. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of MSUS in JIA and its subtypes were calculated. Conclusion: MSUS is a highly sensitive method for detecting synovitis, tenosynovitis, and erosive bone disease, and it helps to make proper therapeutic decisions. There was a significant difference among JIA subtypes regarding MSUS features
Long noncoding RNAs (CTC-471J1.2, NeST) as epigenetic risk factors of active juvenile lupus nephritis: a case-control study
No full textAbstract Background Measurement of the circulating levels of long-non-coding RNAs (lncRNAs) in lupus nephritis (LN) patients could dramatically explore more insights about the disease pathogenesis. Hence, we aimed to quantify the level of expression of CTC-471J1.2 and NeST in LN patients and to correlate it with the disease activity. Method This case-control study was conducted on a group of children with juvenile LN attending to Mansoura University Children’s Hospital (MUCH). Demographics, clinical, and laboratory findings were collected besides the measurement of lncRNAs by quantitative real-time PCR. Results The expression level of lncRNAs-CTC-471J1.2 was significantly down-regulated in children with active LN versus inactive cases or controls. In contrast, the NeST was significantly up-regulated in active LN cases. A significant correlation was found between CTC-471J1.2 expression and LN activity parameters. Additionally, both lncRNAs showed a reasonable sensitivity and specificity in differentiation of active LN. A regression analysis model revealed that CTC-471J1.2 and NeST were independent predictors of active nephritis. Conclusion The expression level of circulatory lncRNAs-CTC-471J1.2 and NeST can be used as sensitive and specific biomarkers for active LN. Furthermore, both could serve as predictors for nephritis activity
Development of the paediatric society of the African league against rheumatism (PAFLAR) JIA registry and clinical profile of JIA in Africa from the PAFLAR JIA registry
Get PDFAbstract Background The spectrum of Juvenile Idiopathic Arthritis (JIA) in Africa is still largely unknown. We thus set out to illustrate how we set up the PAFLAR JIA registry and describe the clinical profile of Juvenile Idiopathic Arthritis across various regions in Africa. Methods We carried out a retrospective observational cohort study where collaborators were trained on use of the existing PAFLAR REDCAP database to enter data for the JIA patients currently under their care capturing their epidemiological data, clinical features, laboratory investigations, diagnosis and therapy at initial diagnosis. Descriptive statistics including means, standard deviations, medians, interquartile ranges (IQR) for continuous variables and proportions for categorical variables were calculated as appropriate. Tests for difference between groups were performed between categorical variables using Pearson’s chi-square or Fisher’s exact tests. All analyses were performed using SPSS version 22 software. Results We enrolled 302 patients, 58.6% (177 of 302) of whom were female. The median age of disease onset was 7 years (range 3–11 years) and the median age at diagnosis was 8.5 years (range 5–12 years). The median duration delay in diagnosis was 6 months (range 1-20.8 months). The JIA categories included Systemic JIA 18.9% (57), Oligoarticular JIA 19.2% (83), Polyarticular RF + ve 5% (15), Polyarticular RF-ve 17.9% (54), Enthesitis Related Arthritis (ERA) 18.2% (55), Psoriatic Arthritis 7% (21) and undifferentiated JIA 5.6% (17). As regards treatment the commonest therapies were NSAID therapy at 31.1%, synthetic DMARDs at 18.1%, synthetic DMARDs combined with NSAIDs at 17.5% and steroid therapy at 9.6%. Biological DMARDs accounted for 2.3% of therapies offered to our patients at diagnosis. The average JADAS score was 10.3 (range 4.8–18.2) and the average CHAQ score was 1.3 (range 0.7-2.0). Conclusion Our study highlights strategies involved in setting up a Pan-African paediatric rheumatology registry that embraces our broad diversity and the vast spectrum of JIA in Africa while comparing the various therapies available to our patients. The PAFLAR JIA registry strives to ensure a comprehensive representation of the diverse healthcare landscapes within the continent. Further longitudinal observation studies are required to ascertain the long-term outcomes of our patients and ultimately help inform policy to create a more favorable health ecosystem to support the healthcare needs of JIA patients in Africa
Consensus evidence-based recommendations for transition of care for adolescents with juvenile idiopathic arthritis: meeting patients’, parents’, and rheumatologists’ perspectives
No full textAbstract Background Transition of care means the process of educating and empowering adolescents and young adults to take an active role in their own healthcare, develop decision-making skills, and eventually transition from paediatric to adult healthcare providers. Most people do not switch doctors until they are young adults, but it can be beneficial to start preparing children earlier. We aimed to develop a specific toolkit tailored to paediatric and adult rheumatologists to assist them in transitioning of care of young people with juvenile onset rheumatic musculoskeletal diseases from the paediatric to adult rheumatology care. Results The expert panel was confined to an online survey (n = 18), all the experts completed the two rounds. At the conclusion of round 2, a total of 10 points were gathered. The range of respondents (ranks 7–9) who agreed with the recommendations was 88.9 to 100%. All 10 clinical standards identified by the scientific committee were written in the same way. Based on the answers to the structured key questions and the literature review, a structured template was developed presenting transition of care integrated pathway. Conclusion The developed rheumatology-specific guideline offers adolescents and young adults a focussed, multidisciplinary transition of care approach with equity of access, quality of care and flexibility and set up standards for transitional care for young adults with juvenile rheumatological diseases
Serum Amyloid A Level in Patients with Juvenile Idiopathic Arthritis
No full textBackground and Aim of the Work: JIA is the commonest rheumatic disease in childhood characterized by inflammatory arthritis lasting more than 6 weeks before the 16th birthday. In addition to routine ESR and CRP, there are other inflammatory biomarkers as SAA. It is one of the major acute phase reactants which was found to be elevated in inflammatory arthritis and a good indicator of disease activity.This study assessed the value of SAA level in a cohort of patients with JIA.Subjects and Methods: 45 JIA patients and 40 healthy controls were recruited from the outpatient clinic of Rheumatology and Rehabilitation Department at MUCH. All patients underwent a thorough clinical evaluation. Assessment of JIA patients involved assessment of disease activity, tenderness and functional status. Laboratory tests were done including: CBC, ESR, CRP and SAA.Results: A significant rise in SAA levels was found in JIA patients compared to control group and it was significantly higher in SJIA subtype. SAA level was positively correlated with JADAS-27, VAS, physician global assessment, C-HAQ, Ritchie articular index score, platelet count and ESR in 1st hour and 2nd hour. The levels of SAA were significantly lower in JIA patients taking methotrexate while it was significantly higher in cyclosporine treated patients.Conclusion: SAA can be used as additional indicator of JIA disease activity. Moreover, it can help in differentiation between subtypes when combined with clinical features of the disease and it may be considered in assessment of patient, s response to therapy
Feasibility of Conducting Comparative Effectiveness Research and Validation of a Clinical Disease Activity Score for Chronic Nonbacterial Osteomyelitis
No full textObjective. Prospective comparative effectiveness research (CER) in chronic nonbacterial osteomyelitis (CNO) is lacking. Our objectives were to (1) determine the use and safety of each consensus treatment plan (CTP) regimen for CNO, (2) assess the feasibility of using the Chronic Nonbacterial Osteomyelitis International Registry (CHOIR) data for CER, and (3) develop and validate a CNO clinical disease activity score (CDAS) using CHOIR. Methods. Consenting children or young adults with CNO were enrolled into CHOIR. Demographic, clinical, and imaging data were prospectively collected. The CNO CDAS was developed through a Delphi survey and nominal group technique. External validation surveys were administered to CHOIR participants. Results. One hundred forty (78.2%) CHOIR participants enrolled between August 2018 and September 2020 received at least 1 CTP regimen. Baseline characteristics from different CTP groups were well matched. Patient pain, patient global assessment, and clinical CNO lesion count were key variables included in the CNO CDAS. The CDAS showed a strong correlation with patient/parent report of difficulty using a limb, back, or jaw and patient/parent report of disease severity, but a weak correlation with patient/parent report of fatigue, sadness, and worry. The change in CDAS was significant in patients reporting disease worsening or improvement (P < 0.001). The CDAS significantly decreased after initiating second-line treatments from median 12.0 (IQR 8.0-15.5) to 5.0 (IQR 3.0-12.0; P = 0.002). Although second-line treatments were well tolerated, psoriasis was the most common adverse event. Conclusion. The CNO CDAS was developed and validated for disease monitoring and assessment of treatment effectiveness. CHOIR provided a comprehensive framework for future CER
EULAR/American College of Rheumatology Classification Criteria for Pediatric Chronic Nonbacterial Osteomyelitis
No full textObjectiveTo develop and validate classification criteria for pediatric chronic nonbacterial osteomyelitis (CNO) jointly supported by EULAR and the American College of Rheumatology (ACR).MethodsThis international initiative had 4 phases: (1) candidate items were proposed in a survey of pediatric rheumatologists, (2) criteria definition and reduction by Delphi and nominal group technique exercises, (3) criteria weighting using multicriteria decision analysis, and (4) refinement of weights and threshold score in a development cohort of 441 patients and validation in another cohort of 514 patients.ResultsThe new EULAR/ACR classification criteria for CNO require typical radiographic or magnetic resonance imaging findings and bone pain as an obligatory entry criterion and exclusion criteria of malignancy, infection, vitamin C deficiency, and hypophosphatasia, followed by additive weighted criteria in 5 clinical (site of bone lesions, pattern of bone lesions, age at onset, coexisting conditions, fever) and 4 pathology/laboratory domains (bone biopsy findings if done, anemia, C-reactive protein level, and erythrocyte sedimentation rate). A total score >= 55 is required for classification as CNO. The new criteria had a sensitivity of 82% and specificity of 98% in the validation cohort.ConclusionThese new classification criteria for pediatric CNO developed with international input reflect current views about CNO, have high specificity and good sensitivity, and provide a key foundation for future CNO research
