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INTRODUCTION: Children are less likely to have severe acute COVID-19, but cases of pediatric multisystem inflammatory syndrome (PIMS) with possible temporal association with previous SARS-CoV-2 infection have been described. OBJECTIVES: Non-systematic review of the literature on epidemiology, pathophysiology, diagnosis, and treatment of PIMS. SOURCE OF DATA: PubMed database, scientific documents of the Brazilian Society of Pediatrics, World Health Organization, Centers for Disease Control and Prevention (CDC), and Royal College of Pediatrics and Child Health. SUMMARY OF FINDINGS: PIMS shares characteristics with Kawasaki disease, toxic shock syndrome, bacterial sepsis and cytokine storm syndrome. It is more frequent in Afrodescendants and Hispanics, schoolchildren and adolescents, and in males. It occurs 2-4 weeks after SARS-CoV-2 infection. Pathophysiology involves direct effects of the virus and/or post-COVID-19 immune dysregulation. The clinical presentation is heterogeneous, fever being very frequent, followed by gastrointestinal, cardiovascular, respiratory, neurological, and renal manifestations. Thorough anamnesis and physical examination, as well as complementary exams to assess inflammatory process, organ involvement and the relationship with SARS-CoV-2 infection (RT-PCR and serology), are essential. Diagnostic criteria proposed by the CDC and WHO support the diagnosis. Treatment must be coordinated by a team of specialists, and directed to inflammatory and organic manifestations. CONCLUSIONS: PIMS is characterized by a broad clinical spectrum, with fever, gastrointestinal, neurological manifestations, shock, and myocardial dysfunction. It requires a high degree of suspicion for early treatment and prevention of potential cardiovascular, respiratory, renal, and neurological complications.

Background To date there are no specific classification criteria for childhood-onset systemic lupus erythematosus (cSLE). Objectives This study aims to compare the performance among the American College of Rheumatology (ACR) 1997, the Systemic Lupus International Collaborating Clinics criteria (SLICC) and the new European League Against Rheumatism (EULAR)/ACR criteria, in a cSLE cohort. Methods Data were retrospectively collected from medical charts of cSLE cases and controls with defined rheumatic diseases, both ANA positive, to establish each ACR1997, SLICC and EULAR/ACR criterion fulfilled, at first visit and 1-year-follow-up. Results 122 cSLE cases and 89 controls were included. At first visit, SLICC criteria had greater sensitivity than ACR 1997 (89.3% versus 70.5%, p Conclusion In this cSLE population, SLICC criteria performed best at first visit and 1-year-follow-up. The adoption of a EULAR/ACR total score ≥ 13, against the initially proposed ≥10 score, was most appropriate to classify cSLE in our study. Further studies are necessary to address if SLICC might allow cSLE classification earlier in disease course and be more inclusive of cSLE subjects for clinical studies. References [1] Tedeschi SK, Johnson SR, Boumpas DA, Daikh D, Dorner T, Jayne D, et al. Developing and Refining New Candidate Criteria for SLE Classification: An International Collaboration. Arthritis Care Res (Hoboken)2018; 70:571-581. [2] Leuchten N, Hoyer A, Brinks R, Schoels M, Schneider M, Smolen J, et al. Performance of Anti-nuclear Antibodies for Classifying Systemic Lupus Erythematosus: a Systematic Literature Review and Meta-regression of Diagnostic Data. Arthritis Care Res (Hoboken)2018; 70:428-438. [3] Aringer M, Costenbader KH, Brinks R, Boumpas D, Daikh D, Jayne D, et al. Validation of New Systemic Lupus Erythematosus Classification Criteria. In: 2018 ACR/AHRP Annual Meeting; Chicago, Illinois, 20-24 October 2018. https://acrabstracts.org. Accessed 24 Oct 2018. Disclosure of Interests None declared

Background To date there are no specific classification criteria for childhood-onset systemic lupus erythematosus (cSLE). This study aims to compare the performance among the American College of Rheumatology (ACR) 1997, the Systemic Lupus International Collaborating Clinics criteria (SLICC) and the new European League Against Rheumatism (EULAR)/ACR criteria, in a cSLE cohort. Methods We conducted a medical chart review study of cSLE cases and controls with defined rheumatic diseases, both ANA positive, to establish each ACR1997, SLICC and EULAR/ACR criterion fulfilled, at first visit and 1-year-follow-up. Results Study population included 122 cSLE cases and 89 controls. At first visit, SLICC criteria had higher sensitivity than ACR 1997 (89.3% versus 70.5%, p

Relapsing polychondritis (RP) is a rare autoimmune systemic disease, especially in childhood. To report three new pediatric RP cases, to provide a literature review and to compare with adulthood disease, retrospective data collection from three childhood RP cases was observed in a Brazilian Pediatric Rheumatology Division. A literature review based on a MEDLINE database search was performed. Arthritis and auricular chondritis were present in our three patients. Two cases presented with early and severe laryngotracheal chondritis, besides initial and symptomatic costochondritis. The other case developed prominent epiphyseal plate involvement. Two patients were refractory to corticosteroids and immunosuppressants and required the use of TNF-alpha inhibitors to improve the symptoms, while corticosteroids plus methotrexate induced remission in the other patient. The literature review showed 44 cases of pediatric-onset disease in English language. Arthritis and ear chondritis are the most common initial and cumulative manifestations of RP in children and adults. Nasal and laryngotracheobronchial chondritis are also common manifestations observed during follow-up in childhood. There is also an early severity of respiratory chondritis in childhood, requiring aggressive treatment with corticosteroids, immunosuppressants and biologic agents. The data presented by those 3 children, considered in conjunction with the data from the 44 published cases, may reflect some distinguishing childhood RP features, such as more severe and frequent respiratory tract involvement, symptomatic costochondritis and the atypical pattern of persistent and destructive arthritis with epiphyseal plate involvement. Response to immunosuppressants and biologic agents is anecdotal, but steroids remain the main drug during the flares.

Respiratory tract disorders in the juvenile rheumatic diseases are not infrequent and can have different clinical features when compared with those in the adult diseases. The purpose of this review article is to show the main manifestations of the respiratory tract, with an emphasis on lung involvement, in the most frequent juvenile rheumatic diseases.

Objectives:To describe a population of children diagnosed with Kawasaki’s disease (KD) in pediatric rheumatology centers of Rio de Janeiro, Brazil, defining the magnitude of the delay period in diagnosing KD and initiating treatment due to confusion with common childhood febrile illnesses and the impact of this delay on the frequency of coronary sequels. Methods:Data analysis from hospital records summarized in a dedicated form, including name, gender, age, date of first recorded clinical signs, date of admission to the specialty service, information about symptoms, clinical evolution, intravenous immunoglobulin (IVIG) use and coronary sequels. Results:Of 125 patients, 63% were males. 40% were under 2 years at diagnosis. Average lapse between earliest signs and KD diagnosis was 12 days (mean fever duration, 14 d). Only 22.4% had a diagnosis of KD before entering the specialty service. For the remainder, initial hy-potheses included: bacterial (60%) and viral infections (12%), rheumatological diseases (4%) and adverse vaccination reactions (1.6%). Hence, prevalent febrile illnesses of childhood were major confounding factors. For records (85.6%) mentioning treatment, 46.7% reported IVIG treatment, beginning after day 10 in 23 cases (21.5%). 20 patients (16%) presented coronary sequels, 9 of which were diagnosed late, including 3 given IVIG after day 10, and 6 given no IVIG. We found no significant association between the frequency of coronary sequels and: a) sex; b) age; c) clinical criteria; d) initiation of IVIG treatment (before or after day 10). Conclusions:Common febrile illnesses of childhood often confound the diagnosis of KD.

OBJECTIVE: To present an updated review concerning the most prevalent diseases with musculoskeletal signs and symptoms that make adolescents seek medical care, giving special emphasis to rheumatic diseases. Our aim is to offer physicians and health care providers the possibility of distinct differential diagnoses, thus allowing them to establish a therapeutic approach and, if necessary, refer the patient to a specialist METHODS: Review of literature using Medline database, data obtained at our department, and the authorś personal experience. RESULTS: Musculoskeletal pain is characteristic of several diseases and usually urges adolescents to seek medical care. Rheumatic diseases, especially rheumatic fever, account for nearly fifty percent of the cases. In adolescents, it is also important that the aspects regarding the diagnosis and treatment of idiopathic juvenile arthritis, arthritis associated with enthesitis, systemic lupus erythematosus, and vasculitis be considered. Fibromyalgia, reflex sympathetic dystrophy, growing pains, hypermobility syndrome, and psychogenic rheumatism are noninflammatory conditions that frequently mimic rheumatic diseases. CONCLUSIONS: Inflammatory and noninflammatory conditions, and diseases of different etiology (infectious, neoplastic, and orthopedic) are frequently associated with musculoskeletal pain. It is important that health professionals diagnose these diseases as early as possible so that prompt action can be taken and prognosis can be improved.

To evaluate the prevalence of genetic defects in clinically suspected autoinflammatory syndromes (AIS) in a Brazilian multicenter study. 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. 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. We have found that approximately one third of the Brazilian patients with a clinical suspicion of AIS have a confirmed genetic diagnosis.

Os autores relatam um caso clínico sobre Síndrome SAPHO, na faixa etária pediátrica, com enfoque dermatológico. Essa entidade deve ser considerada, nos pacientes que tenham dor na parede torácica anterior ou outros sintomas musculoesqueléticos, acompanhados por lesões dermatológicas, como pustulose palmoplantar e acne fulminans. As manifestações cutâneas específicas, diagnóstico e o tratamento utilizado serão apresentados The authors report a case of SAPHO Syndrome, in pediatric age, with a dermatological focus. This entity should be considered in patients who have pain in the anterior chest wall or other musculoskeletal symptoms, accompanied by palmoplantar pustulosis and acne fulminans. The specific cutaneous manifestations, diagnosis and the treatment will be presented

Non-adherence to treatments for chronic diseases may jeopardize patients’ health, increase costs of care, and cause unnecessary clinic appointments and diagnostic studies, as well as additional treatments with potentially serious side effects. Little is known about adherence to methotrexate in pediatric rheumatology. Because this medication is commonly used in JIA, we assessed adherence among children receiving methotrexate in two countries. A total of 76 outpatients (M:F 21:55) with JIA seen in Rio de Janeiro (Brazil) and in Boston (US) taking methotrexate for >2 months were enrolled. Questionnaires were completed by the parents from both centers. Non-adherence was defined as omission of ≥3 prescribed doses in the previous 8 weeks. Patients’ ages ranged from 1 to 17 years. Mean time on methotrexate was 20.5 months (±25). Overall rate of non-adherence was 18%. The rate of reported non-adherence was 8% in Boston and 24% in Rio de Janeiro (P = 0.012). The main reason for non-adherence in Boston was “child refused”; in Rio de Janeiro, the main reason was inability to obtain medication. Age had a negative association with adherence (P < 0.0001). Sex, time on methotrexate, route of administration, or concomitant use of other medications were not associated with adherence. Eighteen percent of children with JIA prescribed methotrexate were non-compliant. The difference in reasons for poor adherence between patients in Rio de Janeiro and Boston suggests that different strategies may be needed to improve adherence in these 2 settings. The rate of non-adherence warrants further investigation.

The authors report a case of SAPHO Syndrome, in pediatric age, with a dermatological focus. This entity should be considered in patients who have pain in the anterior chest wall or other musculoskeletal symptoms, accompanied by palmoplantar pustulosis and acne fulminans. The specific cutaneous manifestations, diagnosis and the treatment will be presented.

OBJETIVO: Avaliar a freqüência e a gravidade dos efeitos adversos durante a infusão de infliximabe. MATERIAL E MÉTODOS: Avaliação retrospectiva de prontuários de 58 pacientes acompanhados em cinco serviços de reumatologia pediátrica. Todos apresentavam doença refratária ou eram intolerantes ao uso de uma ou mais drogas modificadoras de doença e receberam uma ou mais infusões de infliximabe. A análise estatística foi descritiva, levando-se em conta a freqüência e o tipo dos efeitos adversos, assim como sua relação com o número de infusões, a dose de infliximabe e a indicação. RESULTADOS: A média da idade na época da introdução do infliximabe foi de 12 anos e 9 meses e a média do tempo de evolução da doença foi de 7 anos e 7 meses. A principal indicação da medicação foi artrite idiopática juvenil (43). Dos 58 pacientes avaliados, 25 (43,1%) apresentaram efeitos adversos durante a infusão e em 17 (29,3%), a medicação teve de ser suspensa. As reações que ocorreram foram: dispnéia (dez), náuseas e vômitos (oito), rash cutâneo (sete), choque anafilático (seis), rubor facial (cinco), angioedema (quatro), dor torácica (quatro), urticária e hipertensão, entre outras. A reação anafilática foi mais freqüente entre a quarta e a sexta infusão. CONCLUSÃO: A freqüência e a gravidade dos efeitos adversos ao infliximabe devem ser levadas em conta, não apenas por ocasião da sua indicação, mas principalmente por ocasião da escolha do local para a sua aplicação, que deve contar com estrutura para atendimento de urgência. OBJECTIVE: To evaluate the frequency and the severity of the adverse reactions during the infusion of infliximab. METHODS: We performed a retrospective chart review of 58 patients, followed up at 5 paediatric rheumatology centers. All patients presented refractory disease or were intolerant to one or more disease modifying drugs and received one or more infliximab infusions. The data analysis was descriptive, considering the frequency and the kind of adverse reactions as well as the number of the infusions, the infliximab dose and the indication. RESULTS: The mean age at the onset of infliximab was 12 years and 9 months and the mean of the disease duration was 7 years and 7 months. The main indication of the drug was juvenile idiopathic arthritis (43). Twenty five out of 58 patients (43.1%) presented adverse reactions during the infusion and 17 (29,3%) had to withdraw the treatment. The reactions were: dyspnea (ten), nausea and vomiting (eight), rash (seven), anaphylaxis (six), flush (five), angioedema (four), chest pain (four) besides urticaria, hypertension, among others. The anaphylaxis was more frequent between the fourth and the sixth infusion. CONCLUSION: The frequency and the severity of adverse reactions to infliximab must be considered not only in relation to its indication, but mainly for the choice of the center to the drug infusion where emergency treatment may be performed. Universidade Federal de São Paulo (UNIFESP) Departamento de Pediatria Setor de Reumatologia Pediátrica Universidade Federal do Rio de Janeiro Faculdade de Medicina UFRJ Instituto de Puericultura e Pediatria Martagão Gesteira Serviço de Reumatologia Pediátrica UFRJ Faculdade de Medicina setor de Reumatologia do Adolescente Irmandade da Santa Casa de Misericórdia de São Paulo Departamento de Pediatria Serviço de Reumatologia Pediátrica PMSP SMS Hospital Infantil Menino Jesus UNIFESP, Depto. de Pediatria Setor de Reumatologia Pediátrica SciELO

Methotrexate is the most used DMARD drug in the treatment of juvenile idiopathic arthritis and its efficacy has been demonstrated for more than 20 years. The usual dose is 10 to 15mg/m2/week. It is a safe and well-tolerated drug by children and the most common side effects are gastrointestinal, hematologic and liver toxicity.