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1. Determinants of Discordance Between Criteria for Inactive Disease and Low Disease Activity in Juvenile Idiopathic Arthritis

Author

Giancane, Gabriella, Campone, Chiara, Gicchino, Maria Francesca, Alongi, Alessandra, Bava, Cecilia, Rosina, Silvia, Boyko, Yaryna, Martin, Neil, El Miedany, Yasser, Harjacek, Miroslav, Hashad, Soad, Ioseliani, Maka, Burgos‐Vargas, Ruben, Joos, Rik, Scott, Christiaan, Manel, Mejbri, Ayala, Zoilo Morel, Ekelund, Maria, Al‐Abrawi, Safiya, Aiche, Maya‐Feriel, Norambuena, Ximena, Melo‐Gomes, Jose Antonio, Ruperto, Nicolino, Consolaro, Alessandro, and Ravelli, Angelo

Published
2021
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5. Uveitis in Juvenile Idiopathic Arthritis:18-Year Outcome in the Population-based Nordic Cohort Study

Author

Rypdal, Veronika, Glerup, Mia, Songstad, Nils Thomas, Bertelsen, Geir, Christoffersen, Terje, Arnstad, Ellen D, Aalto, Kristiina, Berntson, Lillemor, Fasth, Anders, Herlin, Troels, Ekelund, Maria, Peltoniemi, Suvi, Toftedal, Peter, Nielsen, Susan, Leinonen, Sanna, Bangsgaard, Regitze, Nielsen, Rasmus, Rygg, Marite, Nordal, Ellen, and Lindqvist, S

Subjects
musculoskeletal diseases, long-term outcome, genetic structures, treatment, uveitis cumulative incidence, prospective, eye diseases, population-based, risk factors for ocular complications, juvenile idiopathic arthritis, uveitis, skin and connective tissue diseases, ocular complications, disease activity, SUN criteria
Abstract

PURPOSE: To assess the long-term outcome of uveitis in juvenile idiopathic arthritis (JIA).DESIGN: Population-based, multicenter, prospective JIA cohort, with a cross-sectional assessment of JIA-associated uveitis (JIA-U) 18 years after the onset of JIA.PARTICIPANTS: A total of 434 patients with JIA, of whom 96 had uveitis, from defined geographic areas of Denmark, Finland, Norway, and Sweden.METHODS: Patients with onset of JIA between January 1997 and June 2000 were prospectively followed for 18 years. Pediatric rheumatologists and ophthalmologists collected clinical and laboratory data.MAIN OUTCOME MEASURES: Cumulative incidence of uveitis and clinical characteristics, JIA and uveitis disease activity, ocular complications, visual outcome, and risk factors associated with the development of uveitis-related complications.RESULTS: Uveitis developed in 96 (22.1%) of 434 patients with JIA. In 12 patients (2.8%), uveitis was diagnosed between 8 and 18 years of follow-up. Systemic immunosuppressive medication was more common among patients with uveitis (47/96 [49.0%]) compared with patients without uveitis (78/338 [23.1%]). Active uveitis was present in 19 of 78 patients (24.4%) at the 18-year visit. Ocular complications occurred in 31 of 80 patients (38.8%). Short duration between the onset of JIA and the diagnosis of uveitis was a risk factor for developing ocular complications (odds ratio [OR], 1.4; 95% confidence interval [CI], 1.1-1.8). Patients with a diagnosis of uveitis before the onset of JIA all developed cataract and had an OR for development of glaucoma of 31.5 (95% CI, 3.6-274). Presence of antinuclear antibodies (ANAs) was also a risk factor for developing 1 or more ocular complications (OR, 3.0; 95% CI, 1.2-7.7). Decreased visual acuity (VA) CONCLUSIONS: Our results suggest that uveitis screening should start immediately when the diagnosis of JIA is suspected or confirmed and be continued for more than 8 years after the diagnosis of JIA. Timely systemic immunosuppressive treatment in patients with a high risk of developing ocular complications must be considered early in the disease course to gain rapid control of ocular inflammation.

Published
2021

6. Psoriasis and Temporomandibular Joint Involvement in Juvenile Idiopathic Arthritis (JIA) : A Longitudinal Study of the Nordic JIA Cohort

Author

Ekelund, Maria

Subjects
temporomandibular arthritis, arthritis, classification, juvenile psoriatic arthritis, juvenile idiopathic arthritis, Pediatrik, Pediatrics
Abstract

Juvenile idiopathic arthritis, JIA, is used as an umbrella term covering a heterogeneous group of chronic arthritis forms in children, many of which have important differences compared to adult arthritis, while others possibly represent similar diseases among children and adults. Classification aims to give a better understanding of the pathogenesis, patterns, disease trajectories and treatment responses. For the juvenile psoriatic arthritis, JPsA, the classification criteria are currently being debated. The distribution of affected joints in JIA differs greatly and it is unknown why some joints appear to be more affected than others. The temporomandibular joint (TMJ) can be affected early in the course of the disease and often the symptoms are mild and without obvious swelling. This thesis has its origin in the Nordic Study Group of Paediatric Rheumatology and the population-based prospective study of 510 children with newly diagnosed JIA included between 1997 and 1999. Totally 440 children were included in the eight-year follow-up, and in the TMJ study 265 patients were examined and underwent cone-beam computed tomography, CBCT, 17 years after onset. After eight years a considerable proportion of the children with definite psoriasis were classified as undifferentiated JIA based on the exclusion criteria in the ILAR classification. Our data also presents the heterogenicity of JPsA and the development over time of clinical variables supporting a psoriatic diathesis, as well as the overlap between JPsA and enthesitis-related arthritis in a group of patients. We found that extensive symptoms and dysfunctions of the TMJ are seen in JIA 17 years after disease onset, even in patients registered with inactive disease or remission. Individuals with substantial condylar damage on CBCT were found in all JIA categories. The deeper understanding of a chronic disease over time is crucial for research initiatives to improve care as well as for clinical decisions and planning of the health care. Our findings suggest a need for a more appropriate classification of JPsA and also that aspects of TMJ involvement should be included in the general health assessment in JIA.

Published
2020

7. Uveitis in Juvenile Idiopathic Arthritis 18-Year Outcome in the Population-based Nordic Cohort Study

Author

Rypdal, Veronika, Glerup, Mia, Songstad, Nils Thomas, Bertelsen, Geir, Christoffersen, Terje, Arnstad, Ellen D., Aalto, Kristiina, Berntson, Lillemor, Fasth, Anders, Herlin, Troels, Ekelund, Maria, Peltoniemi, Suvi, Toftedal, Peter, Nielsen, Susan, Leinonen, Sanna, Bangsgaard, Regitze, Nielsen, Rasmus, Rygg, Marite, and Nordal, Ellen

Subjects
long-term outcome, musculoskeletal diseases, treatment, genetic structures, uveitis cumulative incidence, VDP::Medisinske Fag: 700::Basale medisinske, odontologiske og veterinærmedisinske fag: 710, prospective, eye diseases, VDP::Medical disciplines: 700::Basic medical, dental and veterinary science disciplines: 710, population-based, risk factors for ocular complications, Ophthalmology, uveitis, juvenile idiopathic arthritis, Oftalmologi, ocular complications, skin and connective tissue diseases, disease activity, SUN criteria
Abstract

Purpose - To assess the long-term outcome of uveitis in juvenile idiopathic arthritis (JIA). Design - Population-based, multicenter, prospective JIA cohort, with a cross-sectional assessment of JIA-associated uveitis (JIA-U) 18 years after the onset of JIA. Participants - A total of 434 patients with JIA, of whom 96 had uveitis, from defined geographic areas of Denmark, Finland, Norway, and Sweden. Methods - Patients with onset of JIA between January 1997 and June 2000 were prospectively followed for 18 years. Pediatric rheumatologists and ophthalmologists collected clinical and laboratory data. Main Outcome Measures - Cumulative incidence of uveitis and clinical characteristics, JIA and uveitis disease activity, ocular complications, visual outcome, and risk factors associated with the development of uveitis-related complications. Results - Uveitis developed in 96 (22.1%) of 434 patients with JIA. In 12 patients (2.8%), uveitis was diagnosed between 8 and 18 years of follow-up. Systemic immunosuppressive medication was more common among patients with uveitis (47/96 [49.0%]) compared with patients without uveitis (78/338 [23.1%]). Active uveitis was present in 19 of 78 patients (24.4%) at the 18-year visit. Ocular complications occurred in 31 of 80 patients (38.8%). Short duration between the onset of JIA and the diagnosis of uveitis was a risk factor for developing ocular complications (odds ratio [OR], 1.4; 95% confidence interval [CI], 1.1–1.8). Patients with a diagnosis of uveitis before the onset of JIA all developed cataract and had an OR for development of glaucoma of 31.5 (95% CI, 3.6–274). Presence of antinuclear antibodies (ANAs) was also a risk factor for developing 1 or more ocular complications (OR, 3.0; 95% CI, 1.2–7.7). Decreased visual acuity (VA) Conclusions - Our results suggest that uveitis screening should start immediately when the diagnosis of JIA is suspected or confirmed and be continued for more than 8 years after the diagnosis of JIA. Timely systemic immunosuppressive treatment in patients with a high risk of developing ocular complications must be considered early in the disease course to gain rapid control of ocular inflammation.

Published
2020

8. Long-term outcomes in juvenile idiopathic arthritis: 18 years of follow-up in the population-based Nordic Juvenile Idiopathic Arthritis (JIA) cohort

Author

Glerup, Mia, Rypdal, Veronika Gjertsen, Arnstad, Ellen Dalen, Ekelund, Maria, Peltoniemi, Suvi, Aalto, Kristiina, Rygg, Marite, Toftedal, Peter, Nielsen, Susan, Fasth, Anders, Berntson, Lillemor, Nordal, Ellen Berit, and Herlin, Troels

Subjects
VDP::Medical disciplines: 700, VDP::Medisinske Fag: 700
Abstract

This is the peer reviewed version of the following article: Glerup, M., Rypdal, V., Arnstad, E. D., Ekelund, M., Peltoniemi, S., Aalto, K., ...Herlin, T. (2019). Long‐term outcomes in juvenile idiopathic arthritis: 18 years of follow‐up in the population‐based Nordic Juvenile Idiopathic Arthritis (JIA) cohort. Arthritis Care and Research, ?(?), ?. , which has been published in final form at https://doi.org/10.1002/acr.23853 . This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Use of Self-Archived Versions. Objectives - This study assessed the long‐term course, remission rate and disease burden in juvenile idiopathic arthritis (JIA) 18 years after disease onset in a population‐based setting from the early biologic era. Methods - A total of 510 consecutive cases of JIA with disease onset between 1997 and 2000 from defined geographic regions in Denmark, Norway, Sweden and Finland were prospectively included in this 18‐year cohort study. At the follow‐up visit, patient‐reported, demographic and clinical data were collected. Results - The study included 434 (85%) of the 510 eligible JIA participants. The mean age ± SD was 24.0 ± 4.4 years. The median juvenile arthritis disease activity (JADAS71) score was 1.5 (IQR 0‐5), with the ERA category of JIA having the highest median score, 4.5 (IQR 1.5–8.5) (P=0.003). In this cohort, 46% still had active disease, and 66 (15%) were treated with synthetic disease‐modifying anti‐rheumatic drugs and 84 (19%) with biologics. Inactive disease indicated by JADAS71 Conclusions - A high prevalence of the JIA cohort did not achieve CR despite new treatment options during the study period. The ERA category showed the worst outcomes and, in general, there is still a high burden of disease in adulthood for JIA.

Published
2019

10. Fatigue in juvenile idiopatic arthritis after 18 years of follow-up

Author

Arnstad, Ellen Dalen, Glerup, Mia, Rypdal, Veronika, Peltoniemi, Suvi, Ekelund, Maria, Berntson, Lillemor, Fasth, Anders, Nielsen, Susan, Zak, Marek, Aalto, Kristiina, Nordal, Ellen, Herlin, Troels, Romundstad, Pål Richard, and Rygg, Marite

Published
2019

12. Anti-tumour necrosis factor treatment for the prevention of ischaemic events in patients with deficiency of adenosine deaminase 2 (DADA2).

Author

Cooray, Samantha, Omyinmi, Ebun, Hong, Ying, Papadopoulou, Charalampia, Harper, Lorraine, Al-Abadi, Eslam, Goel, Ruchika, Dubey, Shirish, Wood, Mark, Jolles, Stephen, Berg, Stefan, Ekelund, Maria, Armon, Kate, Eleftheriou, Despina, and Brogan, Paul A

Subjects
ISCHEMIC stroke, CHILDREN'S hospitals, RETROSPECTIVE studies, HYDROLASES, TUMOR necrosis factors, HEMATOPOIETIC stem cell transplantation, VASCULITIS
Abstract

Objective To evaluate the impact of anti-Tumour Necrosis Factor-α (anti-TNF) treatment on the occurrence of vasculitic ischaemic events in patients with deficiency of adenosine deaminase 2 (DADA2). Methods A retrospective analysis of DADA2 patients referred from six centres to Great Ormond Street Hospital for Children was conducted. Ischaemic events, vasculitic disease activity, biochemical, immunological, and radiological features were compared, before and after anti-TNF treatment. Results A total of 31 patients with genetically confirmed DADA2 were included in the study. The median duration of active disease activity prior to anti-TNF treatment was 73 months (inter-quartile range [IQR] 27.5–133.5 months). Twenty seven/31 patients received anti-TNF treatment for a median of 32 months (IQR 12.0–71.5 months). The median event rate of central nervous system (CNS) and non-CNS ischemic events before anti-TNF treatment was 2.37 per 100 patient-months (IQR 1.25–3.63); compared with 0.00 per 100 patient-months (IQR 0.0–0.0) post-treatment (p < 0.0001). Paediatric vasculitis activity score (PVAS) was also significantly reduced: median score of 20/63 (IQR 13.0–25.8/63) pre-treatment vs. 2/63 (IQR 0.0–3.8/63) following anti-TNF treatment (p < 0.0001), with mild livedoid rash being the main persisting feature. Anti-TNF treatment was not effective for severe immunodeficiency or bone marrow failure, which required haematopoietic stem cell transplantation (HSCT). Conclusion Anti-TNF treatment significantly reduced the incidence of ischaemic events and other vasculitic manifestations of DADA2, but was not effective for immunodeficiency or bone marrow failure. [ABSTRACT FROM AUTHOR]

Published
2021
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13. Early self-reported pain in juvenile idiopathic arthritis (JIA) is related to long-term outcomes. Results from the Nordic JIA cohort study

Author

Arnstad, Ellen Dalen, Rypdal, Veronika, Peltoniemi, Suvi, Herlin, Troels, Berntson, Lillemor, Fasth, Anders, Nielsen, Susan, Glerup, Mia, Ekelund, Maria, Zak, Marek, Aalto, Kristiina, Nordal, Ellen, Romundstad, Pål Richard, and Rygg, Marite

Subjects
musculoskeletal diseases, VDP::Medisinske Fag: 700::Helsefag: 800, Inflammatory and Immune System: Underpinning Research, VDP::Medical disciplines: 700::Health sciences: 800, Betennelse og immunsystem: Underbyggende Forskning
Abstract

This is the peer reviewed version of the following article: Arnstad, E.D., Rypdal, V., Peltoniemi, S., Herlin, T., Berntson. L., Fasth, A., ..., Rygg, M.. Early self-reported pain in juvenile idiopathic arthritis (JIA) is related to long-term outcomes. Results from the Nordic JIA cohort study. Arthritis care & research, which has been published in final form at https://doi.org/10.1002/acr.23715. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Use of Self-Archived Versions. Objective: To study self‐reported pain early in the disease course of juvenile idiopathic arthritis (JIA) as predictor of long‐term disease outcomes. Methods: Consecutive cases of JIA with disease onset 1997‐2000 from defined geographical areas of Norway, Sweden, Finland and Denmark were prospectively enrolled in this population‐based cohort study. Self‐reported, disease‐related pain was measured on a 10 cm visual analogue scale (VAS pain). Inclusion criteria were a baseline visit with pain score six months after disease onset, followed by an eight‐year study visit. Remission was defined according to Wallace preliminary criteria. Functional disability was measured by Childhood Health Assessment Questionnaire (CHAQ) and Child Health Questionnaire (CHQ‐PF50) if age Results: The final study cohort consisted of 243 participants, and 120 (49%) had oligoarticular onset. At baseline 76% reported VAS pain >0 compared to 57% at eight‐year. Half of those who reported baseline pain also reported pain at eight‐year, but at a lower intensity. Compared to no pain, higher pain intensity at baseline predicted more pain at eight‐year, more functional disability, more damage and less remission off medication. Baseline pain predicted more use of DMARDs/biologics during the disease course. Participants with oligoarticular JIA reporting pain at baseline were more likely to develop extended oligoarticular or other unfavorable JIA categories. Conclusion: Early self‐reported, disease‐related pain among children and adolescents with JIA is common and seems to predict persistent pain and unfavorable long‐term disease outcomes.

Published
2018

14. Long-Term Outcome of Temporomandibular Joint Arthritis in Juvenile Idiopathic Arthritis:Results of 18-Year Follow-up in the Population-Based Nordic JIA Cohort

Author

Glerup, Mia, Stoustrup, Peter Bangsgaard, Matzen, Louise Hauge, Rypdal, Veronica, Nordal, Ellen, Frid, Paula, Arnstad, Ellen Dalen, Rygg, Marite, Thorarensen, Olafur, Ekelund, Maria, Berntson, Lillemor, Fasth, Anders, Nilsson, Håkan, Peltoniemi, Suvi, Aalto, Kristiina, Arte, Sirpa, Toftedal, Peter, Nielsen, Susan, Kreiborg, Sven, Herlin, Troels, and Pedersen, Thomas Klit

Abstract

980 Abstract Number 980

Published
2018

15. Psoriasis and associated variables in classification and outcome of juvenile idiopathic arthritis - an eight-year follow-up study

Author

Ekelund, Maria, Aalto, Kristiina, Fasth, Anders, Herlin, Troels, Nielsen, Susan, Nordal, Ellen, Peltoniemi, Suvi, Rygg, Marite, Zak, Marek, Berntson, Lillemor, Nordic Study Grp Pediat Rheumatol, Children's Hospital, University of Helsinki, Clinicum, and HUS Children and Adolescents

Subjects
Juvenile Rheumatoid, 3123 Gynaecology and paediatrics, Arthritis, 3121 General medicine, internal medicine and other clinical medicine, INTERNATIONAL LEAGUE, Psoriasis, CRITERIA, RHEUMATOLOGY CLASSIFICATION, CATEGORIES, CHILDREN, Child
Abstract

Background: To study the impact of psoriasis and features associated with psoriasis on classification and outcome in a population-based follow-up cohort of children with juvenile idiopathic arthritis (JIA). Methods: In all, 440 children with JIA were followed for a median of 8 years in a prospective Nordic population-based cohort study. Data for remission was available for 427 of these children. The presence of psoriasis, psoriasis-like rash, dactylitis, nail pitting, enthesitis, tenosynovitis and heredity was assessed in relation to ILAR classification and remission. Results: Clinical findings associated with psoriasis developed consecutively during the 8-year period. Six of 14 children with psoriasis were not classified as juvenile psoriatic arthritis according to the ILAR criteria at 8 year follow-up. Dactylitis was more common in children with early onset of JIA. After 8 years we found a cumulative median number of eleven arthritic joints in children with psoriasis or psoriasis- like rash compared with six in the rest of the cohort (p = 0.02). Also, the chance for not being in remission after 8 years increased significantly in patients with psoriasis, psoriasis-like rash or at least two of: 1) first-degree heredity for psoriasis or psoriatic arthritis, 2) dactylitis or 3) nail pitting, compared with the rest of the group (OR 3.32, p = 0.010). Conclusions: Our results indicate a more severe disease over time in psoriasis- associated JIA, as features of psoriasis develop during the disease course. This group is a major challenge to encompass in a future JIA classification in order to facilitate early tailored treatment.

Published
2017

17. Longterm Outcomes of Temporomandibular Joints in Juvenile Idiopathic Arthritis: 17 Years of Followup of a Nordic Juvenile Idiopathic Arthritis Cohort.

Author

Glerup, Mia, Stoustrup, Peter, Matzen, Louise H., Rypdal, Veronika, Nordal, Ellen, Frid, Paula, Arnstad, Ellen Dalen, Rygg, Marite, Thorarensen, Olafur, Ekelund, Maria, Berntson, Lillemor, Fasth, Anders, Nilsson, Håkan, Peltoniemi, Suvi, Aalto, Kristiina, Arte, Sirpa, Toftedal, Peter, Nielsen, Susan, Kreiborg, Sven, and Herlin, Troels

Published
2020
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18. Long-Term Outcomes in Juvenile Idiopathic Arthritis: Eighteen Years of Follow-Up in the Population-Based Nordic Juvenile Idiopathic Arthritis Cohort.

Author

Glerup, Mia, Rypdal, Veronika, Arnstad, Ellen Dalen, Ekelund, Maria, Peltoniemi, Suvi, Aalto, Kristiina, Rygg, Marite, Toftedal, Peter, Nielsen, Susan, Fasth, Anders, Berntson, Lillemor, Nordal, Ellen, Herlin, Troels, and Nordic Study Group of Pediatric Rheumatology

Subjects
BIOTHERAPY, JUVENILE idiopathic arthritis, ANTIRHEUMATIC agents, SEVERITY of illness index, TREATMENT effectiveness, SCANDINAVIANS, DISEASE remission, NORDIC people, LONGITUDINAL method
Abstract

Objective: The present study was undertaken to assess the long-term course, remission rate, and disease burden in juvenile idiopathic arthritis (JIA) 18 years after disease onset in a population-based setting from the early biologic era.Methods: A total of 510 consecutive cases of JIA with disease onset between 1997 and 2000 from defined geographic regions in Denmark, Norway, Sweden, and Finland were prospectively included in this 18-year cohort study. At the follow-up visit, patient-reported demographic and clinical data were collected.Results: The study included 434 (85%) of the 510 eligible JIA participants. The mean ± SD age was 24.0 ± 4.4 years. The median juvenile arthritis disease activity score in 71 joints (JADAS-71) was 1.5 (interquartile range [IQR] 0-5), with the enthesitis-related arthritis (ERA) category of JIA having the highest median score (4.5 [IQR 1.5-8.5], P = 0.003). In this cohort, 46% of patients still had active disease, and 66 (15%) were treated with synthetic disease-modifying antirheumatic drugs and 84 (19%) with biologics. Inactive disease indicated by a JADAS-71 score of <1 was seen in 48% of participants. Clinical remission off medication (CR) was documented in 33% of the participants with high variability among the JIA categories. CR was most often seen in persistent oligoarticular and systemic arthritis and least often in ERA (P < 0.001).Conclusion: A substantial proportion of the JIA cohort did not achieve CR despite new treatment options during the study period. The ERA category showed the worst outcomes, and in general there is still a high burden of disease in adulthood for JIA. [ABSTRACT FROM AUTHOR]

Published
2020
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19. Early Self-Reported Pain in Juvenile Idiopathic Arthritis as Related to Long-Term Outcomes: Results From the Nordic Juvenile Idiopathic Arthritis Cohort Study.

Author

Arnstad, Ellen Dalen, Rypdal, Veronika, Peltoniemi, Suvi, Herlin, Troels, Berntson, Lillemor, Fasth, Anders, Nielsen, Susan, Glerup, Mia, Ekelund, Maria, Zak, Marek, Aalto, Kristiina, Nordal, Ellen, Romundstad, Pål Richard, Rygg, Marite, and Nordic Study Group of Pediatric Rheumatology

Subjects
DISEASE progression, RESEARCH, PAIN measurement, PREDICTIVE tests, SELF-evaluation, TIME, RESEARCH methodology, DISABILITY evaluation, JUVENILE idiopathic arthritis, JOINT pain, EVALUATION research, SEVERITY of illness index, ANTIRHEUMATIC agents, TREATMENT effectiveness, COMPARATIVE studies, QUESTIONNAIRES, SCANDINAVIANS, NORDIC people, LONGITUDINAL method, DISEASE remission
Abstract

Objective: To study self-reported pain early in the disease course of juvenile idiopathic arthritis (JIA) as a predictor of long-term disease outcomes.Methods: Consecutive cases of JIA with disease onset from 1997 to 2000 from defined geographical areas of Norway, Sweden, Finland, and Denmark were prospectively enrolled in this population-based cohort study. Self-reported, disease-related pain was measured on a 10-cm visual analog scale (VAS pain). Inclusion criteria were a baseline visit with a pain score 6 months after disease onset, followed by an 8-year study visit. Remission was defined according to Wallace et al (2004) preliminary criteria. Functional disability was measured by the Childhood Health Assessment Questionnaire and the Child Health Questionnaire Parent Form if the child was age <18 years and by the Health Assessment Questionnaire if age ≥18 years. Damage was scored using the Juvenile Arthritis Damage Index.Results: The final study cohort consisted of 243 participants, and 120 participants (49%) had oligoarticular onset. At baseline, 76% reported a VAS pain score >0 compared to 57% reporting at 8 years. Half of those who reported baseline pain also reported pain at 8 years but at a lower intensity. Compared to no pain, higher pain intensity at baseline predicted more pain at 8 years, more functional disability, more damage, and less remission without medication. Baseline pain predicted more use of disease-modifying antirheumatic drugs/biologics during the disease course. Participants with oligoarticular JIA reporting pain at baseline were more likely to develop extended oligoarticular JIA or other JIA categories with an unfavorable prognosis.Conclusion: Early self-reported, disease-related pain among children and adolescents with JIA is common and seems to predict persistent pain and unfavorable long-term disease outcomes. [ABSTRACT FROM AUTHOR]

Published
2019
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20. Deficiency of Adenosine Deaminase Type 2: A Description of Phenotype and Genotype in Fifteen Cases.

Author

Nanthapisal, Sira, Murphy, Claire, Omoyinmi, Ebun, Hong, Ying, Standing, Ariane, Berg, Stefan, Ekelund, Maria, Jolles, Stephen, Harper, Lorraine, Youngstein, Taryn, Gilmour, Kimberly, Klein, Nigel J., Eleftheriou, Despina, and Brogan, Paul A.

Subjects
ANGIOGRAPHY, BIOLOGICAL assay, METABOLIC disorders, GENETIC mutation, POLYMERASE chain reaction, PROBABILITY theory, TUMOR necrosis factors, PHENOTYPES, GENOMICS, DATA analysis software, GENE expression profiling, SEQUENCE analysis, MANN Whitney U Test, GENOTYPES, CHEMICAL inhibitors
Abstract

Objective To describe the clinical features, genotype, and treatment in a series of subjects with confirmed adenosine deaminase 2 (ADA2) deficiency. Methods All symptomatic subjects were referred for genetic testing for suspected ADA2 deficiency; relatives of index cases were also screened. Demographic, clinical, and laboratory characteristics and treatments were recorded. Genetic analyses included whole-exome sequencing in 4 subjects and Sanger sequencing of CECR1 (the gene for cat eye syndrome chromosome region candidate 1) in all subjects. Assays for ADA2 enzyme activity and quantitative polymerase chain reaction analysis of CECR1 messenger RNA (mRNA) were also performed. Results We identified 15 subjects with ADA2 deficiency, 5 of whom were asymptomatic (relatives of index cases; ages 5-42 years). Homozygous or compound heterozygous mutations in CECR1 were identified in all subjects. Phenotypic manifestations in the patients with symptomatic ADA2 deficiency included livedo racemosa (73.3%), neurologic involvement (53.3%), and immunodeficiency (46.7%). CECR1 mRNA expression in 8 subjects, including 5 who were presymptomatic, was significantly lower than in healthy controls ( P = 0.0016). Subjects with ADA2 deficiency (with or without symptoms) also had lower ADA2 enzyme activity compared to healthy pediatric controls ( P < 0.0001) and patients with sporadic (nonfamilial) childhood polyarteritis nodosa (PAN) without CECR1 mutation ( P = 0.0108). Anti−tumor necrosis factor therapy was required in 9 of the 10 symptomatic subjects. Conclusion The clinical manifestations of ADA2 deficiency ranged in severity from limited cutaneous involvement to severe multisystemic vasculitis; one-third of our cases (5 of 15) were currently asymptomatic, and required close monitoring. We recommend CECR1 screening for unaffected siblings of index cases, cases of familial vasculitis, and cases of PAN that is resistant to standard treatment. [ABSTRACT FROM AUTHOR]

Published
2016
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21. Proceedings of the 23rd Paediatric Rheumatology European Society Congress: part two: Genoa, Italy. 28 September – 01 October 2016

Author

Lomakina, Olga, Alekseeva, Ekaterina, Valieva, Sania, Bzarova, Tatiana, Nikishina, Irina, Zholobova, Elena, Rodionovskaya, Svetlana, Kaleda, Maria, Nakagishi, Yasuo, Shimizu, Masaki, Mizuta, Mao, Yachie, Akihiro, Sugita, Yuko, Okamoto, Nami, Shabana, Kousuke, Murata, Takuji, Tamai, Hiroshi, Smith, Eve M., Yin, Peng, Jorgensen, Andrea L., Beresford, Michael W., Eleuteri, Antonio, Goilav, Beatrice, Lewandowski, Laura, Phuti, Angel, Wahezi, Dawn, Rubinstein, Tamar, Jones, Caroline, Newland, Paul, Marks, Stephen, Corkhill, Rachel, Ekdawy, Diana, Pilkington, Clarissa, Tullus, Kjell, Putterman, Chaim, Scott, Chris, Fisher, Antony C., Jorgensen, Andrea, Batu, Ezgi Deniz, Kosukcu, Can, Taskiran, Ekim, Akman, Sema, Ozturk, Kubra, Sozeri, Betul, Unsal, Erbil, Ekinci, Zelal, Bilginer, Yelda, Alikasifoglu, Mehmet, Ozen, Seza, Lythgoe, Hanna, Brunner, Hermine I., Gulati, Gaurav, Jones, Jordan T., Altaye, Mekibib, Eaton, Jamie, Difrancesco, Mark, Yeo, Joo Guan, Leong, Jingyao, Bathi, Loshinidevi D/O Thana, Arkachaisri, Thaschawee, Albani, Salvatore, Abdelrahman, Nagla, Beresford, Michael W, Leone, Valentina, Groot, Noortje, Shaikhani, D., Bultink, I. E. M., Bijl, M., Dolhain, R. J. E. M., Teng, Y. K. O., Zirkzee, E., de Leeuw, K., Fritsch-Stork, R., Kamphuis, S. S. M., Wright, Rachael D., Abdawani, Reem, Al Shaqshi, Laila, Al Zakwani, Ibrahim, Gormezano, Natali W., Kern, David, Pereira, Oriany L., Esteves, Gladys C. C., Sallum, Adriana M., Aikawa, Nadia E., Pereira, Rosa M., Silva, Clovis A., Bonfa, Eloisa, Beckmann, Jessica, Bartholomä, Nora, Venhoff, Nils, Henneke, Philipp, Salzer, Ulrich, Janda, Ales, Boteanu, Alina Lucica, Corral, Sandra Garrote, Giraldo, Alberto Sifuentes, Gámir, Mariluz Gámir, Mendoza, Antonio Zea, Adrovic, Amra, Dedeoglu, Reyhan, Sahin, Sezgin, Barut, Kenan, Koka, Aida, Oztunc, Funda, Kasapcopur, Ozgur, Rodriguez-Lozano, Ana Luisa, Rivas-Larrauri, Francisco, de la Puente, Silvestre García, Alves, Andressa G. F., Giacomin, Maria F. D. A., Farhat, Juliana, Braga, Alfésio L. F., Sallum, Adriana M. E., Campos, Lúcia M. D. A., Pereira, Luiz A. A., Lichtenfels, Ana J. D. F. C., Silva, Clóvis A., Farhat, Sylvia C. 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Published
2017
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22. Complement lectin pathway protein levels reflect disease activity in juvenile idiopathic arthritis: a longitudinal study of the Nordic JIA cohort.

Author

Glerup, Mia, Thiel, Steffen, Rypdal, Veronika, Arnstad, Ellen Dalen, Ekelund, Maria, Peltoniemi, Suvi, Aalto, Kristiina, Rygg, Marite, Nielsen, Susan, Fasth, Anders, Berntson, Lillemor, Nordal, Ellen, and Herlin, Troels

Subjects
JUVENILE idiopathic arthritis, MACROPHAGE activation syndrome, JUVENILE diseases, BLOOD proteins, LONGITUDINAL method, PROTEINS
Abstract

Background: To determine the serum levels of the lectin pathway proteins early in the disease course and 17 years after disease onset and to correlate the protein levels to markers of disease activity in participants from a population-based Nordic juvenile idiopathic arthritis (JIA) cohort. Additionally, to assess the predictive value of lectin pathway proteins with respect to remission status. Methods: A population-based cohort study of consecutive cases of JIA with a disease onset from 1997 to 2000 from defined geographical areas of Finland, Sweden, Norway and Denmark with 17 years of follow-up was performed. Clinical characteristics were registered and H-ficolin, M-ficolin, MASP-1, MASP-3, MBL and CL-K1 levels in serum were analyzed. Results: In total, 293 patients with JIA were included (mean age 23.7 ± 4.4 years; mean follow-up 17.2 ± 1.7 years). Concentrations of the lectin protein levels in serum were higher at baseline compared to the levels 17 years after disease onset (p ≤ 0.006, n = 164). At baseline, the highest level of M-ficolin was observed in systemic JIA. Further, high M-ficolin levels at baseline and at 17-year follow-up were correlated to high levels of ESR. In contrast, high MASP-1 and MASP-3 tended to correlate to low ESR. CL-K1 showed a negative correlation to JADAS71 at baseline. None of the protein levels had prognostic abilities for remission status 17 years after disease onset. Conclusion: We hypothesize that increased serum M-ficolin levels are associated with higher disease activity in JIA and further, the results indicate that MASP-1, MASP-3 and CL-K1 are markers of inflammation. [ABSTRACT FROM AUTHOR]

Published
2019
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23. Uveitis in Juvenile Idiopathic Arthritis: 18-Year Outcome in the Population-based Nordic Cohort Study.

Author

Rypdal, Veronika, Glerup, Mia, Songstad, Nils Thomas, Bertelsen, Geir, Christoffersen, Terje, Arnstad, Ellen D., Aalto, Kristiina, Berntson, Lillemor, Fasth, Anders, Herlin, Troels, Ekelund, Maria, Peltoniemi, Suvi, Toftedal, Peter, Nielsen, Susan, Leinonen, Sanna, Bangsgaard, Regitze, Nielsen, Rasmus, Rygg, Marite, and Nordal, Ellen

Subjects
*JUVENILE idiopathic arthritis, *IRIDOCYCLITIS, *UVEITIS
Abstract

To assess the long-term outcome of uveitis in juvenile idiopathic arthritis (JIA). Population-based, multicenter, prospective JIA cohort, with a cross-sectional assessment of JIA-associated uveitis (JIA-U) 18 years after the onset of JIA. A total of 434 patients with JIA, of whom 96 had uveitis, from defined geographic areas of Denmark, Finland, Norway, and Sweden. Patients with onset of JIA between January 1997 and June 2000 were prospectively followed for 18 years. Pediatric rheumatologists and ophthalmologists collected clinical and laboratory data. Cumulative incidence of uveitis and clinical characteristics, JIA and uveitis disease activity, ocular complications, visual outcome, and risk factors associated with the development of uveitis-related complications. Uveitis developed in 96 (22.1%) of 434 patients with JIA. In 12 patients (2.8%), uveitis was diagnosed between 8 and 18 years of follow-up. Systemic immunosuppressive medication was more common among patients with uveitis (47/96 [49.0%]) compared with patients without uveitis (78/338 [23.1%]). Active uveitis was present in 19 of 78 patients (24.4%) at the 18-year visit. Ocular complications occurred in 31 of 80 patients (38.8%). Short duration between the onset of JIA and the diagnosis of uveitis was a risk factor for developing ocular complications (odds ratio [OR], 1.4; 95% confidence interval [CI], 1.1–1.8). Patients with a diagnosis of uveitis before the onset of JIA all developed cataract and had an OR for development of glaucoma of 31.5 (95% CI, 3.6–274). Presence of antinuclear antibodies (ANAs) was also a risk factor for developing 1 or more ocular complications (OR, 3.0; 95% CI, 1.2–7.7). Decreased visual acuity (VA) <6/12 was found in 12 of 135 eyes (8.9%) with uveitis, and 4 of 80 patients (5.0%) with JIA-U had binocular decreased VA <6/12. Our results suggest that uveitis screening should start immediately when the diagnosis of JIA is suspected or confirmed and be continued for more than 8 years after the diagnosis of JIA. Timely systemic immunosuppressive treatment in patients with a high risk of developing ocular complications must be considered early in the disease course to gain rapid control of ocular inflammation. [ABSTRACT FROM AUTHOR]

Published
2021
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24. Phenotypic variability and disparities in treatment and outcomes of childhood arthritis throughout the world: an observational cohort study

Author

Adriana Apostol, Matilda Laday, Michael Hofer, Amita Aggarwal, Pierre Quartier, Dirk Foell, Raju Khubchandani, Nikolay Tzaribachev, Patrizia Barone, Angela Pistorio, Ivan Foeldvari, Angela Miniaci, Pamela Weiss, Nicolino Ruperto, Violeta Panaviene, Claudia Saad Magalhães, Betül Sözeri, Gordana Vijatov-Djuric, Erkan Demirkaya, Carine Wouters, Calin Lazar, Rubén Burgos-Vargas, Tamás Constantin, Zoilo Morel Ayala, Carmen De Cunto, Elena Tsitsami, Marta Torcoletti, B Varbanova, Angelo Ravelli, Nahid Shafaie, Soad Hashad, Maria Greca Magnolia, José Melo-Gomes, Kirsten Minden, Ilonka Orbán, Flavio Sztajnbok, Maka Ioseliani, Ingrida Rumba-Rozenfelde, Silvia Magni Manzoni, Francesca Bovis, Berit Flatø, Stella Garay, Olga Arguedas, Maria Cristina Maggio, Yahya Aghighi, Sujata Sawhney, Francesco La Torre, Ruben Cuttica, Jaime de Inocencio, Clara Malagon, Alina Boteanu, Gerd Horneff, Silvana Martino, Sulaiman M. Al-Mayouf, Alberto Martini, Maria Trachana, Christiaan Scott, Rolando Cimaz, Nuray Aktay Ayaz, Maya-Feriel Aiche, Irina Nikishina, Valeria Gerloni, Sylvia Kamphuis, Olga Vougiouka, Dirk Holzinger, Reza Shiari, Sara Pieropan, Nico M Wulffraat, Inmaculada Calvo Penades, MM Katsicas, Pablo Mesa-del-Castillo, Lidia Rutkowska-Sak, Cristina Herrera, Jelena Vojinovic, Daniel J. Lovell, Erbil Unsal, Miroslav Harjacek, Yosef Uziel, Dimitrina Mihaylova, Gordana Susic, Susan Nielsen, Pekka Lahdenne, Soamarat Vilaiyuk, Rita Consolini, Ekaterina Alexeeva, Chris Pruunsild, Gaëlle Chédeville, Nicolae Iagaru, Ozgur Kasapcopur, Troels Herlin, Anne Estmann Christensen, Yaryna Boyko, Carolina Montobbio, Sarah Ringold, Johannes-Peter Haas, Gerd Ganser, Jordi Anton, Marite Rygg, Liisa Kröger, Reem Abdwani, Pavla Dolezalova, Paivi Miettunen, Rosa Anna Podda, Sheila Knupp Feitosa de Oliveira, Graciela Espada, Richard Vesely, Merja Malin, Liora Harel, Veronika Vargova, Mohammad Hasan Moradinejad, Neil A. Martin, Adele Civino, Tadej Avcin, Hans-Iko Huppertz, Ellen Nordal, Ximena Norambuena, Alessandra Alongi, Serena Pastore, Karaman Pagava, Maria Ekelund, Donato Rigante, Rotraud K. Saurenmann, Lillemor Berntson, Tomáš Dallos, Elżbieta Smolewska, Rik Joos, Andrea Militaru, Alessandro Consolaro, C. Ailioaie, Romina Gallizzi, Gabriella Giancane, Agustin Remesal, Anuela Kondi, Safiya Al-Abrawi, Anne Putto-Laurila, Joost F Swart, Paula Vähäsalo, Evert Hendrik Pieter van Dijkhuizen, Amparo Ibanez Estrella, Yasser El Miedany, Consolaro, Alessandro, Giancane, Gabriella, Alongi, Alessandra, van Dijkhuizen, Evert Hendrik Pieter, Aggarwal, Amita, Al-Mayouf, Sulaiman M, Bovis, Francesca, De Inocencio, Jaime, Demirkaya, Erkan, Flato, Berit, Foell, Dirk, Garay, Stella Mari, Lazăr, Călin, Lovell, Daniel J, Montobbio, Carolina, Miettunen, Paivi, Mihaylova, Dimitrina, Nielsen, Susan, Orban, Ilonka, Rumba-Rozenfelde, Ingrida, Magalhães, Claudia Saad, Shafaie, Nahid, Susic, Gordana, Trachana, Maria, Wulffraat, Nico, Pistorio, Angela, Martini, Alberto, Ruperto, Nicolino, Ravelli, Angelo, Abdwani, Reem, Aghighi, Yahya, Aiche, Maya-Feriel, Ailioaie, Constantin, Aktay Ayaz, Nuray, Al-Abrawi, Safiya, Alexeeva, Ekaterina, Anton, Jordi, Apostol, Adriana, Arguedas, Olga, Avcin, Tadej, Barone, Patrizia, Berntson, Lillemor, Boteanu, Alina Lucica, Boyko, Yaryna, Burgos-Vargas, Ruben, Calvo Penades, Inmaculada, Chédeville, Gaëlle, Cimaz, Rolando, Civino, Adele, Consolini, Rita, Constantin, Tama, Cuttica, Ruben, Dallos, Toma, Martin, Neil, Magni Manzoni, Silvia, De Cunto, Carmen, Dolezalova, Pavla, Ekelund, Maria, El Miedany, Yasser, Espada, Graciela, Estmann Christensen, Anne, Foeldvari, Ivan, Gallizzi, Romina, Ganser, Gerd, Gerloni, Valeria, Haas, Johannes-Peter, Harel, Liora, Harjacek, Miroslav, Hashad, Soad, Herlin, Troel, Herrera, Cristina, Hofer, Michael, Holzinger, Dirk, Horneff, Gerd, Huppertz, Hans-Iko, Iagăru, Nicolae, Ibanez Estrella, Amparo, Ioseliani, Maka, Joos, Rik, Knupp Oliveira, Sheila, Kamphuis, Sylvia, Kasapcopur, Ozgur, Katsicas, Maria Martha, Khubchandani, Raju, Kondi, Anuela, Kröger, Liisa, La Torre, Francesco, Laday, Matilda, Lahdenne, Pekka, Maggio, Maria Cristina, Magnolia, Maria Greca, Malagon, Clara, Malin, Merja, Martino, Silvana, Melo-Gomes, Jose Antonio, Mesa-del-Castillo, Pablo, Militaru, Andrea, Minden, Kirsten, Miniaci, Angela, Moradinejad, Mohammad Hasan, Morel Ayala, Zoilo, Nikishina, Irina, Norambuena, Ximena, Nordal, Ellen Berit, Pagava, Karaman, Panaviene, Violeta, Pastore, Serena, Pieropan, Sara, Podda, Rosa Anna, Pruunsild, Chri, Putto-Laurila, Anne, Quartier, Pierre, Remesal, Agustin, Rigante, Donato, Ringold, Sarah, Rutkowska-Sak, Lidia, Rygg, Marite, Saurenmann, Rotraud Katharina, Sawhney, Sujata, Scott, Christiaan, Shiari, Reza, Smolewska, Elzbieta, Sozeri, Betul, Swart, Joost Fran, Sztajnbok, Flavio, Torcoletti, Marta, Tsitsami, Elena, Tzaribachev, Nikolay, Unsal, Erbil, Uziel, Yosef, Vähäsalo, Paula, Varbanova, Boriana, Vargova, Veronika, Vesely, Richard, Vijatov-Djuric, Gordana, Vilaiyuk, Soamarat, Vojinovic, Jelena, Vougiouka, Olga, Weiss, Pamela, Wouters, Carine, Pediatrics, Univ Genoa, Wilhelmina Childrens Hosp, Sanjay Gandhi Postgrad Inst Med Sci, Univ Hosp 12 Octubre, Alfaisal Univ, Western Univ Childrens Hosp, Oslo Univ Hosp, Univ Oslo, Univ Hosp Munster, Hosp Sor Maria Ludovica, Bucharest Emergency Hosp, Childrens Emergency Hosp, Cincinnati Childrens Hosp Med Ctr, Alberta Childrens Prov Gen Hosp, Sofiamed, Rigshosp, Natl Inst Rheumatism & Physiotherapy, Univ Latvia, Universidade Estadual Paulista (Unesp), Shariati Hosp, Inst Rheumatol Belgrade, and Thessaloniki Univ

Subjects
Male, medicine.medical_specialty, Childhood arthritis, Cross-sectional study, Population, Global Health, Pediatrics, 03 medical and health sciences, 0302 clinical medicine, 030225 pediatrics, Epidemiology, medicine, Developmental and Educational Psychology, Journal Article, Humans, Pediatrics, Perinatology and Child Health, 030212 general & internal medicine, Healthcare Disparities, Child, education, Disease burden, Pain Measurement, Retrospective Studies, education.field_of_study, Oligoarthritis, business.industry, Perinatology and Child Health, Juvenile idiopathic arthritis, medicine.disease, JUVENILE IDIOPATHIC ARTHRITIS, OF-RHEUMATOLOGY RECOMMENDATIONS, DISEASE-ACTIVITY SCORE, DEFINING CRITERIA, CLASSIFICATION, CHILDREN, EPIDEMIOLOGY, VALIDATION, COUNTRIES, VALIDITY, Arthritis, Juvenile, childhood arthritis,phenotypic variability,observational cohort study, Cross-Sectional Studies, Biological Variation, Population, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, Antirheumatic Agents, Child, Preschool, Quality of Life, Female, Polyarthritis, Juvenile idiopatic arthritis, of-rheumatology recommentadions, disease-activity score, defining criteria, classification, children, epidemiology, validation, countries, validity, business, Demography, Cohort study
Abstract

Made available in DSpace on 2019-10-05T16:54:20Z (GMT). No. of bitstreams: 0 Previous issue date: 2019-04-01 IRCCS Istituto Giannina Gaslini Background To our knowledge, the characteristics and burden of childhood arthritis have never been studied on a worldwide basis. We aimed to investigate, with a cross-sectional study, the prevalence of disease categories, treatment methods, and disease status in patients from across different geographical areas and from countries with diverse wealth status. Methods In this multinational, cross-sectional, observational cohort study, we asked international paediatric rheumatologists from specialised centres to enrol children with a diagnosis of juvenile idiopathic arthritis, according to International League of Associations for Rheumatology criteria, who were seen consecutively for a period of 6 months. Each patient underwent retrospective and cross-sectional assessments, including measures of disease activity and damage and questionnaires on the wellbeing and quality of life of the children. We qualitatively compared the collected data across eight geographical areas, and we explored an association between disease activity and damage and a country's gross domestic product (GDP) with a multiple logistic regression analysis. Findings Between April 4, 2011, and Nov 21, 2016, 9081 patients were enrolled at 130 centres in 49 countries, grouped into eight geographical areas. Systemic arthritis (125 [33.0%] of 379 patients) and enthesitis-related arthritis (113 [29.8%] of 379) were more common in southeast Asia, whereas oligoarthritis was more prevalent in southern Europe (1360 [56.7%] of 2400) and rheumatoid factor-negative polyarthritis was more frequent in North America (165 [31.5%] of 523) than in the other areas. Prevalence of uveitis was highest in northern Europe (161 [19.1%] of 845 patients) and southern Europe (450 [18.8%] of 2400) and lowest in Latin America (54 [6.4%] of 849), Africa and Middle East (71 [5.9%] of 1209), and southeast Asia (19 [5.0%] of 379). Median age at disease onset was lower in southern Europe (3.5 years, IQR 1.9-7.3) than in other regions. Biological, disease-modifying antirheumatic drugs were prescribed more frequently in northern Europe and North America than in other geographical settings. Patients living in countries with lower GDP had greater disease activity and damage than those living in wealthier countries. Damage was associated with referral delay. Interpretation Our study documents a variability in prevalence of disease phenotypes and disparities in therapeutic choices and outcomes across geographical areas and wealth status of countries. The greater disease burden in lowerresource settings highlights the need for public health efforts aimed at improving equity in access to effective treatments and care for juvenile idiopathic arthritis. Copyright (C) 2019 Elsevier Ltd. All rights reserved. Univ Genoa, Ist Giannina Gaslini, IRCCS, Clin Paediat & Rheumatol, Genoa, Italy Univ Genoa, Ist Giannina Gaslini, IRCCS, Epidemiol & Biostat Serv, Genoa, Italy Univ Genoa, Ist Giannina Gaslini, IRCCS, Sci Directory, Genoa, Italy Univ Genoa, PRINTO, IRCCS, Ist Giannina Gaslini, Genoa, Italy Univ Genoa, Dept Neurosci Rehabil Ophthalmol Genet & Maternal, Genoa, Italy Wilhelmina Childrens Hosp, Dept Pediat Immunol & Rheumatol, Utrecht, Netherlands Sanjay Gandhi Postgrad Inst Med Sci, Lucknow, Uttar Pradesh, India Univ Hosp 12 Octubre, Dept Pediat Rheumatol, Madrid, Spain Alfaisal Univ, Dept Pediat Rheumatol, King Faisal Specialist Hosp, Riyadh, Saudi Arabia Alfaisal Univ, Res Ctr, Riyadh, Saudi Arabia Western Univ Childrens Hosp, Hlth Sci Ctr, London, ON, Canada Oslo Univ Hosp, Dept Rheumatol, Oslo, Norway Oslo Univ Hosp, Med Fac, Oslo, Norway Univ Oslo, Oslo, Norway Oslo Univ Hosp, Norwegian Natl Advisory Unit Rheumat Dis Children, Oslo, Norway Univ Hosp Munster, Dept Pediat Rheumatol & Immunol, Munster, Germany Hosp Sor Maria Ludovica, Rheumatol Serv, La Plata, Buenos Aires, Argentina Bucharest Emergency Hosp, Cluj Napoca, Romania Childrens Emergency Hosp, Cluj Napoca, Romania Cincinnati Childrens Hosp Med Ctr, Div Rheumatol, Cincinnati, OH 45229 USA Alberta Childrens Prov Gen Hosp, Div Pediat Rheumatol, Dept Pediat, Calgary, AB, Canada Sofiamed, Pediat Dept, Sofia, Bulgaria Rigshosp, Juliane Marie Ctr, Paediat Rheumatol Unit, Copenhagen, Denmark Natl Inst Rheumatism & Physiotherapy, Clin Immunol Adult & Paediat Rheumatol Dept, Budapest, Hungary Univ Latvia, Pediat Dept, Riga, Latvia Univ Latvia, Univ Childrens Hosp, Riga, Latvia Univ Estadual Paulista, Botucatu Fac Med, Botucatu, SP, Brazil Shariati Hosp, Rheumatol Res Ctr, Dept Pediat & Rheumatol, Tehran, Iran Inst Rheumatol Belgrade, Div Pediat Rheumatol, Belgrade, Serbia Thessaloniki Univ, Dept Pediat 1, Hippokrat Gen Hosp, Sch Med, Thessaloniki, Greece Univ Estadual Paulista, Botucatu Fac Med, Botucatu, SP, Brazil

Published
2019

25. Development and initial validation of parent and child versions of the Juvenile Arthritis Disease Activity Score.

Author

Naddei R, Ridella F, Bovis F, Trincianti C, Avrusin I, Januskeviciute G, Burrone M, Rebollo-Giménez A, Minden K, Ekelund M, Barone P, Rumba-Rozenfelde I, Shafaie N, Swart JF, Ruperto N, Ravelli A, and Consolaro A

Abstract

Objective: To develop parent- and child-centered versions of the Juvenile Arthritis Disease Activity Score (JADAS) and to provide preliminary evidence of their validity., Methods: Validation analyses were conducted on two large multinational datasets of patients with juvenile idiopathic arthritis (JIA) and included assessment of construct validity, internal consistency and structure, discriminative validity, responsiveness to change, and predictive validity., Results: The parJADAS and patJADAS include four parent/patient-reported outcomes, each measured on a 0-10 scale: assessment of overall disease activity; rating of pain intensity; assessment of activity of joint disease; duration of morning stiffness. Both scores are calculated as the simple linear sum of the scores of their 4 components, which yields for both of them a global score of 0-40. The parJADAS and patJADAS demonstrated good construct validity, yielding high correlations with other JIA composite disease activity measures and moderate correlations with physician global rating and joint counts. Internal consistency was satisfactory, with Cronbach' s alpha > 0.80, and exploratory factor analysis showed that both indices are monodimensional. Both instruments discriminated well between different disease states, with discriminative ability being not affected by the presence of damage, proved able to predict important disease outcomes, and showed fair responsiveness to clinically important change, with standardized response mean of 0.71., Conclusion: Both parJADAS and patJADAS were found to possess good measurement properties and to serve as surrogate of physicians' evaluations. Regular home completion of the two instruments through digital technologies offers a suitable and pragmatic approach to deliver remote symptom monitoring and telehealth., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Published
2024
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26. Psoriasis and associated variables in classification and outcome of juvenile idiopathic arthritis - an eight-year follow-up study.

Author

Ekelund M, Aalto K, Fasth A, Herlin T, Nielsen S, Nordal E, Peltoniemi S, Rygg M, Zak M, and Berntson L

Subjects
Age of Onset, Arthritis, Juvenile classification, Arthritis, Juvenile epidemiology, Arthritis, Psoriatic classification, Arthritis, Psoriatic epidemiology, Arthritis, Psoriatic etiology, Child, Child, Preschool, Female, Follow-Up Studies, Humans, Infant, Male, Nail Diseases classification, Nail Diseases epidemiology, Nail Diseases etiology, Prospective Studies, Psoriasis classification, Psoriasis epidemiology, Scandinavian and Nordic Countries epidemiology, Arthritis, Juvenile complications, Psoriasis etiology
Abstract

Background: To study the impact of psoriasis and features associated with psoriasis on classification and outcome in a population-based follow-up cohort of children with juvenile idiopathic arthritis (JIA)., Methods: In all, 440 children with JIA were followed for a median of 8 years in a prospective Nordic population-based cohort study. Data for remission was available for 427 of these children. The presence of psoriasis, psoriasis-like rash, dactylitis, nail pitting, enthesitis, tenosynovitis and heredity was assessed in relation to ILAR classification and remission., Results: Clinical findings associated with psoriasis developed consecutively during the 8-year period. Six of 14 children with psoriasis were not classified as juvenile psoriatic arthritis according to the ILAR criteria at 8 year follow-up. Dactylitis was more common in children with early onset of JIA. After 8 years we found a cumulative median number of eleven arthritic joints in children with psoriasis or psoriasis-like rash compared with six in the rest of the cohort (p = 0.02). Also, the chance for not being in remission after 8 years increased significantly in patients with psoriasis, psoriasis-like rash or at least two of: 1) first-degree heredity for psoriasis or psoriatic arthritis, 2) dactylitis or 3) nail pitting, compared with the rest of the group (OR 3.32, p = 0.010)., Conclusions: Our results indicate a more severe disease over time in psoriasis-associated JIA, as features of psoriasis develop during the disease course. This group is a major challenge to encompass in a future JIA classification in order to facilitate early tailored treatment.

Published
2017
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