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.

A clinical prediction model for estimating the risk of developing uveitis in patients with juvenile idiopathic arthritis.

OBJECTIVE: To build a prediction model for uveitis in children with JIA for use in current clinical practice. METHODS: Data from the international observational Pharmachild registry were used. Adjusted risk factors as well as predictors for JIA-associated uveitis (JIA-U) were determined using multivariable logistic regression models. The prediction model was selected based on the Akaike information criterion. Bootstrap resampling was used to adjust the final prediction model for optimism. RESULTS: JIA-U occurred in 1102 of 5529 JIA patients (19.9%). The majority of patients that developed JIA-U were female (74.1%), ANA positive (66.0%) and had oligoarthritis (59.9%). JIA-U was rarely seen in patients with systemic arthritis (0.5%) and RF positive polyarthritis (0.2%). Independent risk factors for JIA-U were ANA positivity [odds ratio (OR): 1.88 (95% CI: 1.54, 2.30)] and HLA-B27 positivity [OR: 1.48 (95% CI: 1.12, 1.95)] while older age at JIA onset was an independent protective factor [OR: 0.84 (9%% CI: 0.81, 0.87)]. On multivariable analysis, the combination of age at JIA onset [OR: 0.84 (95% CI: 0.82, 0.86)], JIA category and ANA positivity [OR: 2.02 (95% CI: 1.73, 2.36)] had the highest discriminative power among the prediction models considered (optimism-adjusted area under the receiver operating characteristic curve = 0.75). CONCLUSION: We developed an easy to read model for individual patients with JIA to inform patients/parents on the probability of developing uveitis.

Validation and adaptation of a German screening tool to identify patients at risk of juvenile idiopathic arthritis.

The primary objective was twofold: (1) to determine whether the German version of a screening instrument for clinical practice for juvenile idiopathic arthritis (SICJIA) is reliable in identifying patients at risk for juvenile idiopathic arthritis (JIA), and (2) secondly whether a weighting scheme of individual questions improves its sensitivity. Data were collected and retrospectively analyzed based upon completed SICJIA questionnaires from patients and their guardians at their first clinical visit at the Hamburg Centre for Pediatric and Adolescence Rheumatology. All patients visited the center between August 2015 and July 2017. The survey included 12 disease-orientated questions. For evaluation, only questionnaires of patients diagnosed with JIA or with a non-inflammatory joint pain (NJP) were selected. Standard statistical techniques were used for evaluation. In total, 165 of 800 questionnaires could be used for evaluation. Of the 800 patients who completed questionnaires, 133 were diagnosed with JIA and 32 with NJP. The analysis of the individual questions was performed by comparing the rate of a positive response to the questions between the two groups. Four questions showed a significant difference by comparing the groups, using JIA patients with at least one active joint. The diagnostic accuracy of the weighted sum score increased from 64 to 68% to discriminate between the groups in comparison to the ordinary sum score. An optimal cutoff of 6.0 for referral to a pediatric rheumatologist was calculated. The validation of the SICJIA showed a discriminative difference in patients with clinical diagnosed JIA and a control group diagnosed with NJP. The weighted sum score performed better to differentiate between JIA and NJP patients. The modified SICJIA can be useful to identify patients at risk of JIA.

Lipid profile among girls with systemic lupus erythematosus.

The aim of the study was to describe biomarkers of lipid metabolism associated with increased cardiovascular risk and their correlation with disease variables and markers of inflammation in adolescent females with systemic lupus erythematosus (SLE). This cross-sectional controlled study evaluated 33 adolescent females with juvenile SLE and 33 healthy controls. Anthropometric data, SLE disease activity index (SLEDAI), medications, proteinuria, ultra-sensitive C-reactive protein (us-CRP), lipid profile (total cholesterol, LDL-c, HDL-c and triglycerides), apolipoproteins A and B (Apo A-I and B), paraoxonase, and myeloperoxidase were evaluated. Median age of the patients and the median disease duration were 16.7 years and 54 months, respectively. SLEDAI scores above 4 were observed in 11 (33.3 %) patients. Moreover, 12 (36.4 %) patients were overweight, and 5 (15.2 %) had low height for age ratios. Dyslipidemia was observed in 13 (39.4 %) patients and in 7 (21.2 %) controls with a decrease in HDL-c concentrations in SLE patients even after adjustment for their nutritional status. In the group with SLE, us-CRP concentrations were inversely correlated with LDL-c/ApoB ratio (p = 0.031). After multivariate regression analysis, the SLE group showed lower concentration of Apo A-I and a decreased LDL-c/ApoB ratio. SLE adolescent females with low disease activity, with preserved kidney function and on low dose of corticosteroids, regardless of nutritional status and food intake, have proatherogenic lipid biomarkers, which may contribute to an increased atherosclerotic risk.

Subclinical Pulmonary Hypertension in Childhood Systemic Lupus Erythematosus Associated with Minor Disease Manifestations.

The aim of this study was to evaluate pulmonary hypertension (PH) in 852 childhood-onset systemic lupus erythematosus (cSLE) patients. This was a large multicenter study conducted in 10 Pediatric Rheumatology Services of São Paulo state, Brazil. PH was defined as systolic pulmonary artery pressure >35 mmHg and/or measurement of the mean pulmonary artery pressure >25 mmHg and/or diastolic pressure >15 mmHg by transthoracic echocardiogram. Demographic data, clinical manifestations, disease activity score (SLEDAI-2K), disease damage score (SLICC/ACR-DI) and treatments were also evaluated. Statistical analysis was performed using Bonferroni correction (p < 0.002). PH was observed in 17/852 (2%) cSLE patients. Effort dyspnea occurred in 3/17, chest pain in 1/17 and right ventricle dysfunction in 3/17 cSLE patients. None had pulmonary thromboembolism or antiphospholipid syndrome. Further comparison between 17 cSLE with PH and 85 cSLE control patients without PH with similar disease duration [15 (0-151) vs. 15 (0-153) months, p = 0.448], evaluated at the last visit, revealed higher frequencies of fever (47 vs. 9%, p < 0.001), reticuloendothelial manifestations (41 vs. 7%, p < 0.001) and serositis (35 vs. 5%, p = 0.001) in the former group. Frequencies of renal and neuropsychiatric involvements and antiphospholipid syndrome, as well as the median of SLEDAI-2K and SLICC/ACR-DI scores, were comparable in both groups (p > 0.002). Normal transthoracic echocardiography was evidenced in 9/17 (53%), with median cSLE duration of 17.5 months (1-40) after PH standard treatment. PH was a rare manifestation of cSLE occurring in the first two years of disease. The majority of patients were asymptomatic with mild lupus manifestations. The underlying mechanism seemed not to be related to pulmonary thromboembolism and/or antiphospholipid syndrome.

Nailfold videocapillaroscopy in healthy children and adolescents: description of normal patterns.

OBJECTIVES: to describe normal patterns of nailfold videocapillaroscopy (NVC) in healthy children and adolescents; to quantify the relationship between age and capillary dimensions, intercapillary distance and number of capillaries/mm; to evaluate the inter and intraobserver concordance. METHODS: Cross-sectional study including 100 healthy participants aged 5 to 18 years. Capillary dimensions (capillary loop length, capillary width and intercapillary distance) and number of capillaries/mm were evaluated in 900 capillaries using stereomicroscope under 100x magnification. Intra and inter observer agreements were tested. RESULTS: The capillary dimensions (mean ± SD) were: capillary loop length 278.6±60.3 µm, intercapillary distance 124.1±28.1 µm, capillary width 15.0±2.6 µm. Teenagers between 15 and 18 years had longer and more enlarged capillaries than the other age groups (p<0.001 and p=0.012 respectively). We also found a significant increase in the number of capillaries/mm with age (p<0.001). There was a positive correlation between age and number of capillaries/mm, capillary length, and capillary width (p<0.001, R=0.796; p<0.001, R=0.368; p=0.004, R=0.285, respectively). There was a good intra and interobserver concordance. Enlarged capillary and avascular areas were present in 11% and 10% of capillaries respectively. A weak negative correlation was found between the intercapillary distance and the number of capillaries/mm (p=0.05; R=-0.20). CONCLUSIONS: There is a wide variability in the capillary morphology among healthy individuals. There was a positive correlation between age and number of capillaries/mm, capillary length, and capillary width. In addition, NVC has been shown to be a reproducible method.

Fatigue in children with juvenile idiopathic arthritis: reliability of the "Pediatric Quality of Life Inventory-Multidimensional Fatigue Scale".

The aim of the study was (1) to translate the "Pediatric Quality of Life Inventory-Multidimensional Fatigue Scale" (PedsQL-Fatigue) into Brazilian Portuguese language and culture and evaluate its reliability and (2) to measure fatigue among patients with juvenile idiopathic arthritis (JIA): (1) Translation of the PedsQL-Fatigue by two bilingual researchers; (2) Backtranslation into English assessed by the authors of the original version; (3) Pilot study with five patients followed in the Pediatric Rheumatology Outpatient Clinic and their parents; and (4) Field study and assessment of measurement properties (internal consistency, reproducibility, and construct validity). In this stage, the scale was administered to 67 patients with JIA and 63 healthy individuals, aged from 2 to 18 years old, matched by age (from 2 to 4, 5 to 7, 8 to 12, and from 13 to 18 years old). Cronbach's alpha coefficient ranged from 0.6 to 0.8 for children and parents, indicating the instrument's good internal consistency. The scale's construct validity was confirmed by a satisfactory Spearman's coefficient between the PedsQL-Fatigue and the generic PedsQL 4.0 (0.840 for the children and 0.742 for the parents). Reproducibility was also adequate (0.764 for the children and 0.938 for the parents). No differences were found between the scores obtained by the JIA group and control group, though lower scores were observed among patients with clinically active JIA when compared to those without clinical activity. The PedsQL-Fatigue is a valid and reliable tool, and that can be used to measure fatigue among patients with JIA.

Importance of adherence in the outcome of juvenile idiopathic arthritis.

Medical treatment of juvenile idiopathic arthritis (JIA) has advanced in the last decade, and improved prognosis is a reality in daily clinical practice. Despite this improvement in the quality of treatment, the outcome can still be compromised by modifiable factors, including delayed referral to a specialist, delayed drug treatment, poor adherence to treatment, and early interruption of drug treatment. In this review we discuss the most relevant aspects related to adherence to treatment in JIA, with emphasis on: factors that affect adherence to treatment; effect of poor adherence to treatment on JIA prognosis; when to suspect and how to assess poor adherence to treatment; and strategies to promote adherence to treatment, with an emphasis on information-reinforcement education. Besides presenting the findings of other authors, we also try to report our experience of this subject, which is still a challenge for health professionals.

Enrichment of Rare Variants of Hemophagocytic Lymphohistiocytosis Genes in Systemic Juvenile Idiopathic Arthritis.

OBJECTIVE: To evaluate whether there is an enrichment of rare variants in familial hemophagocytic lymphohistiocytosis (HLH)-associated genes among patients with systemic juvenile idiopathic arthritis (sJIA) with or without macrophage activation syndrome (MAS). METHODS: Targeted sequencing of HLH genes (LYST, PRF1, RAB27A, STX11, STXBP2, UNC13D) was performed in sJIA subjects from an established cohort. Sequence data from control subjects were obtained in silico (dbGaP:phs000280.v8.p2). Rare variant association testing (RVT) was performed with sequence kernel association test (SKAT) package. Significance was defined as p < 0.05 after 100,000 permutations. RESULTS: Sequencing data from 524 sJIA cases were jointly called and harmonized with exome-derived target data from 3000 controls. Quality control operations produced a set of 480 cases and 2924 ancestrally-matched control subjects. RVT of cases and controls revealed a significant association with rare protein-altering variants (minor allele frequency [MAF] < 0.01) of STXBP2 (p = 0.020), and ultra-rare variants (MAF < 0.001) of STXBP2 (p = 0.006) and UNC13D (p = 0.046). A sub-analysis of 32 cases with known MAS and 90 without revealed a significant difference in the distribution of rare UNC13D variants (p = 0.0047) between the groups. Additionally, sJIA patients more often carried ≥ 2 HLH variants than did controls (p = 0.007), driven largely by digenic combinations involving LYST. CONCLUSION: We identified an enrichment of rare HLH variants in sJIA patients compared with controls, driven by STXBP2 and UNC13D. Biallelic variation in HLH genes was associated with sJIA, driven by LYST. Only UNC13D displayed enrichment in patients with MAS. This suggests that HLH variants may contribute to the pathophysiology of sJIA, even without MAS.

Effects of Pilates exercises on health-related quality of life in individuals with juvenile idiopathic arthritis.

OBJECTIVE: To determine the effects of Pilates exercises on health-related quality of life (HRQOL) in individuals with juvenile idiopathic arthritis (JIA). DESIGN: Randomized, prospective, single-blind trial. SETTING: Outpatient clinic of pediatric rheumatology and the rehabilitation department. PARTICIPANTS: Children (N=50) with JIA according to the International League of Associations for Rheumatology criteria. INTERVENTIONS: Participants were randomly assigned into 2 groups. In group I (n=25), the participants were given a conventional exercise program for 6 months. Patients in group II (n=25) participated in a Pilates exercise program for 6 months. MAIN OUTCOME MEASURES: The primary outcome measure was HRQOL, as measured by the Pediatric Quality of Life Inventory version 4.0 (PedsQL 4.0). The secondary outcome measures provided an estimate of the clinical relevance of the primary outcome results and included joint pain intensity (according to a 10-cm visual analog scale), disability (according to the Childhood Health Assessment Questionnaire), joint status (using the Pediatric Escola Paulista de Medicina Range of Motion Scale), and the total PedsQL 4.0 score. RESULTS: All participants completed the study. The scores of the PedsQL 4.0 differed significantly between groups, indicating that Pilates exercises increased these scores when compared with the conventional exercise program. Group II participants showed significant improvements in the 10-cm visual analog scale-joint pain, Childhood Health Assessment Questionnaire, and Pediatric Escola Paulista de Medicina Range of Motion Scale. CONCLUSIONS: The use of Pilates exercises had a positive physical and psychosocial impact on HRQOL in individuals with JIA. Future multicenter studies with a follow-up beyond the period of treatment using more objective parameters will be useful to support the results of the present study.

Hereditary autoinflammatory syndromes: a Brazilian multicenter study.

OBJECTIVE: To evaluate the prevalence of genetic defects in clinically suspected autoinflammatory syndromes (AIS) in a Brazilian multicenter study. METHODS: The study included 102 patients with a clinical diagnosis of Cryopyrin Associated Periodic Syndromes (CAPS), TNF Receptor Associated Periodic Syndrome (TRAPS), Familial Mediterranean Fever (FMF), Mevalonate Kinase Deficiency (MKD) and Pediatric Granulomatous Arthritis (PGA). One of the five AIS-related genes (NLRP3, TNFRSF1A, MEFV, MVK and NOD2) was evaluated in each patient by direct DNA sequencing, based on the most probable clinical suspect. RESULTS: Clinical diagnoses of the 102 patients were: CAPS (n = 28), TRAPS (n = 31), FMF (n = 17), MKD (n = 17) and PGA (n = 9). Of them, 27/102 (26 %) had a confirmed genetic diagnosis: 6/28 (21 %) CAPS patients, 7/31 (23 %) TRAPS, 3/17 (18 %) FMF, 3/17 (18 %) MKD and 8/9 (89 %) PGA. CONCLUSION: We have found that approximately one third of the Brazilian patients with a clinical suspicion of AIS have a confirmed genetic diagnosis.

HEALTH-RELATED QUALITY OF LIFE IN ADOLESCENTS AND YOUNG ADULTS WITH INFLAMMATORY BOWEL DISEASE IS ASSOCIATED WITH REDUCTION IN SCHOOL AND WORK PRODUCTIVITY RATHER THAN PHYSICAL IMPAIRMENT: A MULTIDISCIPLINARY STUDY.

BACKGROUND: Inflammatory bowel diseases (IBD), comprising Crohn's disease and ulcerative colitis, are chronic inflammatory diseases of the gastrointestinal tract that often have their onset among adolescents and young adults (AYA). IBD are characterized by episodes of active disease interspersed with periods of remission, and its activity is inversely correlated with health-related quality of life (HRQL). OBJECTIVE: This study aimed to determine whether AYA in remission or with low IBD activity would exhibit HRQL similar to that of age-matched healthy individuals, and whether demographic and disease factors could affect HRQL using a 'patient-reported outcome' instrument. METHODS: This study enrolled only AYA with IBD, with low activity. This research included five multidisciplinary clinics of two academic hospitals: Paediatric Gastroenterology, Gastroenterology, Coloproctology, Paediatric Rheumatology and Adolescent divisions, São Paulo, Brazil. A total of 59 AYA with IBD (age, 13-25 years) and 60 healthy AYA (age, 13-25 years) completed the Pediatric Quality of Life Inventory 4.0 and 36-Item Short-Form Health Survey questionnaires and the visual analogue scale (VAS) for pain. Demographic data, extra-intestinal manifestations, treatment, and outcomes regarding CD and UC were evaluated. RESULTS: AYA with IBD and healthy controls were similar with respect to median ages (18.63 [13.14-25.80] years vs 20.5 [13.68-25.84] years, P=0.598), proportion of female sex (42% vs 38%, P=0.654), and percentage of upper middle/middle Brazilian socioeconomic classes (94% vs 97%, P=0.596). The school/work score was significantly lower in AYA with IBD than in healthy controls (70 [10-100] vs 75 [5-100], P=0.037). The 'general health-perception' score was significantly lower in AYA with IBD than in healthy controls (50 [10-80] vs 0 [25-90], P=0.0002). The median VAS, FACES pain rating scale, and total VAS scores were similar between the two groups (2 [0-10] vs 3 [0-9], P=0.214). No association between HRQL and clinical and demographic parameters was identified among IBD patients. CONCLUSION: AYA with low IBD activity reported poor HRQL in school/work and general health perception domains, which highlights a disability criterion in this vulnerable population.

Translation and validation of the Transition Readiness Assessment Questionnaire (TRAQ).

OBJECTIVE: To translate and validate the Brazilian Portuguese version of the Transition Readiness Assessment Questionnaire in a population of adolescents and young adults with chronic rheumatologic disorders. This questionnaire evaluates the patient's readiness for making the transition from the pediatric health service to adult care. METHODS: The four-phase methodology for the translation and validation of generic questionnaires was followed, including translation, back-translation, pilot testing and clinical validation of the final tool. The confirmatory factor analysis was used for clinical validation and the Cronbach's alpha coefficient was used to assess the overall internal consistency of the final tool. RESULTS: A total of 150 patients with a mean age of 17.0 years (SD=2.2 years, range 14-21 years) were enrolled for the final tool validation. Of those, 71 patients had juvenile systemic lupus erythematosus (47.3%), 64 had juvenile idiopathic arthritis (42.7%), and 15 had juvenile dermatomyositis (10%). During the confirmatory factor analysis, the dimension "Talking with providers" consisting of two questions, was considered as not fitting the translated questionnaire due to a very high ceiling effect and was therefore excluded. All other translated items favorably contributed to the overall consistency of the final tool; removing that dimension did not result in a substantial increase in Cronbach's alpha, which was 0.776. CONCLUSIONS: The Brazilian Portuguese version of the Transition Readiness Assessment Questionnaire was validated in a population of transitional patients with chronic rheumatologic disorders, after one dimension from the original questionnaire was excluded. It is a non-specific disease questionnaire; thus, it can be used to evaluate the transition readiness of Brazilian patients with other chronic diseases.

Gait in children and adolescents with idiopathic musculoskeletal pain.

INTRODUCTION: Musculoskeletal pain is a constant complaint in pediatric practice. The pain may be related to a number of organic diseases and / or be part of the amplified musculoskeletal pain syndromes. Idiopathic musculoskeletal pain (IMSP) is defined as the presence of intermittent pain in three or more body regions for at least three months, excluding organic diseases that could explain the symptoms. OBJECTIVE: To study the gait of children and adolescents with IMSP by dynamic baropodometry. METHODOLOGY: Thirty-two patients with IMSP and 32 healthy controls, matched by age, sex, social class, and body mass index (BMI) were enrolled. All were evaluated for pain intensity through the visual analogue scale (VAS) and gait evaluation using dynamic baropodometry. RESULTS: The mean age of the IMSP group was 13.6 years (SD = 2.1, range 9.8-16.9) and of the control group was 13.5 years (SD = 2.0, range 9.6-16.5). The mean pain scale was 5.4 cm in the IMSP group and 0 cm in the control group (p < 0.001). In gait, the mean right foot velocity of the IMSP group was significantly lower (p = 0.034), the time of the step of the IMSP group was significantly higher (p = 0.003) and the pace of the IMSP group was significantly lower (p = 0.001). CONCLUSION: In our study we observed differences between the gait of children with IMSP and healthy controls according to the dynamic baropodometry. This finding indicates the need for individualized attention to the gait of children with musculoskeletal pain.

Quality of life of children and adolescents from São Paulo: reliability and validity of the Brazilian version of the Pediatric Quality of Life Inventory version 4.0 Generic Core Scales.

OBJECTIVES: To evaluate the reliability and validity of the Brazilian version of the Pediatric Quality of Life Inventory (PedsQL 4.0) Generic Core Scales and measure the quality of life of healthy children and adolescents and patients with rheumatic diseases. METHODS: We followed the translation methodology proposed by the developer of the original English version of the PedsQL 4.0. The instrument was administered by interviews in two groups: 240 apparently healthy children and adolescents from São Paulo (SP, Brazil) and 105 patients with chronic rheumatic diseases matched by age, as well as their respective parents or caregivers. The parent proxy-report was administered to the children's parents or caregivers separately on the same day. RESULTS: Cronbach's alpha values were between 0.6 and 0.9 for all dimensions, demonstrating adequate internal consistency. Patients with rheumatic diseases reported significantly lower PedsQL scores on all dimensions when compared to the healthy control group (p < 0.0001). Construct validity of the Brazilian Portuguese version of the PedsQL 4.0 was also confirmed. Parent proxy-report of patients with rheumatic diseases highly correlated with child self-report for physical functioning (r = 0.77, p < 0.001) and school functioning (r = 0.73, p < 0.001). Lower correlations were observed for emotional and social functioning (r = 0.40 and 0.59, respectively, p < 0.001). CONCLUSIONS: The tool demonstrated reliability, validity, and the administration was fast and easy. Quality of life in patients with rheumatic diseases was significantly lower than in the healthy control group, supporting the necessity of a comprehensive approach to rheumatic disease management, focused on the psychosocial dimensions.

Pseudotumor cerebri in childhood and adolescence: data from a specialized service.

OBJECTIVE: To report cases of children and adolescents diagnosed with pseudotumor cerebri associated or not with rheumatic disease. METHODS: This was a retrospective study based on medical reports of 29 patients, up to 18 years of age and diagnosed with pseudotumor cerebri, followed up in the Pediatric Rheumatology and Neurology outpatient clinics of a tertiary hospital, until December 2016. RESULTS: Among the 29 patients diagnosed with pseudotumor cerebri, 51.7% were girls and the mean age at the disease onset was 12.3 years. In 18 patients (62%) where an etiology was found, four were associated with a rheumatic disease. The most common symptom was headache (69%) and acetazolamide was the most used medication (69%). Two patients developed blindness and 10 are still being followed up. CONCLUSION: Although rare, pseudotumor cerebri should be considered in children with headaches, especially in patients with rheumatic disease.

Henoch-Schönlein purpura: recurrence and chronicity.

OBJECTIVES: To describe a group of patients treated at our service for Henoch-Schönlein purpura, with emphasis on recurrent and chronic cases, and to compare clinical and demographic characteristics of patients with monocyclic and recurrent disease. METHODS: Data on 67 patients who had been treated since disease onset were analyzed. Twelve patients were excluded because they failed to return for follow-up consultations after less than 3 months, leaving a total of 55 children in the study sample. Recurrence was defined as the presence of a fresh episode after a period of at least 3 months without symptoms, and cases were defined as chronic when cutaneous, abdominal and renal manifestations persisted for a period of 12 months or more. RESULTS: Recurrence was observed in 8/55 patients (14.4%) and four cases were chronic (7.2%). In 29/55 patients (52.7%), infection was identified as the trigger factor. A monocyclic clinical course was observed in 43 patients (26 of whom were girls, with a mean age of 5.4 years). Gastrointestinal and renal involvement was observed in 55.8 and 20.9% of patients, respectively. Among the 12 patients with recurrent or chronic Henoch-Schönlein purpura, three had arthritis, four exhibited signs and symptoms of abdominal involvement and seven of kidney disease: microscopic hematuria in five, macroscopic hematuria in one and hematuria with proteinuria in one other. Late onset was the only variable related to recurrence (p < 0.05). CONCLUSIONS: As is observed in medical literature, monocyclic cases are more common among children with early onset disease. Patients with Henoch-Schönlein purpura should be followed over the long term, since recurrent and chronic cases account for more than 20% of the total.

Functional resonance magnetic imaging (fMRI) in adolescents with idiopathic musculoskeletal pain: a paradigm of experimental pain.

BACKGROUND: Studies on functional magnetic resonance imaging (fMRI) have shown that adults with musculoskeletal pain syndromes tolerate smaller amount of pressure (pain) as well as differences in brain activation patterns in areas related to pain.The objective of this study was to evaluate, through fMRI, the brain activation in adolescents with idiopathic musculoskeletal pain (IMP) while performing an experimental paradigm of pain. METHODS: The study included 10 consecutive adolescents with idiopathic musculoskeletal pain (average age 16.3±1.0) and 10 healthy adolescents age-matched. fMRI exams were performed in a 3 T scanner (Magnetom Trio, Siemens) using an event-related design paradigm. Pressure stimuli were performed in the nondominant hand thumb, divided into two stages, fixed pain and variable pain. The two local Research Ethics Committees (Ethics Committee from Universidade Federal de São Paulo- Brazil, process number 0688/11, on July 1st, 2011 and Ethics Committee from Hospital Israelita Albert Einsten - Brazil, process number 1673, on October 19th, 2011) approved the study. RESULTS: The idiopathic musculoskeletal pain (IMP) group showed a reduced threshold for pain (3.7 kg/cm2 versus 4.45 kg/cm2, p = 0.005). Control group presented increased bain activation when compared to IMP group in the following areas: thalamus (p = 0.00001), precentral gyrus (p = 0.0004) and middle frontal gyrus (p = 0.03). In intragroup analysis, IMP group showed greater brain activation during the unpredictable stimuli of the variable pain stage, especially in the lingual gyrus (p = 0.0001), frontal lobe (p = 0.0001), temporal gyrus (p = 0.0001) and precentral gyrus (p = 0.03), when compared to predictable stimulus of fixed pain. The same intragroup analysis with the control group showed greater activation during the unpredictable stimuli in regions of the precentral gyrus (p = 0.0001), subcallosal area (p = 0.0001), right and left occipital fusiform gyrus (p = 0.0001; (p = 0.0007), middle gyrus (p = 0.01) and precuneus p = (0.02). CONCLUSION: Adolescents with idiopathic musculoskeletal pain (IMP) tend to request higher brain function in cognitive-emotional areas when interpreting unpredictable sensory-perceptual situations. Therefore, it is assumed that this difference in pain processing in adolescents with IMP make the subjective experience of pain something more intense and unpleasant.

Association between demyelinating disease and autoimmune rheumatic disease in a pediatric population.

INTRODUCTION: Multiple sclerosis (MS) and neuromyelitis optica (NMO) are demyelinating diseases of the central nervous system. Autoimmunity in patients with demyelinating disease and in their families has been broadly investigated and discussed. Recent studies show a higher incidence of rheumatic autoimmune diseases among adult patients with MS or NMO and their families, but there are no studies in the pediatric population. OBJECTIVE: To evaluate an association of MS and NMO with autoimmune rheumatic diseases in pediatric patients. METHOD: 22 patients younger than 21 years old with MS or NMO diagnosed before the age of 18 years were evaluated regarding epidemiological data, clinical presentation, association with autoimmune diseases, family history of autoimmune diseases, laboratory findings, imaging studies and presence of auto-antibodies. RESULTS: Among the patients studied, there was a prevalence of females (68.1%). The mean age of symptoms onset was 8 years and 9 months and the mean current age was 16 years and 4 months. Two patients (9%) had a history of associated autoimmune rheumatic disease: one case of juvenile dermatomyositis in a patient with NMO and another of systemic lupus erythematosus in a patient with MS. Three patients (13%) had a family history of autoimmunity in first-degree relatives. Antinuclear antibody was found positive in 80% of patients with NMO and 52% of patients with MS. About 15% of antinuclear antibody-positive patients were diagnosed with rheumatologic autoimmune diseases. CONCLUSION: Among patients with demyelinating diseases diagnosed in childhood included in this study there was a high frequency of antinuclear antibody positivity but a lower association with rheumatologic autoimmune diseases than that observed in studies conducted in adults.

Inflammatory myopathies in childhood: correlation between nailfold capillaroscopy findings and clinical and laboratory data.

OBJECTIVE: Nailfold capillaroscopy is an important tool for the diagnosis and follow-up of patients with rheumatic diseases, in particular dermatomyositis and scleroderma. A relationship has been observed in adults between improved capillaroscopic findings and reduced disease activity. Our aim was to correlate disease activity (clinical and laboratory data) and nailfold capillaroscopy findings in 18 patients with inflammatory myopathies. METHODS: This prospective study included 13 juvenile dermatomyositis patients (Bohan and Peter criteria) (mean age of 8.8 years) and five patients with overlap syndrome (mean age of 15.7 years). We evaluated disease activity (skin abnormalities and muscle weakness, muscle enzymes and acute phase reactants) and its correlation with nailfold capillaroscopy findings (dilatation of isolated loops, dropout of surrounding vessels and giant capillary loops). We used a microscope with special light and magnification of 10 to 16X. RESULTS: Eighteen patients underwent a total of 26 capillaroscopic examinations, seven of them on two or more occasions (13 were performed during the active disease phase and 13 during remission). Twelve of the 13 examinations performed during the active phase exhibited scleroderma pattern and 8 of the 13 examinations performed during remission were normal. Therefore, in 20 of the 26 examinations clinical and laboratory data and nailfold capillaroscopy findings correlated (p = 0.01). CONCLUSIONS: Nailfold capillaroscopy is a non-invasive examination that offers satisfactory correlation with disease activity and could be a useful tool for the diagnosis and follow-up of inflammatory myopathies.