Textbook of Pediatric Rheumatology, 7e

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PRES , submitted. Objectives: To evaluate the performance of the final set of classification criteria, in discriminating patients with the different HRF and PFAPA in a separate set of real patients coming from the Eurofever Registry and to compare their accuracy with respect to existing criteria. Methods: We selected those patients with recurrent HPF coming from the Eurofever Registry excluding patients belonging to the original dataset of patients used for the development of the criteria themselves.

Sensitivity, specificity, accuracy, negative and positive predictive values and AUC-ROC of the new criteria were calculated. Results: A total of new patients coming from the Eurofever Registry were included. The performance of the criteria coming from the Consensus conference in comparison with the criteria of the literature is listed in Table 1. Overall, their performance was superior accuracy ranging from 0. Most of the patients not classified with the new criteria were negative for genetic analysis or carriers of low-penetrance mutations with an inconsistent clinical phenotype.

Conclusion: The validation of the new Eurofever classification criteria in a large group of unselected patients coming from the registry confirms their high specificity and overall better performance in comparison to other criteria available in the literature.

It is recommended to use them as classification rather that diagnostic criteria, for clinical trials and pathogenic studies. Missing items beyond last evaluable measurement were imputed by last observation carried forward LOCF. AIDAI is a validated patient-reported tool to assess disease activity and appears to have good sensitivity to change to be used in comparative trials.

Ann Rheum Dis. N Engl J Med. Rosner: None Declared, A. Tomassini: None Declared, S. Murias: None Declared, O. Karadag: None Declared, J. Levy Consultant for: Novartis, S. Smeets Employee of: Novartis, F. Introduction: CNO is an inflammatory bone disease that can result in bone destruction, persistent bone pain and pathological fractures. For clinical and research purposes, serial MRI exams are needed to determine the objective response to treatment. A previously developed MRI scoring tool for CNO in a single center showed sensitivity to detect imaging change after aggressive treatment.

Objectives: To use a nominal group technique to develop a practical and consensus-based MRI scoring tool for clinical and research use in CNO. Methods: Eleven pediatric radiologists, each with at least 5 years of experience reading musculoskeletal MRI from seven different pediatric hospitals in North America and Europe, discussed definitions and grading of signal intensity, extent of signal abnormality within bone marrow and surrounding tissue, physis damage and vertebral fracture on MRI through monthly conference calls and an in-person meeting Seattle, July Inter-rater agreement of presence of abnormal signal and severity were assessed using Fleiss kappa analysis.

Extent of abnormal signal intensity within bone or surrounding tissues were scored according to the relative affected proportion. Long bones were divided into proximal epiphysis, proximal metaphysis, diaphysis, distal metaphysis and distal epiphysis. Complex bony regions such as the pelvis were divided into easily identifiable anatomical subareas. The agreement among 11 radiologists in readings of femur and tibia were shown in Table 1.

There was moderate to substantial agreement 0. There were 38 tibias and 30 femurs identified as active inflammation by at least 9 of 11 radiologists.


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Data from other bones are being analyzed. There was moderate to substantial agreements among radiologists in majority of parameters of femur and tibia. If proven reproducible, this tool can be validated in a prospective study and will become a key element of disease activity assessment in CNO. Sato: None Declared, M. Beer: None Declared, M. Huang: None Declared, R. Iyer: None Declared, M. McGuire: None Declared, A. Ngo: None Declared, J. Otjen: None Declared, J. Panwar: None Declared, J.

Stimec: None Declared, M. Thapa: None Declared, P. Toma: None Declared, A. Taneja: None Declared, N. Gove: None Declared, P. Caregiver is a cornerstone to achieve disease control however, there are no information on how this conditions affects them; neither a tool to assess this impact was reported.

Phase 1. Phase 2. After pilot study, six items were confuse and modified, 11 were eliminated for being redundant. Items were organized in five domains to develop 3rdQ: disease impact social, economic, family and relationship , knowledge, future, alternative medicine, and religion 13, 27, 2, 2, and 2 items, respectively. Mean age 39 SD 8. Statistical analysis show adequate inter-item reliability in all domains except for religion, consequently items were adapted to create new dimensions Table 1. Correlation matrix shown good results in all domains, except for social and economic due to redundant items.

Further efforts will be performed to validated in other PRD. Historically, the prognosis of sJIA was very poor.

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It is hypothesized that innate immune activation is most prominent in the early phase of sJIA. AR Objectives: To analyse the long-term efficacy of this therapeutic strategy and to separately analyse a subset of sJIA patients without arthritis at disease onset. Inactive disease ID was defined according to the Wallace criteria. Cytokine profiling was performed using Luminex multiplex immunoassay.

Results: Forty-two patients were analysed, with a median follow-up of 5. Twelve patients had no arthritis at onset; 4 of them developed arthritis during flares. The clinical phenotype, inflammatory parameters and cytokine profile of patients without arthritis at onset was similar to patients with arthritis. Hence, after extensive ancillary investigations to exclude other diseases, these non-arthritic patients also received rIL-1RA as first-line therapy. One patient died due to MAS. Patients who achieved ID at one year were younger and had a significantly shorter disease duration, less active joints and higher neutrophil and leukocyte count before start of rIL-1RA.

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Multivariate analysis confirmed high neutrophil count as a significant predictor for ID at one year. Furthermore, ID at one year was highly associated with a good response at 1 month.

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Conclusion: First-line, short course monotherapy with rIL-1RA is a highly efficacious strategy to induce and sustain inactive disease and to prevent disease- and glucocorticoid-related damage. Our data on sJIA patients without arthritis plea for leaving out arthritis as a prerequisite criterion in future disease classification criteria.

The largest study demonstrated a similar renal response rate with mycophenolate mofetil MMF or cyclophosphamide CYC induction treatment in adult patients with LN [1]. In both, MMF had a better safety profile [1,2]. Median values and interquartile ranges quoted.

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Mann-Whitney U tests for continuous data and Fishers exact test for categorical data. Conclusion: This is the largest study to date investigating induction treatments for proliferative LN in children. Remission may be reached quicker in patients treated with CYC. Introduction: Long-standing debate continues on the factors and potential etiological agents of vasculitides and rheumatic diseases, such as Kawasaki disease KD.

Joseph M. Sanzari Children's Hospital

Together with the genetic predisposition to suffer the condition, hitherto undefined environmental factors have been suggested to contribute to the onset of autoimmune vasculitis in susceptible individuals. Objectives: The hypothesis of a windborne role in the generation of KD etiology is further tested in this study for a series of other world locations where the disease is also prevalent and along the suspicion that the interaction between bioaerosols, the air chemistry and predisposed or genetically susceptible patients might similarly be associated with KD incidence there.

Pediatric Rheumatology Disorders

Exploration of the phenological status of vegetation in each of those region is performed to seek further mechanistic explanations on the epidemiological links to KD. Methods: In this study, a retrospective time series analysis of historical daily hospital admissions of KD and reconstruction of air movements and source regions is presented for several locations in all continents US and Canada, Chile, France, the United Kingdom, Catalonia, South Korea, India and Thailand.

The FLEXPART v9 atmospheric particle dispersion model is used and land-surface cover and high-resolution vegetation data employed to explore relationships to stages of vegetation development. Results: Source regions and their land-cover typology is reconstructed for all major KD world sites in terms of disease incidence. Similar strong relationships emerge to cereal croplands in all the different continents. The phenological cycle of vegetation in those areas shows a link to the stages where vegetation is decaying, indicating that a relationship between the major KD season and some kind of agricultural byproducts or remnants might play a role in the onset or exacerbation of this disease.

An application of a statistical model integrating the air physics ssociated to the movement of winds from these sources further demonstrates predictive capacity to anticipate peaks in KD incidence. Conclusion: Long-standing debate continues on the factors and potential etiological agents of vasculitides and rheumatic diseases, such as Kawasaki disease KD in susceptible children.

Together with the genetic predisposition to suffer the condition, hitherto undefined environmental factors are now suggested to contribute to the onset of autoimmune vasculitis in susceptible individuals. The role of bioaerosols and air chemistry and physics is presented pointing to a much clearer combination of factors ultimately appearing to be crucial to the manifestation of epidemiological clusters of this pediatric vasculitis.

A computational model integrating all this new environmental information in combination with epidemiological dynamics proves successful in predicting KD incidence in Japan. Introduction: Juvenile dermatomyositis JDM is a rare, severe autoimmune disease characterised by proximal muscle weakness and skin rash. While JDM and adult-onset dermatomyositis DM share similar clinical and biological features, there are differences in prevalence of these features, including cancer, calcinosis and myositis-specific autoantibodies, suggesting a possible influence of age on pathogenesis.

Genotyping of the replication cohort is ongoing using the HumanCoreExome array. In addition, we now have a replication cohort via our international consortium to validate these findings. Conclusion: This study confirms previous findings regarding HLA involvement. We previously reported that expression of both type I and type II IFN related genes is increased in muscle biopsies of JDM patients and correlates with histological and clinical features of the disease. Interferon regulated genes IRGs have also been reported to be upregulated in peripheral blood of JDM patients and could represent valuable biomarkers of disease activity.


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  • Methods: We collected blood samples from 28 JDM patients at different time points during follow-up. Including time from disease onset to sampling did not change the results.

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