The Libyan Arabic version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR)

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Libyan Arabic language. The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographic, clinical data, and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the 3 Likert assumptions, floor/ceiling effects, internal consistency, Cronbach\u2019s alpha, interscale correlations, test\u2013retest reliability, and construct validity (convergent and discriminant validity). A total of 100 JIA patients (22.0% systemic, 26.0% oligoarticular, 25.0% RF negative polyarthritis, and 27.0% other categories) and 100 healthy children, were enrolled in a paediatric rheumatology centre. The JAMAR components discriminated well healthy subjects from JIA patients. Notably, there is no significant difference between the healthy subjects and their affected peers in the school-related problems variable. All JAMAR components revealed satisfactory psychometric performances. In conclusion, the Libyan Arabic version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research

Performance of current guidelines for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

Publisher Copyright: Copyright © 2014 by the American College of Rheumatology.Results The study sample included 362 patients with systemic JIA and MAS, 404 patients with active systemic JIA without MAS, and 345 patients with systemic infection. The best capacity to differentiate MAS from systemic JIA without MAS was found when the preliminary MAS guidelines were applied. The 3/5-adapted HLH-2004 guidelines performed better than the 4/5-adapted guidelines in distinguishing MAS from active systemic JIA without MAS. The 3/5-adapted HLH-2004 guidelines and the preliminary MAS guidelines with the addition of ferritin levels ≥500 ng/ml discriminated best between MAS and systemic infections. Conclusion The preliminary MAS guidelines showed the strongest ability to identify MAS in systemic JIA. The addition of hyperferritinemia enhanced their capacity to differentiate MAS from systemic infections. The HLH-2004 guidelines are likely not appropriate for identification of MAS in children with systemic JIA. Objective To compare the capacity of the 2004 diagnostic guidelines for hemophagocytic lymphohistiocytosis (HLH-2004) with the capacity of the preliminary diagnostic guidelines for systemic juvenile idiopathic arthritis (JIA)-associated macrophage activation syndrome (MAS) to discriminate MAS complicating systemic JIA from 2 potentially confusable conditions, represented by active systemic JIA without MAS and systemic infection. Methods International pediatric rheumatologists and hemato-oncologists were asked to retrospectively collect clinical information from patients with systemic JIA-associated MAS and confusable conditions. The ability of the guidelines to differentiate MAS from the control diseases was evaluated by calculating the sensitivity and specificity of each set of guidelines and the kappa statistics for concordance with the physician's diagnosis. Owing to the fact that not all patients were assessed for hemophagocytosis on bone marrow aspirates and given the lack of data on natural killer cell activity and soluble CD25 levels, the HLH-2004 guidelines were adapted to enable the diagnosis of MAS when 3 of 5 of the remaining items (3/5-adapted) or 4 of 5 of the remaining items (4/5-adapted) were present.publishersversionPeer reviewe

Predictors of uveitic macular edema and functional prognostic outcomes: real-life data from the international AIDA Network uveitis registry

Objectives: To detect factors capable of predicting the development of macular edema (ME) throughout the disease course in patients affected by non-infectious uveitis (NIU). Methods: Predictive factors leading to the development of ME were analyzed through regression analysis. The functional impact of ME on best corrected visual acuity (BCVA) was also examined. Results: A total of 1,160 NIU patients (1,857 eyes) were analyzed. ME was observed in 148 (12.76%), affecting 211 eyes. It was significantly more frequent in patients with non-anterior NIU (p < 0.0001, RR = 4.01), retinal vasculitis (p < 0.0001), and other structural complications (p = 0.0005). Gender, HLA-B*27 and/or HLA-B*51 positivity, and ethnicity did not show any significant impact on the prevalence of ME (p = 0.635, p = 0.372, p = 0.193, respectively). Four variables were associated with ME development during NIU course: the non-anterior anatomical pattern (p < 0.0001, OR = 4.01), the presence of retinal vasculitis (p = 0.028, OR = 1.68), complications other than ME (p = 0.044, OR = 1.51) and immunosuppressive treatment (p = 0.010, OR 1.69) while the diagnosis of Behçet disease-related uveitis was less likely to be associated with ME development (p = 0.24, OR 0.545). Mean ± SD BCVA was significantly lower in eyes with ME (0.82 ± 0.30) compared to eyes without ME (0.71 ± 0.33). Conclusion: ME can develop across all NIU types, but is more likely in cases involving the posterior segment and retinal vasculitis. Regular and focused monitoring is recommended for these high-risk patients. The study also highlights the limited predictive value of demographic and HLA-related factors, helping refine clinical risk stratification and predictive modeling in NIU

Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project

Objective: To assess current treatment in macrophage activation syndrome (MAS) worldwide and to highlight any areas of major heterogeneity of practice. Methods: A systematic literature search was performed in both EMBASE and PubMed databases. Paper screening was done by two independent teams based on agreed criteria. Data extraction was standardized following the PICO framework. A panel of experts assessed paper validity, using the Joanna Briggs Institute appraisal tools and category of evidence (CoE) according to EULAR procedure. Results: Fifty-seven papers were finally included (80% retrospective case-series), describing 1148 patients with MAS: 889 systemic juvenile idiopathic arthritis (sJIA), 137 systemic lupus erythematosus (SLE), 69 Kawasaki disease (KD) and 53 other rheumatological conditions. Fourteen and 11 studies specified data on MAS associated to SLE and KD, respectively. All papers mentioned glucocorticoids (GCs), mostly methylprednisolone and prednisolone (90%); dexamethasone was used in 7% of patients. Ciclosporin was reported in a wide range of patients according to different cohorts. Anakinra was used in 179 MAS patients, with a favourable outcome in 83% of sJIA-MAS. Etoposide was described by 11 studies, mainly as part of HLH-94/04 protocol. Emapalumab was the only medication tested in a clinical trial in 14 sJIA-MAS, with 93% of MAS remission. Ruxolitinib was the most reported Janus kinase inhibitor in MAS. Conclusion: High-dose GCs together with IL-1 and IFN gamma inhibitors have shown efficacy in MAS, especially in sJIA-associated MAS. However, the global level of evidence on MAS treatment, especially in other conditions, is still poor and requires standardized studies to be confirme

American College of Rheumatology Provisional Criteria for Clinically Relevant Improvement in Children and Adolescents With Childhood-Onset Systemic Lupus Erythematosus

10.1002/acr.23834ARTHRITIS CARE & RESEARCH715579-59

P042 Profile of systemic lupus erythematous patients at presentation 10 years of Single center experience-Tripoli-Libya

Abstract Background Juvenile Systemic Lupus Erythematous (JSLE) is a systemic autoimmune disorder with speckled manifestations that can emerge overstretched period of time and can affect any organ system, most frequently the skin, joints, kidneys, and the nervous, hematologic, and cardiovascular systems. The Aim is to examine the clinical features, serologic and laboratory characteristics associated with SLE. To probe and outline Clinical and Immunologic features of Incomplete Lupus Erythematous (ILE) patients who progressed from ILE to SLE. Methods The files of patients diagnosed as SLE in pediatric rheumatology clinic from 2001 to 5/2021 were retrospectively reviewed. Result Thirty SLE cases were included; Females were more prevalent with a female: male ratio of 14:1. Mean age at presentation 11 ± 4 years (range of 5 months-13 years), Disease onset was before sixth birthday in (7%), above twelve years in (40%) of the patients, and 53% of the patients was among 6 –12 years age group (31% &amp; 37% of them fulfilled the SLICC &amp; ACR criteria respectively at diagnosis) .The Mean duration between the onset of symptoms and SLE diagnosis was 6 months ±2 years. The Pre-pubertal age group presented early. At diagnosis, 50% of the patient got SLICC score criteria &amp;lt;4, on other hand 70% of the patients had ACR score criteria less than four Variable The most common presenting feature was arthritis (83%) (polyarticular arthritis) followed by dermatological manifestations (46%) photosensitivity, malar rash, and discoid rash in order of most frequent, fatigability (37%), renal manifestation (23%) most commonly as hematuria, one case diagnosed as lupus nephritis by renal biopsy. ANA was positive in 87%, anti-ds-DNA positive in 40% and Anti sm positive in 17%. The most frequently used medications were steroids and hydroxychlorqiune, the most commonly used steroid sparing medications were azathioprine (43%), Mycophenolate mofetil (40%) and ciclosporin (13%), 26% were on antihypertensive, 3% required rituximab to control their disease and one patient received Eltromboag to treat refractory thrombocytopenia. the mean follow-up duration 46.9 ± 43.6m, 20% lost follow up, 13% died due to disease complications (renal system involvement, thrombocytopenia and neurological system involvements). Conclusion jSLE in Libya is very rare before the sixth birthday and presented early among 6–12 years age group with a delay of less than one year between the first presentation and time of diagnosis. SLICC criteria was sufficient to diagnose the disease in &amp;gt; 50% of patients. High index of suspension should be maintained because in some patient’s years may be passed before fulfilling the diagnostic criteria. The most common cause of death is renal involvement. Glucocorticoids are the backbone of jSLE treatment in the acute phase. Both azathioprine and Mycophenolate mofetil are sufficient to control the disease in most patients. The disease outcome is accepted in our cohort as most patients have mild disease activity with low dose steroid and a steroid-sparing agent. </jats:sec

Tocilizumab effectiveness in paediatric non-infectious uveitis: data from the International AIDA Network Registries on ocular inflammatory disorders

To describe tocilizumab (TCZ) effectiveness in 15 children with refractory non-infectious uveitis. Reported outcomes are the number of relapses before and after treatment, steroid-sparing effect and drug retention rate. Macular oedema, fluorangiographic findings and ocular complications are also reported. The mean number of ocular relapses significantly decreased from 314 per 100 eyes/year to 106 per 100 eyes/year (p=0.016). A significant steroid-sparing effect was detected (p=0.037). TCZ drug survival was 77.4% at 6 months, followed by 61.9% at 12, 24 and 36 months of follow-up. Macular oedema and retinal vasculitis resolved in all affected eyes

Telehealth in Rheumatology: The 2021 Arab League of Rheumatology Best Practice Guidelines

AbstractBackgroundTelehealth use is increasing and will undeniably continue to play a role beyond the COVID-19 era. Best practice guidelines (BPG) for telehealth add credibility, standardize approaches, facilitate reimbursement, and decrease liability.ObjectivesTo develop BPG for the use of Telehealth In Rheumatology in the Arab region, to identify the top barriers and facilitators of telehealth in the Arab region, and to provide rheumatologists with a practical toolkit for the implementation of telehealth.MethodsGuidelines were drafted by a core steering committee from the Arab League of Associations for Rheumatology (ArLAR) after performing a literature search. A multidisciplinary task force (TF), including 18 rheumatologists, 2 patients, and 2 regulators from 15 Arab countries, assessed the BPG using 3 rounds of anonymous online voting by modified Delphi process. The voting on barriers and facilitators was performed through one voting round. The toolkit was developed based on available literature and discussions during the Delphi rounds.ResultsFour General Principles and twelve Statements were formulated. All statements reached &gt;80% consensus. A teleconsultation was specifically defined for the purpose of these guidelines. The concept of choice in telehealth was highlighted, emphasizing patient confidentiality, medical information security, rheumatologist’s clinical judgment, and local jurisdictional regulations. The top barrier for telehealth was the concern about the quality of care. The toolkit emphasized technical aspects of teleconsultation and proposed a triage system.ConclusionsThe ArLAR BPG provides rheumatologists with a series of strategies about the most reliable, productive, and rational approaches to apply telehealth.Article SummaryStrengths and limitations of this studyBest practice guidelines (BPG) the use of Telehealth In Rheumatology in the Arab region were developed herein under the umbrella of the Arab League of Associations for Rheumatology (ArLAR)A teleconsultation was specifically defined for the purpose of these guidelinesThe concept of choice in telehealth was highlighted, emphasizing patient confidentiality, medical information security, rheumatologist’s clinical judgment, and local jurisdictional regulationsThe top barrier for telehealth was the concern about the quality of careThe ArLAR BPG provides rheumatologists with a series of strategies about the most reliable, productive, and rational approaches to apply telehealth in the rheumatology clinic</jats:sec

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

Abstract 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