Participating as a research team during the COVID-19 pandemic benefits mental health of undergraduate medical students in Brazil.

During the COVID-19 pandemic, undergraduate medical students (UMS) exposed to isolation, social distancing and complete or partial face-to-face educational activities interruption may present increased stress, depression and anxiety. This study was undertaken to evaluate if, during isolation, UMS involved in online group activities as investigators of a research project (volunteer group) would present better mental health than their colleagues, not involved in that research (control group). A Web-based survey, via the Google Forms platform, including details on demographic data, life habits, previous health conditions, worries with the COVID-19 pandemic, sleep pattern modifications and depression, anxiety and mental stress, using the DASS-21 (Depression, Anxiety and Stress Scale) was implemented from 20 July to 31 August 2020. Statistical analysis was performed using the SPSS version 20.0. A p-value <0.05 was significant. A total of 684 UMS were included, 228 as a volunteer group and 456 as a control group. Mean age was 23.15 (3.16) years. The groups were paired for age, gender, ethnicity, life habits and previous health conditions. Older age, male gender, participation in the research project, unchanged sleep pattern during the pandemic, lack of fear from getting the COVID-19 and lack of previous health conditions were associated with lower DASS21 scores (better mental health). Participating as investigators of a research project foreseeing frequent interaction with patients, colleagues and professors (other investigators) lead to better mental health during the COVID-19 quarantine in Brazil.

How Did Patients Living With Immune-Mediated Rheumatic Diseases Face the Beginning of the COVID-19 Pandemic in Brazil? Results of the COnVIDa Study.

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic has brought additional burden to patients living with immune-mediated rheumatic diseases (IMRDs), especially at the beginning of 2020, for which information for this population is lacking. METHODS: COnVIDa is a cross-sectional study on patients with IMRD from all regions of Brazil who were invited to answer a specific and customized Web questionnaire about how they were facing the COVID-19 pandemic, especially focusing on health care access, use of medications, and patient-reported outcomes related to IMRD activity. The questionnaire was applied from June 1 to 30, 2020. RESULTS: In total, 1722 of 2576 patients who answered the Web questionnaire were included in the final analysis. Participants were most frequently women, 56% were between 31 and 50 years old, and most (55%) has private health insurance. The most commonly reported IMRD was rheumatoid arthritis (39%), followed by systemic lupus erythematosus (28%). During the study period, 30.7% did not have access to rheumatology consultations, and 17.6% stopped chronic medications. Telemedicine was reported in 44.8% of patients. CONCLUSION: COnVIDa demonstrated a negative impact on health care access and treatment maintenance of patients living with IMRD during the COVID-19 pandemic. However, it also presented an uptake of telemedicine strategies. Data presented in this study may assist future coping policies.

Incidence and risk factors for moderate/severe COVID-19 in rheumatic diseases patients on hydroxychloroquine: a 24-week prospective cohort.

OBJECTIVES: To evaluate the incidence of COVID-19 and its main outcomes in rheumatic disease (RD) patients on hydroxychloroquine (HCQ) compared to household cohabitants (HC). METHODS: This is a 24-week nationwide prospective multi-centre cohort with a control group without RD and not using HCQ. All participants were monitored through scheduled phone interviews performed by health professionals. Details regarding COVID-19 symptoms, and epidemiological, clinical, and demographic data were recorded on a specific web-based platform. COVID-19 was defined according to the Brazilian Ministry of Health criteria and classified as mild, moderate or severe. RESULTS: A total of 9,585 participants, 5,164 (53.9%) RD patients on HCQ and 4,421 (46.1%) HC were enrolled from March 29th, 2020 to September 30th, 2020, according to the eligibility criteria. COVID-19 confirmed cases were higher in RD patients than in cohabitants [728 (14.1%) vs. 427 (9.7%), p<0.001] in a 24-week follow-up. However, there was no significant difference regarding outcomes related to moderate/ severe COVID-19 (7.1% and 7.3%, respectively, p=0.896). After multiple adjustments, risk factors associated with hospitalisation were age over 65 (HR=4.5; 95%CI 1.35-15.04, p=0.014) and cardiopathy (HR=2.57; 95%CI 1.12-5.91, p=0.026). The final survival analysis demonstrated the probability of dying in 180 days after a COVID-19 diagnosis was significantly higher in patients over 65 years (HR=20.8; 95%CI 4.5-96.1) and with 2 or more comorbidities (HR=10.8; 95%CI 1.1-107.9 and HR=24.8; 95%CI 2.5-249.3, p=0.006, respectively). CONCLUSIONS: Although RD patients have had a higher COVID-19 incidence than individuals from the same epidemiological background, the COVID-19 severity was related to traditional risk factors, particularly multiple comorbidities and age, and not to underlying RD and HCQ.

Recommendations of the Brazilian Society of Rheumatology for the use of JAK inhibitors in the management of rheumatoid arthritis.

Rheumatoid arthritis (RA) is a chronic and autoimmune systemic inflammatory disease that can cause irreversible joint deformities, with increased morbidity and mortality and a significant impact on the quality of life of the affected individual. The main objective of RA treatment is to achieve sustained clinical remission or low disease activity. However, up to 40% of patients do not respond to available treatments, including bDMARDs. New therapeutic targets for RA are emerging, such as Janus kinases (JAKs). These are essential for intracellular signaling (via JAK-STAT) in response to many cytokines involved in RA immunopathogenesis. JAK inhibitors (JAKi) have established themselves as a highly effective treatment, gaining increasing space in the therapeutic arsenal for the treatment of RA. The current recommendations aim to present a review of the main aspects related to the efficacy and safety of JAKis in RA patients, and to update the recommendations and treatment algorithm proposed by the Brazilian Society of Rheumatology in 2017.

Chronic use of hydroxychloroquine did not protect against COVID-19 in a large cohort of patients with rheumatic diseases in Brazil.

BACKGROUND: There is a lack of information on the role of chronic use of hydroxychloroquine during the SARS-CoV-2 outbreak. Our aim was to compare the occurrence of COVID-19 between rheumatic disease patients on hydroxychloroquine with individuals from the same household not taking the drug during the first 8 weeks of community viral transmission in Brazil. METHODS: This baseline cross-sectional analysis is part of a 24-week observational multi-center study involving 22 Brazilian academic outpatient centers. All information regarding COVID-19 symptoms, epidemiological, clinical, and demographic data were recorded on a specific web-based platform using telephone calls from physicians and medical students. COVID-19 was defined according to the Brazilian Ministry of Health (BMH) criteria. Mann-Whitney, Chi-square and Exact Fisher tests were used for statistical analysis and two binary Final Logistic Regression Model by Wald test were developed using a backward-stepwise method for the presence of COVID-19. RESULTS: From March 29th to May 17st, 2020, a total of 10,443 participants were enrolled, including 5166 (53.9%) rheumatic disease patients, of whom 82.5% had systemic erythematosus lupus, 7.8% rheumatoid arthritis, 3.7% Sjögren's syndrome and 0.8% systemic sclerosis. In total, 1822 (19.1%) participants reported flu symptoms within the 30 days prior to enrollment, of which 3.1% fulfilled the BMH criteria, but with no significant difference between rheumatic disease patients (4.03%) and controls (3.25%). After adjustments for multiple confounders, the main risk factor significantly associated with a COVID-19 diagnosis was lung disease (OR 1.63; 95% CI 1.03-2.58); and for rheumatic disease patients were diagnosis of systemic sclerosis (OR 2.8; 95% CI 1.19-6.63) and glucocorticoids above 10 mg/ day (OR 2.05; 95% CI 1.31-3.19). In addition, a recent influenza vaccination had a protective effect (OR 0.674; 95% CI 0.46-0.98). CONCLUSION: Patients with rheumatic disease on hydroxychloroquine presented a similar occurrence of COVID-19 to household cohabitants, suggesting a lack of any protective role against SARS-CoV-2 infection. Trial registration Brazilian Registry of Clinical Trials (ReBEC; RBR - 9KTWX6).

Effects of Participating in a Research Project During the COVID-19 Pandemic on Medical Students' Educational Routines and Mental Health: Protocol for a Web-Based Survey Study.

BACKGROUND: The COVID-19 pandemic has resulted in social isolation, which has a potential negative impact on the educational routines (eg, the suspension of face-to-face appointments) and mental health of medical students. The Mario Pinotti II (MPII) study is a 24-week observational study that conducted scheduled telephone calls every 2 weeks to verify the occurrence of COVID-19 in patients with rheumatic diseases on chronic hydroxychloroquine therapy (from March 29, 2020, to September 30, 2020). The effects of voluntarily participating in a research project (ie, one that involves interactions via telephone contact with patients, professors, rheumatologists, and colleagues) on the daily lives and mental health of medical students requires evaluation. OBJECTIVE: As medical students are professionals in training and have a high level of responsibility in terms of handling the emotional and physical aspects of several diseases, this study aims to evaluate the impacts of the COVID-19 pandemic and participation in the MPII study on the educational routines and mental health of medical students. METHODS: A web-based survey was carried out to perform a cross-sectional comparative assessment of medical students who participated in the MPII study and their colleagues who were not involved in the MPII study. Participants from both groups were matched based on sex, age, and medical school. The web questionnaire was developed by a panel composed of graduate medical students, rheumatologists, medical school professors, and a psychology professor. The questionnaire included details on demographic and life habits data and evaluated participants' impressions of the MPII study and the impact of the COVID-19 pandemic on their educational routines and medical training. In addition, depression, anxiety, and stress were evaluated using the Brazilian version of the Depression, Anxiety, and Stress Scale (DASS)-21, and currently, the DASS-21 scores are grouped as those that indicate a low, moderate, or high risk of mental distress. This project was approved by the Federal University of São Paulo Ethics Committee (CAAE: 34034620.0.0000.5505). RESULTS: Data were collected from both medical student groups from July 20 to August 31, 2020. Data extraction was completed in September 2020. The data analysis is ongoing. We expect the results to be published in the first semester of 2021. CONCLUSIONS: This study will provide insight into the effects of participating in a research project on depression, anxiety, and stress, which will be determined by applying the DASS-21 to a large sample of Brazilian undergraduate medical students. We will also evaluate the impact of the COVID-19 pandemic on medical students' educational routines and medical training. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/24617.

High levels of immunosuppression are related to unfavourable outcomes in hospitalised patients with rheumatic diseases and COVID-19: first results of ReumaCoV Brasil registry.

OBJECTIVES: To evaluate risk factors associated with unfavourable outcomes: emergency care, hospitalisation, admission to intensive care unit (ICU), mechanical ventilation and death in patients with immune-mediated rheumatic disease (IMRD) and COVID-19. METHODS: Analysis of the first 8 weeks of observational multicentre prospective cohort study (ReumaCoV Brasil register). Patients with IMRD and COVID-19 according to the Ministry of Health criteria were classified as eligible for the study. RESULTS: 334 participants were enrolled, a majority of them women, with a median age of 45 years; systemic lupus erythematosus (32.9%) was the most frequent IMRD. Emergency care was required in 160 patients, 33.0% were hospitalised, 15.0% were admitted to the ICU and 10.5% underwent mechanical ventilation; 28 patients (8.4%) died. In the multivariate adjustment model for emergency care, diabetes (prevalence ratio, PR 1.38; 95% CI 1.11 to 1.73; p=0.004), kidney disease (PR 1.36; 95% CI 1.05 to 1.77; p=0.020), oral glucocorticoids (GC) (PR 1.49; 95% CI 1.21 to 1.85; p<0.001) and pulse therapy with methylprednisolone (PR 1.38; 95% CI 1.14 to 1.67; p=0.001) remained significant; for hospitalisation, age >50 years (PR 1.89; 95% CI 1.26 to 2.85; p=0.002), no use of tumour necrosis factor inhibitor (TNFi) (PR 2.51;95% CI 1.16 to 5.45; p=0.004) and methylprednisolone pulse therapy (PR 2.50; 95% CI 1.59 to 3.92; p<0.001); for ICU admission, oral GC (PR 2.24; 95% CI 1.36 to 3.71; p<0.001) and pulse therapy with methylprednisolone (PR 1.65; 95% CI 1.00 to 2.68; p<0.043); the two variables associated with death were pulse therapy with methylprednisolone or cyclophosphamide (PR 2.86; 95% CI 1.59 to 5.14; p<0.018). CONCLUSIONS: Age >50 years and immunosuppression with GC and cyclophosphamide were associated with unfavourable outcomes of COVID-19. Treatment with TNFi may have been protective, perhaps leading to the COVID-19 inflammatory process.

Chronic use of hydroxychloroquine did not protect against COVID-19 in a large cohort of patients with rheumatic diseases in Brazil

Abstract Background: There is a lack of information on the role of chronic use of hydroxychloroquine during the SARS-CoV-2 outbreak. Our aim was to compare the occurrence of COVID-19 between rheumatic disease patients on hydroxychloroquine with individuals from the same household not taking the drug during the first 8 weeks of community viral transmission in Brazil. Methods: This baseline cross-sectional analysis is part of a 24-week observational multi-center study involving 22 Brazilian academic outpatient centers. All information regarding COVID-19 symptoms, epidemiological, clinical, and demographic data were recorded on a specific web-based platform using telephone calls from physicians and medical students. COVID-19 was defined according to the Brazilian Ministry of Health (BMH) criteria. Mann-Whitney, Chi-square and Exact Fisher tests were used for statistical analysis and two binary Final Logistic Regression Model by Wald test were developed using a backward-stepwise method for the presence of COVID-19. Results: From March 29th to May 17st, 2020, a total of 10,443 participants were enrolled, including 5166 (53.9%) rheumatic disease patients, of whom 82.5% had systemic erythematosus lupus, 7.8% rheumatoid arthritis, 3.7% Sjögren's syndrome and 0.8% systemic sclerosis. In total, 1822 (19.1%) participants reported flu symptoms within the 30 days prior to enrollment, of which 3.1% fulfilled the BMH criteria, but with no significant difference between rheumatic disease patients (4.03%) and controls (3.25%). After adjustments for multiple confounders, the main risk factor significantly associated with a COVID-19 diagnosis was lung disease (OR 1.63; 95% CI 1.03-2.58); and for rheumatic disease patients were diagnosis of systemic sclerosis (OR 2.8; 95% CI 1.19-6.63) and glucocorticoids above 10 mg/ day (OR 2.05; 95% CI 1.31-3.19). In addition, a recent influenza vaccination had a protective effect (OR 0.674; 95% CI 0.46-0.98). Conclusion: Patients with rheumatic disease on hydroxychloroquine presented a similar occurrence of COVID-19 to household cohabitants, suggesting a lack of any protective role against SARS-CoV-2 infection. Trial registration Brazilian Registry of Clinical Trials (ReBEC; RBR - 9KTWX6).

A Brazilian Cohort of Patients With Immuno-Mediated Chronic Inflammatory Diseases Infected by SARS-CoV-2 (ReumaCoV-Brasil Registry): Protocol for a Prospective, Observational Study.

BACKGROUND: Patients with immune-mediated rheumatic diseases (IMRD) are at increased risk of infections, including significant morbidity and high mortality. Considering the potential for unfavorable outcomes of SARS-CoV-2 infection in patients with IMRD, several questions were raised regarding the impact of COVID-19 at the start of the pandemic. OBJECTIVE: This paper presents the protocol of a study that aims to prospectively evaluate patients with IMRD and a confirmed COVID-19 diagnosis (using criteria provided by the Brazilian Ministry of Health). METHODS: The study comprised a prospective, observational cohort (patients with IMRD and COVID-19) and a comparison group (patients with only IMRD), with a follow-up time of 6 months to evaluate differences in health outcomes. The primary outcomes will be changes in IMRD disease activity after SARS-CoV-2 infection at 4 time points: (1) at baseline, (2) within 4-6 weeks after infection, (3) at 3 months after the second assessment (±15 days), and (4) at 6 months (±15 days). The secondary outcomes will be the progression rate to moderate or severe forms of COVID-19, need for intensive care unit admission and mechanical ventilation, death, and therapeutic changes related to IMRD. Two outcomes-pulmonary and thromboembolic events in patients with both IMRD and SARS-CoV-2 infection-are of particular interest and will be monitored with close attention (clinical, laboratory, and function tests as well as imaging). RESULTS: Recruitment opened in May 2020, with 1300 participants recruited from 43 sites as of November 2020. Patient recruitment will conclude by the end of December 2020, with follow-up occurring until April 2021. Data analysis is scheduled to start after all inclusion data have been collected, with an aim to publish a peer-reviewed paper in December 2020. CONCLUSIONS: We believe this study will provide clinically relevant data on the general impact of COVID-19 on patients with IMRD. TRIAL REGISTRATION: Brazilian Registry of Clinical Trials RBR-33YTQC; http://www.ensaiosclinicos.gov.br/rg/RBR-33ytqc/. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/24357.

Revisiting hydroxychloroquine and chloroquine for patients with chronic immunity-mediated inflammatory rheumatic diseases.

Hydroxychloroquine and chloroquine, also known as antimalarial drugs, are widely used in the treatment of rheumatic diseases and have recently become the focus of attention because of the ongoing COVID-19 pandemic. Rheumatologists have been using antimalarials to manage patients with chronic immune-mediated inflammatory rheumatic diseases for decades. It is an appropriate time to review their immunomodulatory and anti-inflammatory mechanisms impact on disease activity and survival of systemic lupus erythematosus patient, including antiplatelet effect, metabolic and lipid benefits. We also discuss possible adverse effects, adding a practical and comprehensive approach to monitoring rheumatic patients during treatment with these drugs.

Treatment data from the Brazilian fibromyalgia registry (EpiFibro).

BACKGROUND: EpiFibro (Brazilian Epidemiological Study of Fibromyalgia) was created to study patients with fibromyalgia (FM). Patients were included since 2011 according to the classification criteria for FM of the American College of Rheumatology of 1990 (ACR1990). OBJECTIVE: To analyze the therapeutic measures prescribed by Brazilian physicians. MATERIALS AND METHODS: Cross-sectional study of a multicenter cohort. The therapeutic measures were described using descriptive statistics. RESULTS: We analyzed 456 patients who had complete data in the registry. The mean age was 54.0 ± 11.9 years; 448 were women (98.2%). Almost all patients (98.4%) used medications, 62.7% received health education, and less than half reported practicing physical exercise; these modalities were often used in combination. Most patients who practiced exercises practiced aerobic exercise only, and a significant portion of patients combined it with flexibility exercises. The most commonly used medication was amitriptyline, followed by cyclobenzaprine, and a minority used medication specifically approved for FM, such as duloxetine and pregabalin, either alone or in combination. Combinations of two or three medications were observed, with the combination of fluoxetine and amitriptyline being the most frequent (18.8%). CONCLUSION: In this evaluation of the care of patients with FM in Brazil, it was found that the majority of patients are treated with a combination of pharmacological measures. Non-pharmacological methods are underused, with aerobic exercise being the most commonly practiced exercise type. The most commonly prescribed single drug was amitriptyline, and the most commonly prescribed combination was fluoxetine and amitriptyline. Drugs specifically approved for FM are seldom prescribed.

Revisiting hydroxychloroquine and chloroquine for patients with chronic immunity-mediated inflammatory rheumatic diseases

Abstract Hydroxychloroquine and chloroquine, also known as antimalarial drugs, are widely used in the treatment of rheumatic diseases and have recently become the focus of attention because of the ongoing COVID-19 pandemic. Rheumatologists have been using antimalarials to manage patients with chronic immune-mediated inflammatory rheumatic diseases for decades. It is an appropriate time to review their immunomodulatory and anti-inflammatory mechanisms impact on disease activity and survival of systemic lupus erythematosus patient, including antiplatelet effect, metabolic and lipid benefits. We also discuss possible adverse effects, adding a practical and comprehensive approach to monitoring rheumatic patients during treatment with these drugs.(AU)

Treatment data from the Brazilian fibromyalgia registry (EpiFibro)

Abstract Background: EpiFibro (Brazilian Epidemiological Study of Fibromyalgia) was created to study patients with fibromyalgia (FM). Patients were included since 2011 according to the classification criteria for FM of the American College of Rheumatology of 1990 (ACR1990). Objective: To analyze the therapeutic measures prescribed by Brazilian physicians. Materials and methods: Cross-sectional study of a multicenter cohort. The therapeutic measures were described using descriptive statistics. Results: We analyzed 456 patients who had complete data in the registry. The mean age was 54.0 ± 11.9 years; 448 were women (98.2%). Almost all patients (98.4%) used medications, 62.7% received health education, and less than half reported practicing physical exercise; these modalities were often used in combination. Most patients who practiced exercises practiced aerobic exercise only, and a significant portion of patients combined it with flexibility exercises. The most commonly used medication was amitriptyline, followed by cyclobenzaprine, and a minority used medication specifically approved for FM, such as duloxetine and pregabalin, either alone or in combination. Combinations of two or three medications were observed, with the combination of fluoxetine and amitriptyline being the most frequent (18.8%). Conclusion: In this evaluation of the care of patients with FM in Brazil, it was found that the majority of patients are treated with a combination of pharmacological measures. Non-pharmacological methods are underused, with aerobic exercise being the most commonly practiced exercise type. The most commonly prescribed single drug was amitriptyline, and the most commonly prescribed combination was fluoxetine and amitriptyline. Drugs specifically approved for FM are seldom prescribed.(AU)

Precordial pain, leukocytosis and bicytopenia in a teenager with systemic juvenile idiopathic arthritis under immunosuppressive therapy / Dor precordial, leucocitose e bicitopenia em adolescente com artrite idiopática juvenil sistêmica em terapia imunossupressora

ABSTRACT Objective: To highlight the importance of the new classification criteria for the macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis in order to reduce morbidity and mortality outcome related to this disease. Case description: A 12-year-old female patient with diagnosis of systemic juvenile idiopathic arthritis under immunosuppression therapy for two years developed cough, acute precordial chest pain, tachypnea, tachycardia and hypoxemia for two days. Chest tomography showed bilateral laminar pleural effusion with bibasilar consolidation. The electrocardiogram was consistent with acute pericarditis and the echocardiogram showed no abnormalities. Laboratory exams revealed anemia, leukocytosis and increased erythrocyte sedimentation rate, as well as C-reactive protein rate and serum biomarkers indicative of myocardial injury. Systemic infection and/or active systemic juvenile idiopathic arthritis were considered. She was treated with antibiotics and glucocorticoids. However, 10 days later she developed active systemic disease (fever, evanescent rash and myopericarditis with signs of heart failure) associated with macrophage activation syndrome, according to the 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis. She was treated for five days with pulse therapy, using glucocorticoids, immunoglobulin and cyclosporine A, with improvement of all clinical signs and laboratory tests. Comments: Myopericarditis with signs of heart failure associated with MAS is a rare clinical presentation of systemic juvenile idiopathic arthritis. Macrophage activation syndrome occurs mainly during periods of active systemic juvenile idiopathic arthritis and may be triggered by infection. Knowledge about this syndrome is crucial to reduce morbidity and mortality.

RESUMO Objetivo: Destacar a importância do conhecimento sobre os novos critérios de classificação para síndrome de ativação macrofágica (SAM) na artrite idiopática juvenil sistêmica para reduzir a morbidade e mortalidade desse desfecho. Descrição do caso: Adolescente do sexo feminino de 12 anos de idade, em terapia imunossupressora por diagnóstico de artrite idiopática juvenil sistêmica há 2 anos, com quadro de tosse, dor precordial aguda, taquipneia, taquicardia e hipoxemia há 2 dias. A tomografia de tórax evidenciou efusão pleural laminar bilateral com consolidação bibasal. O eletrocardiograma foi compatível com pericardite aguda, e o ecocardiograma foi normal. Os exames laboratoriais revelaram anemia, leucocitose e aumento da velocidade de hemossedimentação, proteína C-reativa e marcadores séricos de lesão miocárdica. Infecção sistêmica e/ou doença sistêmica em atividade foram consideradas. A paciente foi tratada com antibióticos e glicocorticoide. Entretanto, dez dias depois, evoluiu com doença sistêmica em atividade (febre, exantema e miopericardite com insuficiência cardíaca) associada à SAM, de acordo com o 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis, e necessitou de cinco dias de pulsoterapia com glicocorticoide, imunoglobulina e ciclosporina A, com melhora de todos os parâmetros clínicos e laboratoriais. Comentários: A miopericardite com sinais de insuficiência cardíaca associada à SAM é uma apresentação clínica rara da artrite idiopática juvenil sistêmica, que ocorre principalmente em períodos de atividade sistêmica da doença e pode ser deflagrada por infecções. O conhecimento sobre essa síndrome é fundamental para reduzir morbidade e mortalidade desse grave desfecho.

PRECORDIAL PAIN, LEUKOCYTOSIS AND BICYTOPENIA IN A TEENAGER WITH SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS UNDER IMMUNOSUPPRESSIVE THERAPY.

OBJECTIVE: To highlight the importance of the new classification criteria for the macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis in order to reduce morbidity and mortality outcome related to this disease. CASE DESCRIPTION: A 12-year-old female patient with diagnosis of systemic juvenile idiopathic arthritis under immunosuppression therapy for two years developed cough, acute precordial chest pain, tachypnea, tachycardia and hypoxemia for two days. Chest tomography showed bilateral laminar pleural effusion with bibasilar consolidation. The electrocardiogram was consistent with acute pericarditis and the echocardiogram showed no abnormalities. Laboratory exams revealed anemia, leukocytosis and increased erythrocyte sedimentation rate, as well as C-reactive protein rate and serum biomarkers indicative of myocardial injury. Systemic infection and/or active systemic juvenile idiopathic arthritis were considered. She was treated with antibiotics and glucocorticoids. However, 10 days later she developed active systemic disease (fever, evanescent rash and myopericarditis with signs of heart failure) associated with macrophage activation syndrome, according to the 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis. She was treated for five days with pulse therapy, using glucocorticoids, immunoglobulin and cyclosporine A, with improvement of all clinical signs and laboratory tests. COMMENTS: Myopericarditis with signs of heart failure associated with MAS is a rare clinical presentation of systemic juvenile idiopathic arthritis. Macrophage activation syndrome occurs mainly during periods of active systemic juvenile idiopathic arthritis and may be triggered by infection. Knowledge about this syndrome is crucial to reduce morbidity and mortality.

Sonographic evaluation of salivary glands in juvenile Sjögren's syndrome.

IIntroduction: Sjögren's syndrome in childhood is a rare autoimmune disease and mostly under-diagnosed. The aim of this study is to highlight the importance of ultrasonographic assessment of the salivary glands in children with recurrent parotitis and positive autoantibodies. Two cases of ultrasonographic patterns typical of Sjögren's syndrome have been described below. Case 1: Female, 7 years old, reporting for 2 years recurrent parotitis, xerophthalmia, xerostomia, polyarthralgia and fever. Immunological tests were positive for antinuclear antibodies, rheumatoid factor, anti-SSA/Ro and anti-SSB/La. Salivary glands ultrasound was consistent with Grade 4 by the B-mode method and the spectral Doppler with presence of intense Power Doppler signal and decreased vessels internal resistance, supporting the diagnosis of juvenile Sjögren's syndrome. Case 2: Female, 10 years old, reporting recurrent parotitis for 1 year and polyarthritis for 10 days. The supplementary tests revealed positive antibodies for Sjögren's syndrome. Salivary glands Ultrasound and Spectral Doppler were consistent with chronic and active inflammatory process of the salivary glands in the juvenile Sjögren's syndrome. DISCUSSION: Salivary glands ultrasound can be a useful exam in the diagnosis of juvenile Sjögren's syndrome.

New guidelines for the diagnosis of fibromyalgia.

OBJECTIVE: To establish guidelines based on scientific evidence for the diagnosis of fibromyalgia. MATERIAL AND METHODS: Evidence collection was performed based on 9 questions regarding the diagnosis of fibromyalgia, structured using the Patient, Intervention or Indicator, Comparison and Outcome (P.I.C.O.), with searches in the main, primary databases of scientific information. After defining the potential studies to support the recommendations, they were graded according to evidence and degree of recommendation.

EpiFibro (Brazilian Fibromyalgia Registry): data on the ACR classification and diagnostic preliminary criteria fulfillment and the follow-up evaluation / EpiFibro (Registro Brasileiro de Fibromialgia): dados sobre a classificação do ACR e preenchimento dos critérios diagnósticos preliminares e avaliação de seguimento

Abstract Introduction: EpiFibro (Brazilian Epidemiological Study of Fibromyalgia) was created to study Fibromyalgia patients. Patients were included since 2011 according to the 1990 American College of Rheumatology Classification Criteria for Fibromyalgia (ACR1990). Objectives: To determine how many patients still fulfill the ACR1990 and the ACR2010 criteria in 2014; to determine the correlation between the impact of FM and to describe data on the follow-up evaluation. Methods: This is a cross sectional study in a multicenter cohort of patients. The data was collected between 2013 and 2015. Physician included patients that fulfilled the ACR1990 criteria on the date of entry. The follow-up data were considered only for patients with at least two evaluations. A minimally significant change was considered to be a 30% variation of parameters scores. Results: 810 patients’ data were analyzed. Patients presented a mean age of 51.8 ± 11.5 years old. There were 786 female. Most patients met both criteria. There was a greater fulfilling of the ACR2010. There was a moderate correlation between Polysymptomatic Distress Scale and Fibromyalgia Impact Questionnaire. Three hundred fourteen patients with more than one assessment were found, but 88 patients were excluded. Thus, 226 patients with one follow-up monitoring parameter were considered (Fibromyalgia Impact Questionnaire: 222; Polysymptomatic Distress Scale: 199; both: 195). The mean follow-up time was 9.1 ± 7.5 months (1–44). Most patients became stable. Conclusion: InEpiFibro, most patients fulfill simultaneously the ACR1990 and ACR2010. A larger number of patients fulfill the ACR2010 at the time of the evaluation. There was a moderate correlation between the Polysymptomatic Distress Scale and the Fibromyalgia Impact Questionnaire. Most patients remained stable over time.

Resumo Introdução: O EpiFibro (Estudo Epidemiológico Brasileiro de Fibromialgia) foi criado para estudar pacientes com fibromialgia. Foram incluídos pacientes desde 2011 de acordo com os critérios de classificação para a fibromialgia do American College of Rheumatology de 1990 (ACR1990). Objetivos: Determinar quantos pacientes ainda atendem aos critérios ACR1990 e ACR2010 em 2014; determinar a correlação entre o impacto da FM medido pelo Questionário de Impacto da Fibromialgia (FIQ) e pela Polysymptomatic Distress Scale (PDS) e descrever dados sobre a avaliação de seguimento. Métodos: Estudo transversal em uma coorte multicêntrica de pacientes. Os dados foram coletados entre 2013 e 2015. O médico incluiu pacientes que atenderam aos critérios ACR1990 no momento da entrada. Consideraram-se os dados de seguimento apenas dos pacientes com pelo menos duas avaliações. Uma variação de 30% nos escores dos parâmetros foi considerada uma alteração minimamente significativa. Resultados: Analisaram-se os dados de 810 pacientes. Os pacientes apresentaram média de 51,8 ± 11,5 anos. Havia 786 mulheres. A maior parte dos pacientes atendeu a ambos os critérios. Houve um maior atendimento aos critérios ACR2010. Houve uma correlação moderada entre a PDS e o FIQ. Encontraram-se 314 pacientes com mais de uma avaliação, mas 88 pacientes foram excluídos. Assim, foram considerados 226 pacientes com um parâmetro de monitoramento no seguimento. (FIQ: 222; PDS: 199; ambos: 195). O tempo médio de seguimento foi de 9,1 ± 7,5 meses (1 a 44). A maior parte dos pacientes tornou-se estável. Conclusão: No EpiFibro, a maior parte dos pacientes atendia simultaneamente ao ACR1990 e ao ACR2010. Uma maior quantidade de pacientes atendia ao ACR2010 no momento da avaliação. Houve uma correlação moderada. A maior parte dos pacientes manteve-se estável ao longo do tempo.

EpiFibro (Brazilian Fibromyalgia Registry): data on the ACR classification and diagnostic preliminary criteria fulfillment and the follow-up evaluation.

INTRODUCTION: EpiFibro (Brazilian Epidemiological Study of Fibromyalgia) was created to study Fibromyalgia patients. Patients were included since 2011 according to the 1990 American College of Rheumatology Classification Criteria for Fibromyalgia (ACR1990). OBJECTIVES: To determine how many patients still fulfill the ACR1990 and the ACR2010 criteria in 2014; to determine the correlation between the impact of FM and to describe data on the follow-up evaluation. METHODS: This is a cross sectional study in a multicenter cohort of patients. The data was collected between 2013 and 2015. Physician included patients that fulfilled the ACR1990 criteria on the date of entry. The follow-up data were considered only for patients with at least two evaluations. A minimally significant change was considered to be a 30% variation of parameters scores. RESULTS: 810 patients' data were analyzed. Patients presented a mean age of 51.8±11.5 years old. There were 786 female. Most patients met both criteria. There was a greater fulfilling of the ACR2010. There was a moderate correlation between Polysymptomatic Distress Scale and Fibromyalgia Impact Questionnaire. Three hundred fourteen patients with more than one assessment were found, but 88 patients were excluded. Thus, 226 patients with one follow-up monitoring parameter were considered (Fibromyalgia Impact Questionnaire: 222; Polysymptomatic Distress Scale: 199; both: 195). The mean follow-up time was 9.1±7.5 months (1-44). Most patients became stable. CONCLUSION: InEpiFibro, most patients fulfill simultaneously the ACR1990 and ACR2010. A larger number of patients fulfill the ACR2010 at the time of the evaluation. There was a moderate correlation between the Polysymptomatic Distress Scale and the Fibromyalgia Impact Questionnaire. Most patients remained stable over time.

New guidelines for the diagnosis of fibromyalgia / Novas diretrizes para o diagnóstico da fibromialgia

Abstract Objective To establish guidelines based on scientific evidence for the diagnosis of fibromyalgia. Material and methods Evidence collection was performed based on 9 questions regarding the diagnosis of fibromyalgia, structured using the Patient, Intervention or Indicator, Comparison and Outcome (P.I.C.O.), with searches in the main, primary databases of scientific information. After defining the potential studies to support the recommendations, they were graded according to evidence and degree of recommendation.

Resumo Objetivo Estabelecer diretrizes baseadas em evidências científicas para o diagnóstico da fibromialgia. Material e métodos A coleta de evidências foi elaborada a partir de nove questões sobre diagnóstico da fibromialgia, estruturadas por meio do PICO (Paciente, Intervenção ou Indicador, Comparação e Outcome), com busca nas principais bases primárias de informação científica. Após definir os estudos potenciais para sustentação das recomendações, esses foram graduados pela força da evidência e grau de recomendação. Resultados e conclusões As questões resultaram em nove recomendações para o diagnóstico da fibromialgia com base nas evidências de literatura e na opinião dos experts que participaram do trabalho.