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1.
Artigo em Inglês | MEDLINE | ID: mdl-33506861

RESUMO

OBJECTIVE: Structural equation modelling (SEM) was applied to data from the Research in Arthritis in Canadian Children emphasizing Outcomes (ReACCh-Out) cohort to help elucidate causal pathways to decreased health-related quality of life (HRQoL) in children with juvenile idiopathic arthritis (JIA). METHODS: Based on published literature and clinical plausibility, a priori models were constructed with explicit root causes (disease activity, treatment intensity) and mediators (pain, disease symptoms, functional impairments) leading to HRQoL measured by the Quality of my Life (QoML) scale and the Juvenile Arthritis Quality of Life Questionnaire (JAQQ), at five disease stages: 1) diagnosis, 2) 3-9 months after diagnosis, 3) flare, 4) remission on medications, 5) remission off medications. Following SEM, a posteriori models were selected based on data fit and clinical plausibility. RESULTS: We included 561, 887, 137, 186 and 182 patients at each stage, respectively. In a posteriori models for active disease stages, paths from disease activity led through pain, functional impairments, and disease symptoms, directly or through restrictions in participation, to decreased QoML scores. Treatment intensity had detrimental effects through psychosocial domains; while treatment side effects had a lesser role. Pathways were similar for QoML and JAQQ, but JAQQ models provided greater specificity. Models for remission stages were not supported by the data. CONCLUSION: Our findings support disease activity and treatment intensity as root causes of decreased HRQoL in children with JIA, with pain, functional impairments and participation restrictions as mediators for disease activity; and psychosocial effects and side effects as mediators for treatment intensity.

2.
Artigo em Inglês | MEDLINE | ID: mdl-32976694

RESUMO

OBJECTIVE: The aim of this study was to examine the impact of timing of childhood-onset systemic lupus erythematosus (cSLE) diagnosis relative to menarchal status, on final height, accounting for and disease associated factors. METHODS: A cohort study of female cSLE patients <18 years of age at diagnosis, followed at a tertiary care, pediatric center from July 1982 to March 2016, restricted to patients with documented age of menarche and final height. We compared final height between patients diagnosed pre- and post-menarche. We tested the association of timing of cSLE diagnosis with final height, adjusted for ethnicity, in linear regression models. We performed subgroup analyses of patients with growth during follow-up, additionally adjusting for average daily corticosteroid dose and disease activity. RESULTS: Of 401 female cSLE patients in the study, 115 patients (29%) were diagnosed pre-menarche and 286 (71%) post-menarche. Patients diagnosed pre-menarche were older at menarche compared with patients diagnosed post-menarche (13.5 ± 1.4 vs. 12.5 ± 1.3 years; p < 0.001). The mean final height for females diagnosed post-menarche (161.4 cm [SD 6.9 cm]) was greater than those diagnosed pre-menarche (158.8 cm [SD 7.3 cm], P=0.001). In regression analysis, those diagnosed post-menarche were significantly taller than those diagnosed pre-menarche, adjusted for ethnicity, and disease severity (Beta=2.6cm, [SD 0.7cm], P=0.0006). CONCLUSION: In this large cohort study of females with cSLE, patients diagnosed post-menarche achieved a taller final height than those diagnosed pre-menarche even after accounting for ethnicity and disease severity.

3.
J Rheumatol ; 2020 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-32739897

RESUMO

OBJECTIVE: To evaluate the feasibility of studying creatine in juvenile dermatomyositis (JDM). Secondary objectives were to determine the effect of creatine on muscle function and metabolism, aerobic capacity, fatigue, physical activity and quality of life, as well as its safety. METHODS: We conducted a 6-month double-blind, randomized, multiple-baseline design; patients were assigned to creatine or placebo. Feasibility was assessed using attended study visits, completed study procedures, and adherence. Muscle function, aerobic capacity and muscle strength were assessed with standardized exercise tests. Muscle metabolism was assessed using a 31-Phosphorus Magnetic Resonance Spectroscopy protocol. Fatigue, physical activity and quality of life were assessed by questionnaires. Statistical significance was estimated using a randomization (permutation) test. Changes in outcome measures taken at baseline and end-of-study were calculated using paired t tests. RESULTS: Median (range) adherence to the study drug was 88.5% (20.5-95.5%) and the proportion of subjects with 80% adherence or higher was 76.9%. There were no missed study visits. There were no statistically significant changes in muscle function, strength, aerobic capacity, disease activity, fatigue, physical activity or quality of life while subjects were on creatine compared to placebo. There were statistically significant adaptations in muscle metabolism (e.g., decrease in change in muscle pH following exercise, and decrease in Phosphate/Phosphocreatine ratio) at the end-of-study, compared to baseline. There were no significant adverse effects. CONCLUSION: Creatine supplementation in children with JDM is feasible to study, and is safe and well-tolerated; it may lead to improvements in muscle metabolism.

4.
Arthritis Res Ther ; 22(1): 53, 2020 03 19.
Artigo em Inglês | MEDLINE | ID: mdl-32192528

RESUMO

BACKGROUND: The evaluation of quality of care in juvenile idiopathic arthritis (JIA) is critical for advancing patient outcomes but is not currently part of routine care across all centers in Canada. The study objective is to review the current landscape of JIA quality measures and use expert panel consensus to define key performance indicators (KPIs) that are important and feasible to collect for routine monitoring in JIA care in Canada. METHODS: Thirty-seven candidate KPIs identified from a systematic review were reviewed for inclusion by a working group including 3 pediatric rheumatologists. A shortlist of 14 KPIs was then assessed using a 3-round modified Delphi panel based on the RAND/UCLA Appropriateness Method. Ten panelists across Canada participated based on their expertise in JIA, quality measurement, or lived experience as a parent of a child with JIA. During rounds 1 and 3, panelists rated each KPI on a 1-9 Likert scale on themes of importance, feasibility, and priority. In round 2, panelists participated in a moderated in-person discussion that resulted in minor modifications to some KPIs. KPIs with median scores of ≥ 7 on all 3 questions without disagreement were included in the framework. RESULTS: Ten KPIs met the criteria for inclusion after round 3. Five KPIs addressed patient assessments: pain, joint count, functional status, global assessment of disease activity, and the clinical Juvenile Arthritis Disease Activity Score (cJADAS). Three KPIs examined access to care: wait times for consultation, access to pediatric rheumatologists within 1 year of diagnosis, and frequency of clinical follow-up. Safety was addressed through KPIs on tuberculous screening and laboratory monitoring. KPIs examining functional status using the Childhood Health Assessment Questionnaire (CHAQ), quality of life, uveitis, and patient satisfaction were excluded due to concerns about feasibility of measurement. CONCLUSIONS: The proposed KPIs build upon existing KPIs and address important processes of care that should be measured to improve the quality of JIA care. The feasibility of capturing these measures will be tested in various data sources including the Understanding Childhood Arthritis Network (UCAN) studies. Subsequent work should focus on development of meaningful outcome KPIs to drive JIA quality improvement in Canada and beyond.

7.
ACR Open Rheumatol ; 2(3): 138-146, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31997575

RESUMO

OBJECTIVE: To identify barriers and facilitators to the uptake of information from research by parents of children with juvenile idiopathic arthritis (JIA). METHODS: Parents of children with JIA participated in focus group and telephone interviews at four Canadian pediatric rheumatology centers. The semistructured interviews focused on perceptions about JIA research, how new information about JIA was obtained and used, and what information was of most interest. Transcripts were analyzed using a general inductive approach. RESULTS: Twenty-eight parents participated in the study. Parents were very interested in research that addresses the outcomes of JIA and side effects of medications. Parents communicated an expectation that information from research be communicated to them by their child's pediatric rheumatologist as part of clinical care. Parents felt that it would be helpful to have information available to them in a variety of formats including written, video, and online. The timing of information delivery is an important factor, with parents being most interested and engaged in learning about new information about JIA at diagnosis and disease flares. We found that parents were overall unaware of new findings from JIA research and therefore may not be optimally utilizing this potentially helpful information in the care of their children. CONCLUSION: This study has led to an understanding of Canadian parents' perceptions about research and existing gaps in the translation of research knowledge. This information will facilitate the development, implementation, and evaluation of future knowledge translation interventions aimed at improving the uptake of research information in the care of children with JIA.

8.
Rheumatology (Oxford) ; 59(1): 90-98, 2020 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-31236574

RESUMO

OBJECTIVE: LN is one of the most common and severe manifestations of SLE. Our aim was to test the association of SLE risk loci with LN risk in childhood-onset SLE (cSLE) and adult-onset SLE (aSLE). METHODS: Two Toronto-based tertiary care SLE cohorts included cSLE (diagnosed <18 years) and aSLE patients (diagnosed ⩾18 years). Patients met ACR and/or SLICC SLE criteria and were genotyped on the Illumina Multi-Ethnic Global Array or Omni1-Quad arrays. We identified those with and without biopsy-confirmed LN. HLA and non-HLA additive SLE risk-weighted genetic risk scores (GRSs) were tested for association with LN risk in logistic models, stratified by cSLE/aSLE and ancestry. Stratified effect estimates were meta-analysed. RESULTS: Of 1237 participants, 572 had cSLE (41% with LN) and 665 had aSLE (30% with LN). Increasing non-HLA GRS was significantly associated with increased LN risk [odds ratio (OR) = 1.26; 95% CI 1.09, 1.46; P = 0.0006], as was increasing HLA GRS in Europeans (OR = 1.55; 95% CI 1.07, 2.25; P = 0.03). There was a trend for stronger associations between both GRSs and LN risk in Europeans with cSLE compared with aSLE. When restricting cases to proliferative LN, the magnitude of these associations increased for both the non-HLA (OR = 1.30; 95% CI 1.10, 1.52; P = 0.002) and HLA GRS (OR = 1.99; 95% CI 1.29, 3.08; P = 0.002). CONCLUSION: We observed an association between known SLE risk loci and LN risk in children and adults with SLE, with the strongest effect observed among Europeans with cSLE. Future studies will include SLE-risk single nucleotide polymorphisms specific to non-European ancestral groups and validate findings in an independent cohort.


Assuntos
Idade de Início , Loci Gênicos/genética , Predisposição Genética para Doença/genética , Lúpus Eritematoso Sistêmico/genética , Nefrite Lúpica/genética , Adolescente , Adulto , Criança , Grupo com Ancestrais do Continente Europeu/genética , Feminino , Predisposição Genética para Doença/etnologia , Genótipo , Humanos , Modelos Logísticos , Lúpus Eritematoso Sistêmico/etnologia , Nefrite Lúpica/etnologia , Masculino , Razão de Chances , Polimorfismo de Nucleotídeo Único/genética , Fatores de Risco , Adulto Jovem
9.
Paediatr Child Health ; 24(2): 85-91, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30996598

RESUMO

Since first defined in 1998, paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) and its later, broader iteration, paediatric acute-onset neuropsychiatric syndrome (PANS), have garnered significant attention and controversy. The role of streptococcal infection in children with explosive onset obsessive-compulsive disorder and new onset tics, the natural history of this entity, and the role of symptomatic and disease-modifying therapies, including antibiotics, immunotherapy, and psychoactive drugs, are all issues that have yet to be definitively addressed. While definitive proof of the autoimmune hypothesis of PANDAS is lacking, given the heightened attention to this entity and apparent rise in use of this diagnostic category, addressing questions around diagnosis, treatment, and etiology is imperative. In this paper, we review current working definitions of PANDAS/PANS, discuss published evidence for interventions related to this entity, and propose a clinical approach to children presenting with acute symptoms satisfying criteria for PANDAS/PANS.

10.
Artigo em Inglês | MEDLINE | ID: mdl-30805629

RESUMO

OBJECTIVE: Childhood-onset SLE (cSLE) manifests differently than adult-onset SLE (aSLE). This study determined whether ethnic differences contribute to the differences in clinical presentation between the two groups. METHODS: This cross-sectional study used data from a multi-centred registry from eight adult and four paediatric Canadian centres gathered at study entry. We compared the frequency of clinical manifestations and autoantibodies between aSLE and cSLE. For those with a significant difference, a multivariable logistic regression was performed, adjusting for ethnicity, SLE onset (cSLE vs aSLE), disease duration and centre. Disease activity and damage between aSLE and cSLE were compared after stratifying by disease duration. RESULTS: Of 552 aSLE subjects, 502 (90.9%) were female and 381 (69.0%) were Caucasian. Mean age at diagnosis was 37.0 ± 13.6 years and disease duration 10.9 ± 9.6 years. Of 276 cSLE subjects, 231 (83.7%) were female and 101 (36.6%) were Caucasian. Mean age at diagnosis was 12.7 ± 3.3 years and disease duration 5.6 ± 8.2 years. In multivariable regression analysis, aSLE was associated with decreased odds of having a neurologic disorder (odds ratio = 0.49) and increased odds of having aCL antibodies (odds ratio = 1.85). Disease activity and damage accrual scores were higher in aSLE than cSLE within the same disease duration strata, although the differences were not clinically significant. Ethnicity was not associated with any differences in clinical manifestations or autoantibody frequency between aSLE and cSLE. CONCLUSIONS: Although a crude comparison of aSLE and cSLE yielded several differences in clinical symptoms and autoantibodies, this difference was not attributable to ethnic differences between aSLE and cSLE.

11.
J Rheumatol ; 46(7): 731-738, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-30770510

RESUMO

OBJECTIVE: Currently there are 2 different classification criteria for systemic lupus erythematosus (SLE): American College of Rheumatology (ACR) and Systemic Lupus International Collaborating Clinics (SLICC). The aim of this study was to compare the sensitivities of ACR and SLICC criteria in childhood-onset SLE (cSLE) using a large, multiethnic cohort. METHODS: We conducted a retrospective study of 722 patients diagnosed with cSLE at The Hospital for Sick Children (SickKids). Prospectively collected data from SickKids' Lupus Database were reviewed/validated against medical records prior to ACR and SLICC scoring based on cumulative symptoms up to the last visit. Sensitivities were compared using McNemar's test. Descriptive statistics were used to identify SLE features unique to each set of criteria and autoantibodies not included in either. RESULTS: ACR and SLICC sensitivities were as follows: 92.4% and 96.3% overall (p = 0.001); 82.5% and 91.3% (p = 0.01) in those scored ≤ 1 year from diagnosis; 92.7% and 97.9% (p = 0.02) in those scored 2-3 years from diagnosis. Forty-eight of 55 (87.3%) patients who did not meet ACR criteria met SLICC criteria through SLICC-specific criterion or renal biopsy. Twenty of 27 (74.1%) patients who did not meet SLICC criteria met ACR criteria as a result of photosensitivity (73.9%) and ACR lymphopenia criteria (26.1%). Six of 7 patients (85.7%) who were clinically diagnosed with cSLE but did not meet either SLICC or ACR criteria had anti-Ro antibodies. CONCLUSION: SLICC criteria were significantly more sensitive than ACR criteria in cSLE classification, especially early in the disease course. Because of the extreme rarity of primary Sjögren syndrome in children, one may consider adding anti-Ro antibodies to the classification criteria for cSLE because they are present in ∼40% of patents with cSLE.


Assuntos
Lúpus Eritematoso Sistêmico/classificação , Lúpus Eritematoso Sistêmico/imunologia , Projetos de Pesquisa , Adolescente , Anticorpos Antinucleares/sangue , Biópsia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Cooperação Internacional , Rim/patologia , Leucopenia , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/patologia , Linfopenia , Masculino , Estudos Prospectivos , Estudos Retrospectivos , Sensibilidade e Especificidade , Estados Unidos
12.
Arthritis Care Res (Hoboken) ; 71(5): 579-590, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30680946

RESUMO

OBJECTIVE: To develop a Childhood Lupus Improvement Index (CHILI) as a tool to measure response to therapy in childhood-onset systemic lupus erythematosus (cSLE), with a focus on clinically relevant improvement (CRIc SLE ). METHODS: Pediatric nephrology and rheumatology subspecialists (n = 213) experienced in cSLE management were invited to define CRIc SLE and rate a total of 433 unique patient profiles for the presence/absence of CRIc SLE . Patient profiles included the following cSLE core response variables (CRVs): global assessment of patient well-being (patient-global), physician assessment of cSLE activity (MD-global), disease activity index score (here, we used the Systemic Lupus Erythematosus Disease Activity Index), urine protein-to-creatinine ratio, and Child Health Questionnaire physical summary score. Percentage and absolute changes in these cSLE-CRVs (baseline versus follow-up) were considered in order to develop candidate algorithms and validate their performance (sensitivity, specificity, area under the receiver operating characteristic curve [AUC]; range 0-1). RESULTS: During an international consensus conference, unanimous agreement on a definition of CRIc SLE was achieved; cSLE experts (n = 13) concurred (100%) that the preferred CHILI algorithm considers absolute changes in the cSLE-CRVs. After transformation to a range of 0-100, a CHILI score of ≥54 had outstanding accuracy for identifying CRIc SLE (AUC 0.93, sensitivity 81.1%, and specificity 84.2%). CHILI scores also reflect minor, moderate, and major improvement for values exceeding 15, 68, and 92, respectively (all AUC ≥0.92, sensitivity ≥93.1%, and specificity ≥73.4%). CONCLUSION: The CHILI is a new, seemingly highly accurate index for measuring CRI in cSLE over time. This index is useful to categorize the degree of response to therapy in children and adolescents with cSLE.


Assuntos
Antirreumáticos/uso terapêutico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Avaliação de Resultados em Cuidados de Saúde/métodos , Índice de Gravidade de Doença , Adolescente , Algoritmos , Criança , Técnica Delfos , Humanos
13.
Arthritis Rheumatol ; 71(3): 411-419, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30225865

RESUMO

OBJECTIVE: End points currently used in lupus nephritis (LN) clinical trials lack uniformity and questionably reflect long-term kidney survival. This study was undertaken to identify short-term end points that predict long-term kidney outcomes for use in clinical trials. METHODS: A database of 944 patients with LN was assembled from 3 clinical trials and 12 longitudinal cohorts. Variables from the first 12 months of treatment after diagnosis of active LN (prediction period) were assessed as potential predictors of long-term outcomes in a 36-month follow-up period. The long-term outcomes examined were new or progressive chronic kidney disease (CKD), severe kidney injury (SKI), and the need for permanent renal replacement therapy (RRT). To predict the risk for each outcome, hazard index tools (HITs) were derived using multivariable analysis with Cox proportional hazards regression. RESULTS: Among 550 eligible subjects, 54 CKD, 55 SKI, and 22 RRT events occurred. Variables in the final CKD HIT were prediction-period CKD status, 12-month proteinuria, and 12-month serum creatinine level. The SKI HIT variables included prediction-period CKD status, International Society of Nephrology (ISN)/Renal Pathology Society (RPS) class, 12-month proteinuria, 12-month serum creatinine level, race, and an interaction between ISN/RPS class and 12-month proteinuria. The RRT HIT included age at diagnosis, 12-month proteinuria, and 12-month serum creatinine level. Each HIT validated well internally (c-indices 0.84-0.92) and in an independent LN cohort (c-indices 0.89-0.92). CONCLUSION: HITs, derived from short-term kidney responses to treatment, correlate with long-term kidney outcomes, and now must be validated as surrogate end points for LN clinical trials.


Assuntos
Biomarcadores/análise , Nefrite Lúpica/mortalidade , Insuficiência Renal Crônica/mortalidade , Terapia de Substituição Renal/mortalidade , Índice de Gravidade de Doença , Lesão Renal Aguda/mortalidade , Lesão Renal Aguda/terapia , Adulto , Fatores Etários , Ensaios Clínicos como Assunto , Creatinina/sangue , Bases de Dados Factuais , Feminino , Humanos , Estudos Longitudinais , Nefrite Lúpica/terapia , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Valor Preditivo dos Testes , Modelos de Riscos Proporcionais , Proteinúria/urina , Insuficiência Renal Crônica/terapia , Reprodutibilidade dos Testes
14.
J Rheumatol ; 46(3): 294-300, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30442824

RESUMO

OBJECTIVE: To determine barriers and facilitators to the uptake of findings from the Research in Arthritis in Canadian Children emphasizing Outcomes (ReACCh-Out) study into clinical care by pediatric rheumatologists (PR) and allied health professionals (AHP) caring for children with juvenile idiopathic arthritis (JIA) in Canada. METHODS: PR and AHP participated in this qualitative study through telephone interviews. Interview guides were developed using the Theoretical Domains Framework and focused on the use of information from the ReACCh-Out study in the practice of counseling patients and families. A directed content analysis approach was used for coding. RESULTS: Nineteen interviews (8 PR and 11 AHP) were completed. All PR had knowledge of the ReACCh-Out study. Three major themes were identified: (1) both groups are motivated to use information from research in clinical care; (2) volume and emotional effect of information on families are barriers; and (3) specific timepoints in care trigger providing this information. AHP had less knowledge of the ReACCh-Out study, did not feel it was their primary role to provide this information, and have a desire for more opportunity to participate in academic forums related to research. CONCLUSION: We have described a comprehensive overview of the barriers and facilitators perceived by healthcare providers in the translation of knowledge from JIA research into use in clinical practice. These findings provide a foundation for the development of knowledge translation strategies in the care of children with JIA and other rheumatic diseases.


Assuntos
Pessoal Técnico de Saúde/psicologia , Artrite Juvenil/psicologia , Conhecimentos, Atitudes e Prática em Saúde , Avaliação de Resultados em Cuidados de Saúde , Reumatologistas/psicologia , Pesquisa Médica Translacional , Adolescente , Canadá , Criança , Barreiras de Comunicação , Feminino , Humanos , Entrevistas como Assunto , Masculino , Pesquisa Qualitativa
15.
Arthritis Care Res (Hoboken) ; 70(6): 813-822, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29693328

RESUMO

OBJECTIVE: To validate the preliminary criteria of global flare for childhood-onset SLE (cSLE). METHODS: Pediatricians experienced in cSLE care (n = 268) rated unique patient profiles; results of standard cSLE laboratory testing and information about the cSLE flare descriptors were presented as follows: global assessment of patient well-being, physician global assessment of disease activity (MD-global), Disease Activity Index score, protein/creatinine ratio (PCR), and erythrocyte sedimentation rate (ESR). Using rater interpretation of the course of cSLE (baseline versus followup as the gold standard), performance (sensitivity, specificity, area under the receiver operating characteristic curve [AUC]) of the preliminary flare criteria was tested. An international consensus conference was held to rank the preliminary flare criteria as per the American College of Rheumatology recommendations and delineate threshold scores for minor, moderate, and major flares. RESULTS: The accuracy of the 2 highest-ranked candidate criteria that consider absolute changes (∆) of the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) or British Isles Lupus Assessment Group (BILAG) (numeric scoring: A = 12, B = 8, C = 1, and D/E = 0), MD-global, PCR, and ESR were confirmed (both AUC >0.93). For the SLEDAI-based criteria (0.5 × ∆SLEDAI + 0.45 × ∆PCR + 0.5 × ∆MD-global + 0.02 × ∆ESR) flare scores ≥6.4/3.0/0.6 constituted major/moderate/minor flares, respectively. For the BILAG-based algorithm (0.4 × ∆BILAG + 0.65 × ∆PCR + 0.5 × ∆MD-global + 0.02 × ∆ESR) flare scores ≥7.4/3.7/2.2 delineated major/moderator/minor flares, respectively. These threshold values (SLEDAI, BILAG) were all >82% sensitive and specific for capturing flare severity. CONCLUSION: Provisional criteria for global flares in cSLE are available to identify patients who experienced a flare. These criteria also allow for discrimination of the severity of cSLE exacerbations.


Assuntos
Lúpus Eritematoso Sistêmico , Exacerbação dos Sintomas , Adolescente , Algoritmos , Estudos de Coortes , Feminino , Humanos , Masculino
16.
Arthritis Rheumatol ; 70(4): 616-624, 2018 04.
Artigo em Inglês | MEDLINE | ID: mdl-29342508

RESUMO

OBJECTIVE: To describe the features and treatment of macrophage activation syndrome (MAS) in a single-center cohort of patients with childhood-onset systemic lupus erythematosus (SLE), and to compare childhood-onset SLE manifestations and outcomes between those with and those without MAS. METHODS: We included all patients with childhood-onset SLE followed up at The Hospital for Sick Children from 2002 to 2012, and identified those also diagnosed as having MAS. Demographic, clinical, and laboratory features of MAS and SLE, medication use, hospital and pediatric intensive care unit (PICU) admissions, as well as damage indices and mortality data were extracted from the Lupus database. Student's t-tests and Fisher's exact tests were used to compare continuous and categorical variables, respectively. We calculated incidence rate ratios of hospital and PICU admissions comparing patients with and those without MAS, using Poisson models. Kaplan-Meier survival analysis was used to examine the time to disease damage accrual. RESULTS: Of the 403 patients with childhood-onset SLE, 38 (9%) had MAS. The majority (68%) had concomitant MAS and SLE diagnoses. Fever was the most common MAS clinical feature. The frequency of renal and central nervous system disease, hospital admissions, the average daily dose of steroids, and time to disease damage were similar between those with and those without MAS. We observed a higher mortality rate among those with MAS (5%) than those without MAS (0.2%) (P = 0.02). CONCLUSION: MAS was most likely to develop concomitantly with childhood-onset SLE diagnosis. The majority of the MAS patients were successfully treated with corticosteroids with no MAS relapses. Although the numbers were small, there was a higher risk of death associated with MAS compared to SLE without MAS.


Assuntos
Lúpus Eritematoso Sistêmico/imunologia , Síndrome de Ativação Macrofágica/mortalidade , Adolescente , Corticosteroides/uso terapêutico , Idade de Início , Criança , Feminino , Hospitalização/estatística & dados numéricos , Humanos , Unidades de Terapia Intensiva Pediátrica/estatística & dados numéricos , Estimativa de Kaplan-Meier , Síndrome de Ativação Macrofágica/tratamento farmacológico , Síndrome de Ativação Macrofágica/imunologia , Masculino , Estudos Prospectivos
17.
Arthritis Care Res (Hoboken) ; 70(1): 134-144, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-28320056

RESUMO

OBJECTIVE: To describe changes in health-related quality of life (HRQoL) over time in children with juvenile idiopathic arthritis (JIA), relative to other outcomes, and to identify predictors of unfavorable HRQoL trajectories. METHODS: Children with JIA in the Research in Arthritis in Canadian Children emphasizing Outcomes (ReACCh-Out) cohort were included. The Juvenile Arthritis Quality of Life Questionnaire (JAQQ, a standardized instrument), health-related Quality of My Life (HRQoML, an instrument based on personal valuations), and JIA core variables were completed serially. Analyses included median values, Kaplan-Meier survival curves, and latent trajectory analysis. RESULTS: A total of 1,249 patients enrolled at a median of 0.5 months after diagnosis were followed for a median of 34.2 months. The degree of initial HRQoL impairment and probabilities of reaching the best possible HRQoL scores varied across JIA categories (best for oligoarthritis, worst for rheumatoid factor-positive polyarthritis). Median times to attain best possible HRQoL scores (JAQQ 59.3 months, HRQoML 34.5 months), lagged behind those for disease activity, pain, and disability measures. Most patients followed trajectories with minimal or mild impairment; however, 7.6% and 13.8% of patients, respectively, followed JAQQ and HRQoML trajectories with persistent major impairment in HRQoL. JIA category, aboriginal ethnicity, and baseline disease activity measures distinguished between membership in trajectories with major and minimal impairments. CONCLUSION: Improvement in HRQoL is slower than for disease activity, pain, and disability. Improvement of a measure based on respondents' preferences (HRQoML) is more rapid than that of a standardized measure (JAQQ). Higher disease activity at diagnosis heralds an unfavorable HRQoL trajectory.


Assuntos
Comportamento do Adolescente , Artrite Juvenil/diagnóstico , Artrite Juvenil/psicologia , Comportamento Infantil , Qualidade de Vida , Inquéritos e Questionários , Adolescente , Desenvolvimento do Adolescente , Fatores Etários , Artrite Juvenil/fisiopatologia , Artrite Juvenil/terapia , Canadá , Criança , Desenvolvimento Infantil , Pré-Escolar , Avaliação da Deficiência , Feminino , Humanos , Estudos Longitudinais , Masculino , Medição da Dor , Valor Preditivo dos Testes , Prognóstico , Fatores de Tempo
18.
Pain ; 159(1): 57-66, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-28937578

RESUMO

We studied children enrolled within 90 days of juvenile idiopathic arthritis diagnosis in the Research in Arthritis in Canadian Children Emphasizing Outcomes (ReACCh-Out) prospective inception cohort to identify longitudinal trajectories of pain severity and features that may predict pain trajectory at diagnosis. A total of 1062 participants were followed a median of 24.3 months (interquartile range = 16.0-37.1 months). Latent trajectory analysis of pain severity, measured in a 100-mm visual analogue scale, identified 5 distinct trajectories: (1) mild-decreasing pain (56.2% of the cohort); (2) moderate-decreasing pain (28.6%); (3) chronically moderate pain (7.4%); (4) minimal pain (4.0%); and (5) mild-increasing pain (3.7%). Mean disability and quality of life scores roughly paralleled the pain severity trajectories. At baseline, children with chronically moderate pain, compared to those with moderate-decreasing pain, were older (mean 10.0 vs 8.5 years, P = 0.01) and had higher active joint counts (mean 10.0 vs 7.2 joints, P = 0.06). Children with mild-increasing pain had lower joint counts than children with mild-decreasing pain (2.3 vs 5.2 joints, P < 0.001). Although most children with juvenile idiopathic arthritis in this cohort had mild or moderate initial levels of pain that decreased quickly, about 1 in 10 children had concerning pain trajectories (chronically moderate pain and mild-increasing pain). Systematic periodic assessment of pain severity in the months after diagnosis may help identify these concerning pain trajectories early and lay out appropriate pain management plans. Focused research into the factors leading to these concerning trajectories may help prevent them.


Assuntos
Artrite Juvenil/diagnóstico , Artrite Juvenil/fisiopatologia , Qualidade de Vida , Adolescente , Criança , Pré-Escolar , Avaliação da Deficiência , Progressão da Doença , Feminino , Humanos , Masculino , Dor , Medição da Dor , Índice de Gravidade de Doença
19.
J Rheumatol ; 44(11): 1619-1623, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-28916546

RESUMO

OBJECTIVE: To describe treatment practices for childhood pure membranous lupus nephritis (MLN). METHODS: Survey study of Childhood Arthritis and Rheumatology Research Alliance and American Society of Pediatric Nephrology members. RESULTS: There were 117 respondents who completed the survey (60 pediatric nephrologists, 57 pediatric rheumatologists). Steroids and nonsteroid immunosuppression (NSI) were routinely used by the majority for MLN. Mycophenolate mofetil was the favored initial NSI. Nephrologists used steroids (60% vs 93%) and NSI (53% vs 87%) less often than did rheumatologists for MLN without nephrotic syndrome (NS). CONCLUSION: Pediatric rheumatologists and nephrologists both recommend steroids and NSI for children with MLN, with or without NS.


Assuntos
Imunossupressores/uso terapêutico , Nefrite Lúpica/tratamento farmacológico , Ácido Micofenólico/uso terapêutico , Padrões de Prática Médica , Criança , Feminino , Pesquisas sobre Serviços de Saúde , Humanos , Masculino , Nefrologistas , Pediatras , Reumatologistas
20.
Pediatr Rheumatol Online J ; 15(1): 68, 2017 Aug 22.
Artigo em Inglês | MEDLINE | ID: mdl-28830457

RESUMO

BACKGROUND: With modern treatments, the effect of juvenile idiopathic arthritis (JIA) on growth may be less than previously reported. Our objective was to describe height, weight and body mass index (BMI) development in a contemporary JIA inception cohort. METHODS: Canadian children newly-diagnosed with JIA 2005-2010 had weight and height measurements every 6 months for 2 years, then yearly up to 5 years. These measurements were used to calculate mean age- and sex-standardized Z-scores, and estimate prevalence and cumulative incidence of growth impairments, and the impact of disease activity and corticosteroids on growth. RESULTS: One thousand one hundred forty seven children were followed for median 35.5 months. Mean Z-scores, and the point prevalence of short stature (height < 2.5th percentile, 2.5% to 3.4%) and obesity (BMI > 95th percentile, 15.8% to 16.4%) remained unchanged in the whole cohort. Thirty-three children (2.9%) developed new-onset short stature, while 27 (2.4%) developed tall stature (>97.5th percentile). Children with systemic arthritis (n = 77) had an estimated 3-year cumulative incidence of 9.3% (95%CI: 4.3-19.7) for new-onset short stature and 34.4% (23-49.4) for obesity. Most children (81.7%) received no systemic corticosteroids, but 1 mg/Kg/day prednisone-equivalent maintained for 6 months corresponded to a drop of 0.64 height Z-scores (0.56-0.82) and an increase of 0.74 BMI Z-scores (0.56-0.92). An increase of 1 in the 10-cm physician global assessment of disease activity maintained for 6 months corresponded to a drop of 0.01 height Z-scores (0-0.02). CONCLUSIONS: Most children in this modern JIA cohort grew and gained weight as children in the general population. About 1 in 10 children who had systemic arthritis, uncontrolled disease and/or prolonged corticosteroid use, had increased risk of growth impairment.


Assuntos
Artrite Juvenil/complicações , Glucocorticoides/efeitos adversos , Transtornos do Crescimento/epidemiologia , Ganho de Peso/efeitos dos fármacos , Adolescente , Antropometria , Artrite Juvenil/tratamento farmacológico , Canadá/epidemiologia , Criança , Pré-Escolar , Feminino , Seguimentos , Glucocorticoides/uso terapêutico , Transtornos do Crescimento/etiologia , Humanos , Incidência , Masculino , Prevalência , Estudos Prospectivos
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