Updates in diagnosis and treatment of pediatric antiphospholipid syndrome.

PURPOSE OF REVIEW: This review offers an overview of the most important recent articles on pediatric APS. RECENT FINDINGS: Non-thrombotic extra criteria manifestations were prevalent in pediatric APS. Pregnancy morbidity has been described as the first manifestation of APS at youth age, impairing gestational outcomes. The 2023 APS criteria were developed for adult APS patients, and there is still a lack of pediatric-specific APS criteria. Catastrophic APS was more commonly reported as the initial manifestation of pediatric APS than in adults. Regarding treatment, direct oral anticoagulants have been recently approval for pediatric patients with venous thrombosis. New approaches have been proposed for severe cases, for arterial thrombosis, and rituximab for refractory cases. Recurrences typically occurred early and were associated with older age at diagnosis. Current studies highlighted the multifaceted nature of pediatric APS. Further large prospective multicenter studies evaluating new medications capable of reducing recurrence risk and improving prognosis in this population will be required.

Engaging women to set the research agenda for assisted vaginal birth.

INTRODUCTION: Public and patient involvement can provide crucial insights to optimise research by enhancing relevance and appropriateness of studies. The World Health Organization (WHO) engaged in an inclusive process to ensure that both technical experts and women had a voice in defining the research gaps and needs to increase or reintroduce the use of assisted vaginal birth (AVB) in settings where this intervention is needed but unavailable or underused. METHODS: We describe the methods and outcomes of online workshops led by WHO to obtain women representatives' perspectives about AVB research gaps and needs. RESULTS: After technical experts created a list of research questions based on various evidence syntheses, WHO organised four online workshops with 31 women's representatives from 27 mostly low- and middle-income (LMIC) countries. Women rated the importance and priority of the research questions proposed by the technical experts, improving and broadening some of them, added new questions, and voiced their main concerns and views about AVB. Women helped to put the research questions into context in their communities, highlighted neglected factors/dimensions that influence practices and affect women's experience during labour and childbirth, underscored less salient consequences of AVB, and highlighted the main concerns of women about research on AVB. The consolidated vision of technical experts and women's representatives resulted in a technical brief published by WHO. The technical brief is expected to stimulate global research and action closely aligned with women's priorities. CONCLUSIONS: We describe a successful experience of engaging women, mostly from LMICs, in the identification of research gaps and needs to reintroduce AVB use. This process contributed to better aligning research questions with women's views, concerns, and priorities. Given the scarcity of reports about engaging women from LMICs to optimise research, this successful experience can serve as an inspiration for future work. PATIENT OR PUBLIC CONTRIBUTION: Women representatives were involved at every stage of the workshops described in full in this manuscript.

Risk factors for mortality in 1528 Brazilian childhood-onset systemic lupus erythematosus patients.

OBJECTIVES: To identify associations between mortality in cSLE patients and their characteristics: clinical and laboratory features, disease activity and damage scores, and treatment; to evaluate risk factors associated with mortality in cSLE; and to determine the most frequent causes of death in this group of patients. METHODS: We performed a multicenter retrospective cohort using data from 1,528 cSLE patients followed in 27 pediatric rheumatology tertiary centers in Brazil. Patients' medical records were reviewed according to a standardized protocol, in which information regarding demographic and clinical features, disease activity and damage scores, and treatment were collected and compared between deceased cSLE patients and survivors. Univariate and multivariate analyses by Cox regression model were used to calculate risk factors for mortality, whereas survival rates were analyzed by Kaplan-Meier plots. RESULTS: A total of 63/1,528 (4.1%) patients deceased, 53/63 were female (84.1%), median age at death was 11.9 (9.4-13.1) years and median time interval between cSLE diagnosis and death was 3.2 (0.5-5.3) years. Sepsis was the main cause of death in 27/63 (42.8%) patients, followed by opportunistic infections in 7/63 (11.1%), and alveolar hemorrhage in 6/63 (9.5%) patients. The regression models resulted in neuropsychiatric lupus (NP-SLE) (HR = 2.56, 95% CI = 1.48-4.42) and chronic kidney disease (CKD) (HR = 4.33, 95% CI = 2.33-4.72), as risk factors significantly associated with mortality. Overall patient survival after cSLE diagnosis at 5, 10, and 15 years were 97%, 95.4%, and 93.8%, respectively. CONCLUSIONS: This study confirmed that the recent mortality rate in cSLE in Brazil is low, but still of concern. NP-SLE and CKD were the main risk factors for mortality, indicating that the magnitude of these manifestations was significantly high.

Promoting plant-based eating in meat-centric meal contexts: a field study.

OBJECTIVE: Shifting from meat-centric to plant-rich diets may help to enable healthier and more sustainable food systems. Here we present the results of a 1-week intervention to promote plant-based eating in a meat-centric food context (i.e. canteen). DESIGN: The intervention included environmental restructuring strategies (e.g. promotional materials and menu redevelopment) and improvements to the offer of plant-based meals. The evaluation (sales data; pre-registered) spanned 3 weeks prior to the intervention (baseline), 1 week during the intervention (immediate/short-term impact) and 3 weeks after the intervention (follow-up). Opinion surveys were also used to collect data with customers during the intervention. SETTING: Canteen unit of a university campus in Portugal (Lisbon metropolitan area). PARTICIPANTS: In addition to sales data (baseline: 7965 meals; immediate/short-term: 2635 meals; follow-up: 7135 meals), we used opinion surveys to assess customers' meal appraisals during the intervention (n = 370). RESULTS: The odds of a sold meal being vegetarian were 24 % higher in the intervention week compared with the pre-intervention period [OR = 1·24, 95 % CI (1·10, 1·40)] and 9 % higher in the post-intervention period compared with the pre-intervention period [OR = 1·09, (95 % CI (1·00, 1·19)]. Survey data showed that vegetarian meals compared favourably to meat and fish alternatives in liking, sustainability and satiety. CONCLUSIONS: A short-term, theory-driven, operationally feasible intervention was effective in promoting increased plant-based meal choices in a collective meal context. Nevertheless, these changes were not entirely sustained over time. Future studies could test whether prolonged or more transformative interventions are necessary to unlock entrenched food practices more effectively in meat-centric collective meal contexts.

Insights into parents' and teachers' support for policies promoting increased plant-based eating in schools.

Global environmental and public health challenges related to current food systems call for large-scale shifts towards increasingly plant-based diets, especially in Western meat-centric societies. School meal systems can play a role in these changes due to their widespread prevalence and multi-sectoral impact. However, there is a lack of evidence about how adults involved in the school meals system perceive school-based pro-environmental food policies, which limits the ability to align those policies with the needs and expectations of the school community. This study aimed to address this knowledge gap by exploring parents' (n = 104) and teachers' (n = 252) support for policies to promote increased plant-based eating in public schools in a highly meat-centric EU country (Portugal). Overall, teachers seemed to be slightly more supportive of such policies and displayed more favorable (injunctive and dynamic) norms toward plant-based eating, more negative appraisals of meals with meat (i.e., perceived healthiness, naturalness, and sustainability), and lower attachment to meat consumption. Furthermore, injunctive norms in favor of plant-based meals were linked with higher support for measures promoting plant-based meals in schools, in both samples (parents, teachers). Lower meat attachment and favorable perceived meal attributes (e.g., perceptions about plant-based and fish meals) were associated with teachers' support for measures promoting plant-based meals in schools. These findings suggest that future efforts and research with parents and teachers to enable less meat-centric and more flexitarian food practices in schools should consider social and motivation variables relevant to plant-forward transitions.

How to enable healthier and more sustainable food practices in collective meal contexts: A scoping review.

Collective meal contexts such as restaurants, cafeterias and canteens can help accelerate transitions to healthier and more sustainable diets. However, evidence from intervention studies on these contexts lacks integration. This scoping review aimed to map determinants of dietary change in collective meal contexts across multiple settings, interventions, target groups, and target behaviors. The review provided two main outcomes: (i) identifying intervention components to promote dietary change in collective meal contexts, based on the existing body of evidence; and (ii) classifying and integrating these intervention components into an overarching framework of behavior change (i.e., COM-B system). The review encompassed twenty-eight databases via two indexing services and extracted information from 232 primary sources (27,458 records selected for title and abstract screening, 574 articles selected for full-text screening). We identified a total of 653 intervention activities, which were classified into intervention components and grouped under three broad themes, namely contextual and environmental changes, social influence, and knowledge and behavioral regulation. Multi-component interventions tended to report overall positive outcomes. The review proposes several directions for future research, including: (i) moving toward more theory-based interventions in collective meal contexts; (ii) providing more detailed information about intervention settings, implementation, target groups, activities, and materials; and (iii) improving the use of open science practices in the field. Furthermore, the review offers a free, original, open-access list and synthesis of 277 intervention studies in collective meal contexts, which can help intervention planners and evaluators optimize their efforts to promote healthier and more sustainable food practices in these contexts.

Is positron emission tomography/magnetic resonance imaging a reliable tool for detecting vascular activity in treated childhood-onset Takayasu's arteritis? A multicentre study.

OBJECTIVES: Toassess whether 18F-fluordeoxiglucose (18F-FDG) PET/MRI) with angiographic sequences can contribute to detecting vessel wall inflammation in patients with childhood-onset Takayasu's arteritis (c-TA) under immunosuppressive therapy. METHODS: A three-centre cross-sectional study was conducted. 18F-FDG PET/MRI scans were performed in c-TA patients and in oncologic patients, who served as the control group. Clinical and laboratory characteristics were also analysed. RESULTS: Seventeen c-TA patients (65% females) between the ages of 6 and 21 years with a mean disease duration of 9.4 years were recruited. Only one patient presented clinical disease activity and six (35.6%) had increased ESR and/or CRP levels. The most frequent magnetic resonance angiography (MRA) findings were stenosis and thickening, observed in 82.4 and 70.6% of c-TA patients, respectively. 18F-FDG PET revealed 18F-FDG uptake greater than the liver in at least one arterial segment in 15 (88.2%) patients in a qualitative analysis and a median maximum standardized uptake value (SUVmax) of 3.22 (interquartile range 2.76-3.69) in a semi-quantitative analysis. c-TA patients presented significantly higher SUVmax values than oncologic patients (P < 0.001). A positive correlation between SUVmax and CRP levels (ρ = 0.528, P = 0.029) was seen. CONCLUSION: A state-of-the-art imaging modality was used in c-TA patients and revealed a strong arterial FDG uptake even in patients in apparent remission. We suppose that this finding may represent silent activity in the vessel wall; however, we cannot exclude the possibility of arterial remodelling. Importantly, a negative imaging scan may help in immunosuppression withdrawal in daily clinical practice.

Hydroxychloroquine blood levels predicts flare in childhood-onset lupus nephritis.

OBJECTIVE: Low hydroxychloroquine (HCQ) blood levels are predictors of flare in adult lupus. Childhood-onset systemic lupus erythematosus (cSLE) has high morbidity with renal involvement in up to 80% of cases. The aim of this study is to determine the HCQ cut-off levels which predicts flare in childhood-onset lupus nephritis (LN). METHODS: Sixty LN patients on HCQ use for at least 6-months were prospectively evaluated at baseline (BL) and about 6-months later for cSLE flare and HCQ blood levels (ng/mL) measured by liquid chromatography-tandem mass spectrometry. RESULTS: There were 19 patients (32%) with flare, during the study with median SLEDAI increase of 4 (0-8). Median (IQR) BL HCQ levels of the flare group were lower compared to stable patients [557.5 (68.6-980.3) vs. 1061.9 (534.8-1590.0 ng/mL); p=0.012]. ROC curve analysis demonstrated that HCQ levels≤1075 ng/mL were associated with a 5.08 (95%CI 1.28-20.13; p=0.021) times increased risk of flare. Six-month HCQ levels revealed that most patients 24/54 (44%) had persistently low levels (≤1075) during follow-up. Among those, 11/24 (46%) had flare. Multiple logistic regression analysis including prednisone use, baseline SLEDAI-2K, adherence based on pharmacy refill and BL HCQ blood levels as possible predictors of flare revealed that only HCQ blood level was independently associated with flare (OR 0.999, 95%CI 0.998-1.0, p=0.013). CONCLUSIONS: We demonstrated that HCQ blood cut-off level under 1075 ng/mL predicts flare in childhood-onset LN patients under prescribed HCQ dose of 4.0-5.5 mg/kg/day. We further observed that most of these patients have compliance issues reinforcing the need for a close surveillance particularly in those with levels below the defined cut-off.

A home-based exercise program during COVID-19 pandemic: Perceptions and acceptability of juvenile systemic lupus erythematosus and juvenile idiopathic arthritis adolescents.

OBJECTIVES: To investigate the perceptions and acceptability of a home-based exercise intervention in systemic lupus erythematosus (JSLE) and juvenile idiopathic arthritis (JIA) adolescent patients during the COVID-19 pandemic, and to explore the effects of the intervention on health-related quality of life (HRQoL), sleep quality, and mental health conditions parameters. METHODS: This was a randomized controlled trial of a 12-week, home-based exercise training program conducted between October and December 2020. During this period, social distancing measures were in place in Brazil to contain the spread of COVID-19. Adolescent patients diagnosed with JSLE and JIA participated in the study. Health-related qualitative and quantitative data were collected before and after the follow-up. RESULTS: 21 JSLE patients and 30 JIA patients were analyzed. Six themes emerged from patients' feedback: 1) Suitability of the home-based format; 2) Appropriate trainer supervision, 3) Motivators and facilitators for the program; 4) Barriers to the program; 5) Health benefits; 6) Patients' suggestions to improve the program. Overall, data indicated that the intervention showed good acceptability and elicited improvements in the perceived HRQoL and fatigue in JIA and JSLE patients during the pandemic. However, further quantitative analyses with validated HRQoL, sleep quality, and mental health conditions instruments did not capture these benefits (p>0.05). CONCLUSION: Our main findings based on in-depth qualitative assessments suggest that a home-based exercise training program was suitable and well-accepted by adolescents with JSLE and JIA during the COVID-19 pandemic. Nonetheless, adherence was not high, particularly among JIA patients, suggesting that facilitators and barriers identified in the current study should be explored to improve the quality of new home-based exercise programs implementation, particularly in a future emerging crisis.

Mental Health Impact in Latin American Pediatric Rheumatologists During the COVID-19 Pandemic.

OBJECTIVE: The aim of this study was to assess mental health in Latin American pediatric rheumatologists (LAPRs) during the COVID-19 pandemic. METHODS: A cross-sectional study was performed with 318 LAPRs based on an online, self-rated survey about clinical practice/mental health impacts during the COVID-19 pandemic. Validated self-reported scales for anxiety (Generalized Anxiety Disorder [GAD-7]) and depression (Patient Health Questionnaire [PHQ-9]) were evaluated. RESULTS: The response rate was 126 of 318 (40%), including 13 of 20 (65%) Latin American countries. Working on the COVID-19 frontline was reported by 27% of LAPRs. Anxiety and moderate/severe depression were observed in 49% and 25%, respectively. No LAPRs reported previous mental health disorders. Deaths of childhood-onset systemic lupus erythematosus and juvenile idiopathic arthritis patients with confirmed/suspected COVID-19 were reported by 8% and 2% of LAPRs, respectively. Further analysis of LAPRs revealed that the median current age was significantly lower in LAPRs with anxiety than in those without anxiety (39 [29-43] vs 45 [30-70] years, p = 0.029). Working on the frontline of COVID-19 (37% vs 17%, p = 0.015), feeling helpless (39% vs 17%, p = 0.009), and experiencing burnout (39% vs 11%, p = 0.0001) were factors significantly higher in LAPRs with anxiety. Median nighttime sleep abnormalities measured by the visual analog scale (VAS) (8 [0-10] vs 4 [0-10], p = 0.009) were significantly higher in the anxiety group, whereas the physical activity VAS was lower (0.5 [0-10] vs 3 [0-10], p = 0.005). A positive Spearman correlation was shown between the GAD-7 score and nighttime sleep abnormality VAS score (r = +0.348, p < 0.001), and a negative correlation was shown between the GAD-7score and physical activity VAS score (r = -0.192, p = 0.031). CONCLUSIONS: Anxiety and depression were relevant to the experience of LAPRs during the COVID-19 pandemic, impacting their mental health. Reporting information about mental health is essential to planning future preventive and health promotion strategies.

Heart function in juvenile idiopathic arthritis patients: A biventricular two-dimensional speckle-tracking echocardiography study.

OBJECTIVES: We evaluated cardiac function in juvenile idiopathic arthritis (JIA) patients by 2D speckle-tracking echocardiography (2DSTE) and to assess possible associations with clinical, laboratorial, and treatment data. METHODS: A group of 42 JIA patients and 42 healthy controls were evaluated using both conventional echocardiography and 2DSTE. JIA patients underwent clinical and laboratory assessment. RESULTS: Conventional echocardiography data demonstrated normal left ventricular (LV) ejection fraction in both groups (71 vs. 71%; p = .69). 2DSTE analysis demonstrated that JIA patients presented significantly lower LV global systolic longitudinal strain (LVGLS) (-18.76 vs. -22%; p < .0001), LV systolic strain rate (LVSSR) (1.06 vs. 1.32 s-1; p < .0001), LV diastolic strain rate (LVDSR) (1.58 vs. 1.8 s-1; p < .0137), right ventricular global systolic strain (RVGLS) (-24.1% vs. -27.7%; p = .0002), and right ventricular systolic strain rate (RVSSR) (1.4 vs. 1.8 s-1; p = .0035). JIA patients under biological agents presented higher LVGLS (p = .02) and RVLS (p = .01). We also detected an association between LVGLS and C-reactive protein [CRP; -20% in normal CRP (10/42) vs. -18% in elevated CRP patients (32/42), p = .03]. CONCLUSIONS: JIA patients present different echocardiographic status from healthy patients. Moreover, our data suggest that JIA patients under biological agents present association with better cardiac function as shown by strain analysis.

Influence of air pollution on renal activity in patients with childhood-onset systemic lupus erythematosus.

BACKGROUND: Childhood-onset systemic lupus erythematosus (cSLE) is an autoimmune and multifactorial disease that can affect the renal system. Exposure to air pollution can trigger systemic inflammation in cSLE patients and increase risk of disease activity. We evaluated effects of individual real-time exposure to air pollutants on renal activity in cSLE patients using the Systemic Lupus Erythematosus Disease Activity Index 2000. METHODS: Longitudinal panel study of 108 repetitive measures from 9 pediatric lupus patients. Over three consecutive weeks, daily individual levels of fine particulate matter (PM2.5) and nitrogen dioxide (NO2) were measured, as well as weekly clinical evaluation and laboratory tests. This was repeated every 10 weeks over a 1-year period. Specific generalized estimating equation models were used to evaluate the impact of these pollutants on risk of nephritis and anti-dsDNA > 20 UI/mL and on 24-h urine protein and serum complement (C3) levels. RESULTS: An interquartile range (IQR) increase of 18.12 µg/m3 in PM2.5 daily concentration was associated with increased risk of nephritis and positive results for anti-dsDNA. Moreover, increase in 24-h urine protein and decrease in C3 serum levels also associated with exposure to pollutants. An IQR increase in PM2.57-day moving average was associated with increased risks of leukocyturia (3.4; 95% CI 2.6:4.3), positive anti-dsDNA (3.1; 95% CI 2.1:4.0), and 36.3-mg increase (95% IC 20.2:52.3) in 24-h urine protein. An IQR increase (63.1 µg/m3) in 7-day cumulative NO2 levels was associated with decreased serum C3 levels. CONCLUSIONS: This prospective study suggests exposure to air pollution can trigger renal activity in cSLE patients.

Classism in Pain Care: The Role of Patient Socioeconomic Status on Nurses' Pain Assessment and Management Practices.

OBJECTIVE: Research on social disparities in pain care has been mainly focused on the role of race/racism and sex/sexism. Classism in pain assessment and management practices has been much less investigated. We aimed to test the effect of patient socioeconomic status (SES; a proxy of social class) on nurses' pain assessment and management practices and whether patient SES modulated the effects of patient distress and evidence of pathology on such practices. DESIGN: Two experimental studies with a two (patient SES: low/high) by two (patient distress or evidence of pathology: absent/present) between-subject design. SUBJECTS: Female nurses participated in two experimental studies (N = 150/N = 158). METHODS: Nurses were presented with a vignette/picture depicting the clinical case of a female with chronic low back pain, followed by a video of the patient performing a pain-inducing movement. Afterwards, nurses reported their pain assessment and management practices. RESULTS: The low-SES patient's pain was assessed as less intense, more attributed to psychological factors, and considered less credible (in the presence of distress cues) than the higher-SES patient's pain. Higher SES buffered the detrimental impact of the presence of distress cues on pain assessment. No effects were found on management practices. CONCLUSIONS: Our findings point to the potential buffering role of SES against the detrimental effect of certain clinical cues on pain assessments. This study contributes to highlighting the need for further investigation of the role of SES/social class on pain care and its underlying meanings and processes.

Evans Syndrome at Childhood-Onset Systemic Lupus Erythematosus Diagnosis: A Large Multicenter Study.

BACKGROUND: Evans syndrome (ES) in childhood-onset systemic lupus erythematosus (cSLE) patients has been rarely reported and limited to small populations. PROCEDURES: A retrospective multicenter cohort study (Brazilian cSLE group) was performed in 10 Pediatric Rheumatology services including 850 patients with cSLE. ES was assessed at disease diagnosis and defined by the combination of immune thrombocytopenia and autoimmune hemolytic anemia. RESULTS: ES was observed in 11 of 850 (1.3%) cSLE patients. The majority of them had hemorrhagic manifestations (91%) and active disease (82%). All patients with ES were hospitalized and none died. Comparisons of cSLE patients with and without ES at diagnosis revealed similar frequencies of female gender, multiorgan involvement, autoantibodies profile, and low complement (P > 0.05). Patients with ES had a lower frequency of malar rash (9% vs. 53%, P = 0.003) and musculoskeletal involvement (18% vs. 69%, P = 0.001) than those without this complication. The frequencies of intravenous methylprednisolone (82% vs. 43%, P = 0.013) and intravenous immunoglobulin use (64% vs. 3%, P < 0.0001) were significantly higher in the ES group, with similar current prednisone dose between groups (1.1 [0.76-1.5] vs. 1.0 mg/kg/day [0-30], P = 0.195). CONCLUSIONS: Our large multicenter study identified ES as a rare and severe initial manifestation of active cSLE with good outcome. Diagnosis is challenging due to the lack of typical signs and symptoms of lupus and the requirement to exclude infection and primary immunodeficiency.

Hooked from the deep: a rare new species of Taeniogyrus (Holothuroidea, Chiridotidae) from the continental slope of Brazil, southwestern Atlantic.

Most species of Taeniogyrus Semper, 1867 are known from shallow water in the Indo-Pacific, with other records in Antarctica, Mediterranean Sea, and the Atlantic. A new species of Taeniogyrus is described and illustrated here from the continental slope of Campos Basin, southeast of Brazil. In this species, sigmoid hooks (336-405 µm) are much larger than in any other in the genus, bearing a long and conspicuous hook region. Wheels with six spokes (86-169 µm), inner margin with 60-125 continuous teeth, are confined to round papillae along each interradius. Polian vesicles are ventral, numerous (15-21), of different sizes, and tubular shaped with a terminal round region. This new species represents the deepest record of the genus Taeniogyrus. It increases to three the number of chiridotids in Brazilian waters, and the number of Taeniogyrus species in the Atlantic. Additionally, Taeniogyrus furcipraeditus (Salvini-Plawen, 1972) from the Mediterranean Sea and Taeniogyrus havelockensis (Rao, 1975) from the Andaman Sea are proposed as new combinations.

Tc9 cells, a new subset of CD8(+) T cells, support Th2-mediated airway inflammation.

Similar to T-helper (Th) cells, CD8(+) T cells also differentiate into distinct subpopulations. However, the existence of IL-9-producing CD8(+) T (Tc9) cells has not been elucidated so far. We show that murine CD8(+) T cells activated in the presence of IL-4 plus TGF-ß develop into transient IL-9 producers characterized by specific IFN-γ and IL-10 expression patterns as well as by low cytotoxic function along with diminished expression of the CTL-associated transcription factors T-bet and Eomesodermin. Similarly to the CD4(+) counterpart, Tc9 cells required for their differentiation STAT6 and IRF4. Tc9 cells deficient for these master regulators displayed increased levels of Foxp3 that in turn suppressed IL-9 production. In an allergic airway disease model, Tc9 cells promoted the onset of airway inflammation, mediated by subpathogenic numbers of Th2 cells. This support was specific for Tc9 cells because CTLs failed to exert this function. We detected increased Tc9 frequency in the periphery in mice and humans with atopic dermatitis, a Th2-associated skin disease that often precedes asthma. Thus, our data point to the existence of Tc9 cells and to their supportive function in Th2-dependent airway inflammation, suggesting that these cells might be a therapeutic target in allergic disorders.

Takayasu arteritis in a Brazilian multicenter study: children with a longer diagnosis delay than adolescents.

OBJECTIVES: To evaluate and compare demographic, clinical, laboratory and angiographic data of Brazilian children and adolescents with Takayasu's arteritis. METHODS: In this Brazilian multicentre, retrospective study which included 10 paediatric rheumatology centres, we identified 71 children and adolescents with Takayasu's arteritis which were diagnosed before their 19th birthday. The patients' demographic, clinical, laboratorial and angiographic data were recorded. The participants were divided into two groups: children, defined by the WHO as younger than 10 years old (group 1: 36 patients) and adolescents, defined as individuals aged 10 to 19 years old (group 2: 35 patients). Features of both groups concerning disease manifestations were compared. RESULTS: A total of 21 (58.3%) patients in group 1 and 30 (85.7%) patients in group 2 were girls (p=0.01). The mean age at disease onset, the mean time to diagnosis, and the mean follow-up time were 5.7 and 12.7, 1.8 and 0.7, 7.2 and 3.6 years, respectively, in groups 1 and 2 (p<0.001, 0.001 and <0.001). At initial evaluation, constitutional symptoms (77.5%) were the most predominant symptoms and decreased peripheral pulses (85.9%) was the most predominant clinical sign without differences between groups. The main laboratory findings were increased erythrocyte sedimentation rate followed by leukocytosis. Anaemia, thrombocytosis and higher platelet levels were significantly more frequent in group 1 (p=0.031, 0.001 and 0.018). Angiographic data were similar in both groups. CONCLUSIONS: Children presented more laboratory abnormalities but clinical and angiographic characteristics were similar to those presented by the adolescents. Diagnosis delay is longer in younger patients.