Pancreatitis Subtypes Survey in 852 Childhood-Onset Systemic Lupus Erythematosus Patients.

OBJECTIVE: Pancreatitis is a rare and a life-threatening systemic lupus erythematosus (SLE) manifestation in childhood-onset SLE (cSLE). The objective of this study was to systematically classify pancreatitis in cSLE according to the International Study Group of Pediatric Pancreatitis and determine the overall prevalence, clinical features, laboratory, and first episode outcomes. METHODS: A multicenter cohort study in 10 pediatric rheumatology centers, including 852 patients with cSLE. RESULTS: Pancreatitis was diagnosed in 22 of 852 (2.6%) patients with cSLE. It was classified as acute pancreatitis in 20 (91%), acute recurrent pancreatitis in 2 (9%), and none of them had chronic pancreatitis. None of them had gallstones, traumatic pancreatitis, or reported alcohol/tobacco use. The comparison of patients with pancreatitis (first episode) and without this complication revealed a shorter disease duration (1 [0-10] vs 4 [0-23] years, P < 0.0001) and higher median of Systemic Lupus Erythematosus Disease Activity Index 2000 (21 [0-41] vs 2 [0-45], P < 0.0001). The frequencies of fever (P < 0.0001), weight loss (P < 0.0001), serositis (P < 0.0001), nephritis (P < 0.0001), arterial hypertension (P < 0.0001), acute renal failure (P < 0.0001), macrophage activation syndrome (P < 0.0001), and death (P = 0.001) were also higher in patients with pancreatitis. The frequencies of intravenous methylprednisolone use (P < 0.0001) and the median of prednisone dose (55 [15-60] vs 11 [1-90] mg/day, P < 0.0001) were significantly higher in patients with pancreatitis. Of note, the 2 patients with acute recurrent pancreatitis had 2 episodes, with pain-free interval of 1 and 4 years. CONCLUSIONS: This was the first study characterizing pancreatitis using the International Study Group of Pediatric Pancreatitis standardized definitions in patients with cSLE showing that the predominant form is acute pancreatitis seen in association with glucocorticoid treatment and active severe disease.

Incidence of active mycobacterial infections in Brazilian patients with chronic inflammatory arthritis and negative evaluation for latent tuberculosis infection at baseline--a longitudinal analysis after using TNFa blockers.

Several studies point to the increased risk of reactivation of latent tuberculosis infection (LTBI) in patients with chronic inflammatory arthritis (CIAs) after using tumour necrosis factor (TNF)a blockers. To study the incidence of active mycobacterial infections (aMI) in patients starting TNFa blockers, 262 patients were included in this study: 109 with rheumatoid arthritis (RA), 93 with ankylosing spondylitis (AS), 44 with juvenile idiopathic arthritis (JIA) and 16 with psoriatic arthritis (PsA). All patients had indication for anti-TNFa therapy. Epidemiologic and clinical data were evaluated and a simple X-ray and tuberculin skin test (TST) were performed. The control group included 215 healthy individuals. The follow-up was 48 months to identify cases of aMI. TST positivity was higher in patients with AS (37.6%) than in RA (12.8%), PsA (18.8%) and JIA (6.8%) (p < 0.001). In the control group, TST positivity was 32.7%. Nine (3.43%) patients were diagnosed with aMI. The overall incidence rate of aMI was 86.93/100,000 person-years [95% confidence interval (CI) 23.6-217.9] for patients and 35.79/100,000 person-years (95% CI 12.4-69.6) for control group (p < 0.001). All patients who developed aMI had no evidence of LTBI at the baseline evaluation. Patients with CIA starting TNFa blockers and no evidence of LTBI at baseline, particularly with nonreactive TST, may have higher risk of aMI.

LDPE and PLA Active Food Packaging Incorporated with Lemon by-Products Extract: Preparation, Characterization and Effectiveness to Delay Lipid Oxidation in Almonds and Beef Meat.

Low-density polyethylene-based packaging with 4% lemon extract (LDPE/4LE) and two polylactic-based (PLA) packaging materials with 4% and 6% lemon extract (PLA/PEG/4LE and PLA/6LE) were produced. O2 and water permeability tests were performed, the total and individual phenolic compounds content were measured, and the films' antioxidant activities were determined. The films' ability to delay lipid oxidation was tested in two model foods: almonds, packaged with LDPE/4LE, PLA/4LE and PLA/6LE for a maximum period of 60 days at 40 °C (accelerated assay); and beef meat, packaged with the PLA/6LE for a maximum period of 11 days at 4 °C. The LE improved the WVP in all of the active films by 33%, 20% and 60% for the LDPE/4LE, PLA/4LE and PLA/6LE films, respectively. At the end of 10 days, the migration of phenolic compounds through the PLA films was measured to be 142.27 and 114.9 µg/dm2 for the PLA/4LE and PLA/6LE films, respectively, and was significantly higher than phenolic compounds migration measured for the LDPE/4LE (15.97 µg/dm2). Naringenin, apigenin, ferulic acid, eriocitrin, hesperidin and 4-hydroxybenzoic acid were the main identified compounds in the PLA, but only 4-hydroxybenzoic acid, naringenin and p-coumaric acid were identified in the LDPE films. Regarding the films' ability to delay lipid oxidation, LDPE/4LE presented the best results, showing a capacity to delay lipid oxidation in almonds for 30 days. When applied to raw beef meat, the PLA/6LE packaging was able to significantly inhibit lipid oxidation for 6 days, and successfully inhibited total microorganisms' growth until the 8th day of storage.

Immune response and tolerability of varicella vaccine in children and adolescents with systemic lupus erythematosus previously exposed to varicella-zoster virus.

OBJECTIVES: The aim of the present paper is to evaluate the immune response and tolerability of varicella vaccine in children and adolescents with systemic lupus erythematosus previously exposed to varicella-zoster virus. METHODS: We performed a prospective and controlled study on a group of 54 SLE patients that were chosen at random to be or not to be vaccinated (28 were vaccinated and 26 were not). Twenty-eight healthy controls, of matching age and sex were also vaccinated. All were submitted to a questionnaire, physical evaluation and laboratory assays: lymphocyte immune-phenotyping by flow cytometry, plasma varicella zoster virus (VZV) serology by ELISA and in vitro interferon gamma (IFNγ) production by T-cells after stimulus with VZV antigen. They were evaluated before vaccination and at 30, 45, 180 and 360 days afterwards. RESULTS: We did not observe any differences in the frequency of adverse events in both vaccinated groups. At study entry, all individuals were seropositive for VZV antibodies. The serum VZV antibody titres similarly increased after vaccination. The frequency of flares and the SLEDAI score were also similar among the patients. Thirty days after vaccination the production of IFNγ specific to VZV was lower in the SLE group compared to healthy controls. In the follow-up we observed 4 cases of herpes zoster in the SLE unvaccinated group, but no zoster in the vaccinated group. CONCLUSIONS: The varicella vaccine was well tolerated in SLE group, who had pre-existing immunity to varicella. The varicella vaccine immunogenicity measurement by serum antibody titres was appropriate. The incidence of HZ was lower in the vaccinated lupus group.

Citrus By-Products: Valuable Source of Bioactive Compounds for Food Applications.

Citrus production produces about 15 million tons of by-products/waste worldwide every year. Due to their high content of bioactive compounds, several extraction techniques can be applied to obtain extracts rich in valuable compounds and further application into food applications. Distillation and solvent extraction continues to be the most used and applied extraction techniques, followed by newer techniques such as microwave-assisted extraction and pulsed electric field extraction. Although the composition of these extracts and essential oils directly depends on the edaphoclimatic conditions to which the fruit/plant was exposed, the main active compounds are D-limonene, carotenoids, and carbohydrates. Pectin, one of the most abundant carbohydrates present in Citrus peels, can be used as a biodegradable polymer to develop new food packaging, and the extracted bioactive compounds can be easily added directly or indirectly to foods to increase their shelf-life. One of the applications is their incorporation in active food packaging for microbiological and/or oxidation inhibition, prolonging foods' shelf-life and, consequently, contributing to reducing food spoilage. This review highlights some of the most used and effective extraction techniques and the application of the obtained essential oils and extracts directly or indirectly (through active packaging) to foods.

Industrial Fruits By-Products and Their Antioxidant Profile: Can They Be Exploited for Industrial Food Applications?

Fruit by-products have a low economic value and have proven biological activities, such as antioxidant capacity due to the presence of active compounds. The main objective of this study was to obtain and determine the antioxidant capacity, through DPPH radical assay and ß-carotene bleaching assay, of three food grade extracts from apple, lemon, and orange industrial by-products. Furthermore, the extracts were characterized by ultra-high performance liquid chromatography coupled to mass spectrometry (UHPLC-MS/MS). LC with diode array detector (LC-DAD) was used for the quantification of the main polyphenols. Lemon extract presented the highest inhibition percentage of DPPH radical (51.7%) and the highest total phenolics content (43.4 mg GAE/g) from the by-products studied. Orange by-product was that with the higher number of polyphenols while lemon extract was that with the highest content of individual phenolics. The by-product obtained from the lemon was that with higher amounts of hydroxycinnamic acids (407 µg/g of by-product), mainly chlorogenic acid (386.7 µg/g), followed by the apple by-product (128.0 µg/g of by-product), which showed higher amounts of rosmarinic and chlorogenic acids. These industrial by-products have great potential as a source of natural antioxidants to be used directly as food additives or to be incorporated in packaging to produce active food packaging.

A New Insight on Cardoon: Exploring New Uses besides Cheese Making with a View to Zero Waste.

Cardoon, Cynara cardunculus L., is a perennial plant whose flowers are used as vegetal rennet in cheese making. Cardoon is native from the Mediterranean area and is commonly used in the preparation of salads and soup dishes. Nowadays, cardoon is also being exploited for the production of energy, generating large amount of wastes, mainly leaves. These wastes are rich in bioactive compounds with important health benefits. The aim of this review is to highlight the main properties of cardoon leaves according to the current research and to explore its potential uses in different sectors, namely the food industry. Cardoon leaves are recognized to have potential health benefits. In fact, some studies indicated that cardoon leaves could have diuretic, hepato-protective, choleretic, hypocholesterolemic, anti-carcinogenic, and antibacterial properties. Most of these properties are due to excellent polyphenol profiles, with interesting antioxidant and antimicrobial activities. These findings indicate that cardoon leaves can have new potential uses in different sectors, such as cosmetics and the food industry; in particular, they can be used for the preparation of extracts to incorporate into active food packaging. In the future, these new uses of cardoon leaves will allow for zero waste of this crop.

Safety and immunogenicity of the quadrivalent human papillomavirus vaccine in patients with juvenile dermatomyositis: a real-world multicentre study.

BACKGROUND: Concerns about the safety and efficacy of vaccines in patients with autoimmune diseases (AID) have led to contradictions and low vaccination coverage in this population, who are at a higher risk of infections, including by human papillomavirus (HPV). Although HPV vaccines have been recommended for immunocompromised patients, there is still a lack of data to support its use for AID patients, such as juvenile dermatomyositis (JDM) patients. The aim of this study was to assess the safety and immunogenicity of the quadrivalent HPV (qHPV) vaccine in a cohort of JDM patients. METHODS: JDM patients aged from 9 to 20 years and healthy controls (HC) were enrolled to receive a 3-dose schedule of qHPV vaccine from March/2014 to March/2016. Study visits were performed before the first dose, 1 month after the second and third doses, and 6 months after the third dose. Participants completed a diary of possible adverse events for 14 days following each dose of vaccination (AEFV). Disease activity and current therapy were analyzed at each visit for JDM patients. In addition, serum samples from all participants were collected to test antibody concentrations against HPV16 and 18 at each visit. Participant recruitment was conducted in ten Brazilian centres. From 47 eligible JDM patients and 41 HC, 42 and 35, respectively, completed the 3-dose schedule of the vaccine, given that five JDM patients and two HC had received doses prior to their inclusion in the study. RESULTS: The AEFVs presented by the participants were mild and in general did not differ between JDM and HC groups. No severe AEFVs were related to the vaccination. Disease activity was stable, or even improved during the follow-up. One month after the third dose of the vaccine the JDM group presented seropositivity of 100% for HPV16 and 97% for HPV18, similarly to the HC group, who presented 100% for both serotypes (p = 1.000). Six months after the third dose the seropositivity for the patient group was 94% for both HPV types. CONCLUSIONS: The HPV vaccination in this cohort of JDM patients was safe and immunogenic. Since the seropositivity against HPV16 and 18 was very high after the 3-dose schedule, this regimen should be recommended for JDM patients. TRIAL REGISTRATION: Brazilian Clinical Trials Registry, number: RBR-9ypbtf . Registered 20 March 2018 - Retrospectively registered.

Idiopathic uveitis presents different characteristics and outcome from juvenile idiopathic arthritis- related uveitis / Uveíte idiopática apresenta características e desfecho diferentes da uveíte associada à Artrite Idiopática Juvenil

ABSTRACT Objective: To describe the clinical features and outcomes of patients with uveitis associated with juvenile idiopathic arthritis (JIA) and idiopathic uveitis. Methods: This was an observational, retrospective study, conducted in a tertiary center. Patients under 18 years old who experienced at least one episode of uveitis and followed between 2000 and 2019 were included. Results: A total of 82 patients were included, of whom 43 had idiopathic uveitis and 39 had uveitis associated with JIA. Anterior uveitis was the primary site of ocular inflammation (76.8%) and occurred in 24 and 39 patients with idiopathic uveitis and uveitis associated with JIA arthritis, respectively (p=0.02). The complete response to corticotherapy was more frequent in uveitis associated with JIA (p=0.001). Total and partial responses to biological disease modifying antirheumatic drugs were more frequent in uveitis associated with JIA (p=0.025) and idiopathic uveitis (p=0.045), respectively. There were 203 complications: cataracts were more frequently present in idiopathic uveitis (p=0.05), while synechiae was more frequent in uveitis associated with JIA (p=0.02). Conclusion: Idiopathic uveitis and uveitis associated JIA frequently follow a chronic course and an increased risk of visual loss in childhood. The uveitis associated with JIA showed better response to systemic corticotherapy and total response to biologic disease modifying antirheumatic drugs more frequently.

RESUMO Objetivos: Descrever as características clínicas e desfechos dos pacientes com uveíte associada à Artrite Idiopática Juvenil (AIJ) e da Uveíte Idiopática. Métodos: Este foi um estudo retrospectivo observacional conduzido em um centro terciário. Foram incluídos pacientes abaixo dos 18 anos de idade que apresentaram pelo menos um episódio de uveíte e que estiveram em acompanhamento médico entre os anos de 2000 e 2019. Resultados: Foram incluídos 82 pacientes, sendo 43 com uveíte idiopática e 39 com uveíte associada à AIJ. A uveíte anterior foi o sítio primário de acometimento (76,8%) em 24 e 39 pacientes com uveíte idiopática e uveíte associada à AIJ, respectivamente (p=0.02). Resposta total à corticoterapia foi mais frequente na uveíte associada à AIJ (p=0.001). Respostas total e parcial às drogas antirreumáticas modificadoras de doença biológicas foram mais frequentes na uveíte associada à AIJ (p=0.025) e na uveíte idiopática (p=0.045), respectivamente. Foram encontradas 203 complicações: a catarata foi mais frequente na uveíte idiopática (p=0.05), enquanto a sinéquia foi mais frequente na uveíte associada à AIJ (p=0.02). Conclusão: A uveíte idiopática e a uveíte associada à AIJ frequentemente apresentam um curso crônico e um risco elevado de perda visual na infância. A uveíte associada à AIJ apresentou melhor resposta à corticoterapia sistêmica e resposta total às drogas modificadoras de doença reumática biológicas mais frequentemente.

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.

Stevens-Johnson syndrome and toxic epidermal necrolysis in childhood-onset systemic lupus erythematosus patients: a multicenter study.

OBJECTIVE: To assess Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in a large population of childhood-onset systemic lupus erythematosus (cSLE) patients. METHODS: Multicenter study including 852 cSLE patients followed in Pediatric Rheumatology centers in São Paulo, Brazil. SJS was defined as epidermal detachment below 10% of body surface area (BSA), overlap SJS-TEN 10-30% and TEN greater than 30% of BSA. RESULTS: SJS and TEN was observed in 5/852 (0.6%) cSLE female patients, three patients were classified as SJS and two patients were classified as overlap SJS-TEN; TEN was not observed. The mean duration of SJS and overlap SJS-TEN was 15 days (range 7-22) and antibiotics induced four cases. Regarding extra-cutaneous manifestations, hepatomegaly was observed in two cSLE patients, nephritis in two and neuropsychiatric involvement and conjunctivitis were observed respectively in one patient. Hematological involvement included lymphopenia in four, leucopenia in three and thrombocytopenia in two patients. The mean SLEDAI-2K score was 14.8 (range 6-30). Laboratory analysis showed low C3, C4 and/or CH50 in two patients and the presence of anti-dsDNA autoantibody in two patients. One patient had lupus anticoagulant and another one had anticardiolipin IgG. All patients were treated with steroids and four needed additional treatment such as intravenous immunoglobulin in two patients, hydroxychloroquine and azathioprine in two and intravenous cyclophosphamide in one patient. Sepsis was observed in three cSLE patients. Two patients required intensive care and death was observed in one patient. CONCLUSION: Our study identified SJS and overlap SJS-TEN as rare manifestations of active cSLE associated with severe multisystemic disease, with potentially lethal outcome.

Anti-RO/SSA and anti-La/SSB antibodies: Association with mild lupus manifestations in 645 childhood-onset systemic lupus erythematosus.

BACKGROUND: To our knowledge there are no studies assessing anti-Ro/SSA and anti-La/SSB autoantibodies in a large population of childhood-systemic lupus erythematosus (cSLE) patients. METHODS: This was a retrospective multicenter cohort study performed in 10 Pediatric Rheumatology services, São Paulo state, Brazil. Anti-Ro/SSA and anti-La/SSB antibodies were measured by enzyme linked immunosorbent assay (ELISA) in 645 cSLE patients. RESULTS: Anti-Ro/SSA and anti-La/SSB antibodies were evidenced in 209/645 (32%) and 102/645 (16%) of cSLE patients, respectively. Analysis of cSLE patients with and without anti-Ro/SSA antibodies revealed higher frequencies of malar rash (79% vs. 71%, p=0.032), photosensitivity (73% vs. 65%, p=0.035), cutaneous vasculitis (43% vs. 35%, p=0.046) and musculoskeletal involvement (82% vs. 75%, p=0.046) in spite of long and comparable disease duration in both groups (4.25 vs. 4.58years, p=0.973). Secondary Sjögren syndrome was observed in only five patients with this antibody (2.5% vs. 0%, p=0.0035), two of them with concomitant anti-La/SSB. The presence of associated autoantibodies: anti-Sm (50% vs. 30%, p<0.0001), anti-RNP (39% vs. 21%, p<0.0001) and anti-ribossomal P protein (46% vs. 21%, p=0.002) was also significantly higher in patients with anti-Ro/SAA antibodies. Further evaluation of cSLE patients with the presence of anti-La/SSB antibodies compared to those without these autoantibodies showed that the frequency of alopecia (70% vs. 51%, p=0.0005), anti-Sm (59% vs. 31%, p<0.0001) and anti-RNP (42% vs. 23%, p<0.0001) were significantly higher in the former group. CONCLUSIONS: Our large multicenter cohort study provided novel evidence in cSLE that anti-Ro/SSA and/or anti-La/SSB antibodies were associated with mild manifestations, particularly cutaneous and musculoskeletal. Secondary Sjögren syndrome was rarely observed in these patients, in spite of comparable frequencies of anti-Ro/SSA and/or anti-La/SSB reported for adult SLE.

Juvenile fibromyalgia syndrome: Blunted heart rate response and cardiac autonomic dysfunction at diagnosis.

OBJECTIVE: To assess aerobic capacity and cardiac autonomic modulation in juvenile fibromyalgia syndrome (JFM) patients at diagnosis in response to graded exercise text. METHODS: A multicenter cross-sectional study included 25 JFM patients and 25 healthy controls. Both groups participated only in physical education classes at school. A treadmill graded cardiorespiratory test was performed and the heart-rate (HR) response during exercise was evaluated by the chronotropic reserve (CR). Pain, functional ability, and health-related quality of life (HRQL) were assessed. RESULTS: The median current age was similar in JFM and controls (15 vs. 15 years, p = 0.890), as well as body mass index (p = 0.332), female gender (p = 1.000), and Tanner stages (p = 0.822). The medians of HRQL parameters (total score/physical health/psychosocial health) were significantly lower in JFM vs. controls according to patient and parent self-reports (p < 0.001). The median of peak HR [181 (150-198) vs. 197 (181-202)bpm, p < 0.001], chronotropic reserve [84 (53-98) vs. 99 (84-103)%, p < 0.001], and resting to peak [96 (65-181) vs. 127 (61-185)bpm, p = 0.010] were significantly lower in JFM compared to controls. The median of ΔHRR1 [15 (3-39) vs. 35 (9-52)bpm, p < 0.001], ΔHRR2 [37 (20-57) vs. 51 (32-94)bpm, p < 0.001], peak VO2 [32.34 (24.24-39.65) vs. 36.4 (28.56-52.71)ml/kg/min, p = 0.005], peak speed [5 (4-6.3) vs. 5.9 (4.0-6.3)mph, p = 0.001], time to exhaustion [11.5 (8.5-14.5) vs. 14 (11-18)min, p < 0.001], and working capacity on power [3.37 (2.04-5.6) vs. 3.89 (2.91-6.55)W/kg, p = 0.006] were significantly lower in JFM compared to controls. The frequency of chronotropic incompetence (≤80%) was significantly higher in JFM vs. controls (p = 0.0006). CONCLUSIONS: This study identified chronotropic incompetence and delayed HR recovery in JFM patients, indicating autonomic dysfunction. Aerobic exercise training should be considered in all JFM patients and may improve cardiac autonomic impairment, thus reducing cardiovascular risk.

Features of 847 Childhood-Onset Systemic Lupus Erythematosus Patients in Three Age Groups at Diagnosis: A Brazilian Multicenter Study.

OBJECTIVE: To evaluate demographic data and clinical and laboratory features at disease diagnosis in 3 different age groups of childhood-onset systemic lupus erythematosus (SLE): group A, early-onset (<6 years); group B, school age (≥6 to <12 years); and group C, adolescent (≥12 to <18 years). METHODS: This was a Brazilian multicenter cohort retrospective study in 10 pediatric rheumatology centers, including 847 childhood-onset SLE patients. RESULTS: Patients were divided into 3 groups: group A with 39 patients (4%), group B with 395 patients (47%), and group C with 413 patients (49%). Of 39 childhood-onset SLE patients in group A, 3 (8%) were ages <2 years, 4 (10%) were ≥2 to <3 years, and 32 (82%) were ≥3 and <6 years. A total of 74 childhood-onset SLE patients were analyzed for C1q levels, and complete C1q deficiency was observed in 3 of 74 patients (4%), all in group A. Groups were similar regarding high frequencies of female sex, nephritis, neuropsychiatric involvement, Systemic Lupus Erythematosus Disease Activity Index 2000 score ≥8, autoantibody profile, elevated acute phase proteins, and low complement levels (P > 0.05). However, the frequency of fever (78% versus 61% versus 47%; P < 0.0001), hepatomegaly (42% versus 29% versus 14%; P < 0.0001), splenomegaly (28% versus 12% versus 4%; P < 0.0001), and discoid lupus (13% versus 4% versus 4%; P = 0.020) was significantly higher in group A compared to groups B and C. The frequency of weight loss >2 kg (19% versus 28% versus 36%; P = 0.017), photosensitivity (34% versus 41% versus 51%; P = 0.006), leukopenia <4,000/mm3 (14% versus 25% versus 30%; P = 0.048), and lymphopenia <1,500/mm3 (22% versus 41% versus 47%; P = 0.011) was significantly lower in group A. CONCLUSION: Our large multicenter study identified the finding that the initial appearance of childhood-onset SLE is characterized by comparable high frequency of internal organ involvement and some distinct clinical and laboratory features in early-onset and adolescent groups.

Diagnosis of malignancies in children with musculoskeletal complaints.

CONTEXT: Musculoskeletal complaints may be associated with neoplasias as an initial manifestation of the disease. When these symptoms predominate at the onset of the disease, the differential diagnosis includes several rheumatic diseases. OBJECTIVE: To assess the frequency, clinical features and types of cancer manifested in children presenting with musculoskeletal complaints over a seven-year period. TYPE OF STUDY: Retrospective. SETTING: Discipline of Allergy, Clinical Immunology and Rheumatology, Universidade Federal de São Paulo-Escola Paulista de Medicina. METHODS: The medical records of patients with musculoskeletal complaints and final diagnosis of malignant disease were reviewed. The data collected were: age when symptoms initially presented, age at diagnosis, clinical features presented, laboratory findings, and the initial and final diagnoses. RESULTS: A final diagnosis of cancer was found in nine out of 3,528 patients (0.25%) whose initial symptom was musculoskeletal pain. The mean time between disease onset and final diagnosis was five months. The most common features presented were pauciarticular arthritis or arthralgia involving the large joints. Juvenile rheumatoid arthritis was the most frequent initial diagnosis, in four out of nine patients. Anemia was the most frequent initial hematological change. Six out of eight patients had an increased erythrocyte sedimentation rate. The lactate dehydrogenase level was raised in five out of eight patients. The malignancies found included acute lymphocytic leukemia, acute myeloid leukemia, lymphoma, neuroblastoma and Ewing's sarcoma. DISCUSSION: The frequency of neoplasia in patients with musculoskeletal pain resembled reports in the literature. Consumptive symptoms were not the warning signal in most of our patients. In subsidiary tests, progressive anemia was the most common finding, although the peripheral blood cell count may continue to be normal for weeks or months after symptom onset. CONCLUSION: Malignancy always needs to be ruled out in cases of children with musculoskeletal complaints. Uncharacteristic clinical manifestations and nonspecific laboratory tests may cause difficulty in the final diagnosis, and rigorous investigation should be performed.

Rituximab use in young adults diagnosed with juvenile idiopathic arthritis unresponsive to conventional treatment: report of 6 cases.

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood. Without an effective therapy, patients may progress quickly to functional disability. Recently, depletion of B cells emerged as a new approach for the treatment of autoimmune diseases, including JIA. We describe six cases of JIA patients followed at a referral center for Rheumatology and Pediatric Rheumatology, submitted to treatment with rituximab (RTX) after refractoriness to three anti-TNF agents. Patients received RTX cycles with two infusions every six months. Response to treatment was assessed by DAS28, HAQ/CHAQ, and an overall assessment by the doctor and the patient. Of our six patients, four were girls (mean age at onset of disease: 6.1 years; mean disease evolution time: 15.1 years; mean age upon receiving RTX: 21.6 years). Four patients belonged to polyarticular subtype (1 rheumatoid factor [RF]-negative, 3 FR-positive), a patient with systemic JIA subtype with a polyarticular course and arthritis related to enthesitis. Of our six patients, five responded to treatment; and during the course of 12 months, the clinical response was maintained, although not sustained. However, discontinuation by infusion reactions caused the withdrawal of RTX in two patients. The use of RTX in JIA is restricted to cases refractory to other biological agents and, even considering that this study was held in a small number of advanced patients, RTX proved to be an effective therapeutic option.

A Multicenter Study of Invasive Fungal Infections in Patients with Childhood-onset Systemic Lupus Erythematosus.

OBJECTIVE: To study the prevalence, risk factors, and mortality of invasive fungal infections (IFI) in patients with childhood-onset systemic lupus erythematosus (cSLE). METHODS: A retrospective multicenter cohort study was performed in 852 patients with cSLE from 10 pediatric rheumatology services. An investigator meeting was held and all participants received database training. IFI were diagnosed according to the European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group Consensus Group criteria (proven, probable, and possible). Also evaluated were demographic, clinical, and laboratory data, and disease activity [SLE Disease Activity Index 2000 (SLEDAI-2K)], cumulative damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index), treatment, and outcomes. RESULTS: IFI were observed in 33/852 patients (3.9%) with cSLE. Proven IFI was diagnosed in 22 patients with cSLE, probable IFI in 5, and possible IFI in 6. Types of IFI were candidiasis (20), aspergillosis (9), cryptococcosis (2), and 1 each disseminated histoplasmosis and paracoccidioidomycosis. The median of disease duration was lower (1.0 vs 4.7 yrs, p < 0.0001) with a higher current SLEDAI-2K [19.5 (0-44) vs 2 (0-45), p < 0.0001] and current prednisone (PRED) dose [50 (10-60) vs 10 (2-90) mg/day, p < 0.0001] in patients with IFI compared with those without IFI. The frequency of death was higher in the former group (51% vs 6%, p < 0.0001). Logistic regression analysis revealed that SLEDAI-2K (OR 1.108, 95% CI 1.057-1.163, p < 0.0001), current PRED dose (OR 1.046, 95% CI 1.021-1.071, p < 0.0001), and disease duration (OR 0.984, 95% CI 0.969-0.998, p = 0.030) were independent risk factors for IFI (R(2) Nagelkerke 0.425). CONCLUSION: To our knowledge, this is the first study to characterize IFI in patients with cSLE. We identified that disease activity and current glucocorticoid use were the main risk factors for these life-threatening infections, mainly in the first years of disease course, with a high rate of fatal outcome.

[Musculoskeletal manifestations as the onset of acute leukemias in childhood]. / Manifestações músculo-esqueléticas como apresentação inicial das leucemias agudas na infância.

OBJECTIVE: To study the frequency, the clinical features and laboratory exams of patients with musculoskeletal symptoms at the onset of acute leukemia. METHODS: Retrospective, descriptive study including patients diagnosed with acute leukemia, and treated at the Institute of Pediatric Oncology of UNIFESP, carried out from November 1999 to February 2000. The data on musculoskeletal complaints were obtained from a questionnaire. The medical records were revised in order to get data on the clinical examination and laboratory tests at the beginning of the illness. RESULTS: Sixty-one children were included in this trial, 93% with acute lymphoid leukemia, and 7% with acute myeloid leukemia. Thirty-eight patients (62%) had musculoskeletal pain at the onset. Arthritis was observed in eight patients. The mean number of involved joints was 2.5 (1-6). The most frequently involved joints were the knees, ankles and elbows. Three patients (4.9%) had normal blood count. Low hemoglobin was reported in 54 patients (88%) (in six patients it was the only hematological abnormality), leukopenia in 14 (22%), leukocytosis in 26 (42%) and low platelet count in 46 (75%). CONCLUSIONS: The musculoskeletal symptoms are common at the onset of acute leukemia so, malignancy should always be ruled out in patients presenting chronic or acute arthritis or benign limb pain. The laboratory tests may be normal at the onset of the illness, making differential diagnosis more difficult.

Percepção e Satisfação da Imagem Corporal em Adolescentes de Escolas Estaduais do Estado de Sergipe / Perception and Satisfaction of Body Image in Adolescents of State Schools of the State of Sergipe

Introdução: A adolescência é uma fase da vida, que é caracterizada por várias mudanças, sendo elas, corporais, comportamentais e sociais. Dentro destas mudanças, estão as alimentares, que influenciam no estado nutricional e corporal do indivíduo. Também se deve levar em consideração a forma como o adolescente vê sua imagem corporal. Objetivo: Avaliar o estado nutricional e fazer a análise da satisfação corporal de 371 adolescentes da cidade de AracajuSE, traçando relações com idade, sexo e prática de atividades físicas. Metodologia: O estudo transversal foi realizado em 372 adolescentes, de ambos os sexos, com idade de 10 a 18 anos, em escolas estaduais da cidade de Aracaju-SE. Para a avaliação do estado nutricional, foram feitas as medidas antropométricas, para classificação do IMC e para a análise da satisfação corporal, foi aplicado o Body Shape Questionnaire (BSQ). Resultados: Dos 371 pesquisados, 67,4% estavam em estado eutrófico, representando a maioria. Quanto à satisfação com o estado corporal, de todos pesquisados, 87,9% estavam satisfeitos. Conclusão: Percebeu-se que muitos que estavam em estado nutricional, fora do esperado, estavam satisfeitos com o corpo

Introduction: Adolescence is a phase of life, which is characterized by various changes, being, bodily, behavioral and social. Within these changes, are the alimentary, that influence in the nutritional and corporal state of the individual. Consideration should also be given to how the adolescent sees his or her body image. Objective: To evaluate the nutritional status and analyze the corporal satisfaction of 371 adolescents from the city of Aracaju-SE, drawing relationships with age, sex and physical activity practice. Methods: The cross-sectional study was performed in 371 adolescents of both sexes, aged 10 to 18 years, in state schools in the city of Aracaju-SE. For the assessment of nutritional status, anthropometric measurements were made, for BMI classification and for body satisfaction analysis, the Body Shape Questionnaire (BSQ) was applied. Results: Of the 371 studied, 67.4% were in the eutrophic state, representing the majority. Regarding the satisfaction with body condition, of all surveyed, 87.9% were satisfied. It was noticed that many who were in nutritional status, beyond expected, were satisfied with the body. Conclusion: Many who were in the nutritional state, outside of the expected, were satisfied with the body

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Profile of paediatric rheumatology specialists and services in the state of São Paulo.

INTRODUCTION: Paediatric rheumatology (PR) is an emerging specialty, practised by a limited number of specialists. Currently, there is neither a record of the profile of rheumatology patients being treated in Brazil nor data on the training of qualified rheumatology professionals in the country. OBJECTIVE: To investigate the profile of PR specialists and services, as well as the characteristics of paediatric patients with rheumatic diseases, for estimating the current state of rheumatology in the state of São Paulo. PATIENTS AND METHODS: In 2010, the scientific department of PR of the Paediatric Society of São Paulo administered a questionnaire that was answered by 24/31 accredited specialists in PR practising in state of São Paulo and by 8/21 institutions that provide PR care. RESULTS: Most (91%) of the surveyed professionals practise in public institutions. Private clinics (28.6%) and public institutions (37.5%) reported not having access to nailfold capillaroscopy, and 50% of the private clinics reported not having access to acupuncture. The average duration of professional practise in PR was 9.4 years, and 67% of the physicians had attended postgraduate programmes. Seven (87.5%) public institutions perform teaching activities, in which new paediatric rheumatologists are trained, and five (62.5%) offer postgraduate programmes. Two-thirds of the surveyed specialists use immunosuppressants and biological agents classified as "restricted use" by the Health Secretariat. The disease most frequently reported was juvenile idiopathic arthritis (29.1-34.5%), followed by juvenile systemic lupus erythematosus (JSLE) (11.6-12.3%) and rheumatic fever (9.1-15.9%). The incidence of vasculitis (including Henoch-Schönlein purpura, Wegener's granulomatosis, and Takayasu's arteritis) and autoinflammatory syndromes was higher in public institutions compared to other institutions (P = 0.03, P = 0.04, P = 0.002, and P < 0.0001, respectively). Patients with JSLE had the highest mortality rate (68% of deaths), mainly due to infection. CONCLUSION: The field of PR in the state of São Paulo has a significant number of specialists with postgraduate degrees who mostly practise at teaching institutions with infrastructures appropriate for the care of high-complexity patients.