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.

Medication adherence in patients with juvenile idiopathic arthritis.

OBJECTIVE: The aim of this study was to investigate pharmacological treatment adherence of patients with juvenile idiopathic arthritis, attended in an outpatient pharmacy at a tertiary hospital in northeastern Brazil. METHODS: The analysis of adherence was performed along with caregivers, through a structured questionnaire based on Morisky, Green and Levine, which enabled the categorization of adherence in "highest", "moderate" or "low" grades, and through evaluating medication dispensing registers, which classified the act of getting medications at the pharmacy as "regular" or "irregular". Drug Related Problems (DRP) were identified through the narrative of caregivers and classified according to the Second Granada Consensus. Then, a pharmaceutical orientation chart with information about the therapeutic regimen was applied, in order to function as a guide for issues that influenced adherence. RESULTS: A total of 43 patients was included, with a mean age of 11.12 years, and 65.1% (n=28) were female. Applying the questionnaire, it was found "highest" adherence in 46.5% (n=20) patients, "moderate" adherence in 48.8% (n=21), and "low" adherence in 4.7% (n=2). Through an analysis of the medication dispensing registers, a lower level of adherence was observed: only 25.6% (n=11) of the participants received "regularly" the medications. Twenty-six DRP was identified, and 84.6% (n=22) were classified as real. There were no significant associations between socio-demographic variables and adherence, although some caregivers have reported difficulty in accessing the medicines and in understanding the treatment. CONCLUSION: Our findings showed problems in the adherence process related to inattention, forgetfulness and irregularity in getting medicines, reinforcing the need for the development of strategies to facilitate a better understanding of treatment and to ensure adherence.

Medication adherence in patients with juvenile idiopathic arthritis / Adesão ao tratamento farmacológico em pacientes com artrite idiopática juvenil por meio de questionários

ABSTRACT Objective: The aim of this study was to investigate pharmacological treatment adherence of patients with juvenile idiopathic arthritis, attended in an outpatient pharmacy at a tertiary hospital in northeastern Brazil. Methods: The analysis of adherence was performed along with caregivers, through a structured questionnaire based on Morisky, Green and Levine, which enabled the categorization of adherence in “highest”, “moderate” or “low” grades, and through evaluating medication dispensing registers, which classified the act of getting medications at the pharmacy as “regular” or “irregular”. Drug Related Problems (DRP) were identified through the narrative of caregivers and classified according to the Second Granada Consensus. Then, a pharmaceutical orientation chart with information about the therapeutic regimen was applied, in order to function as a guide for issues that influenced adherence. Results: A total of 43 patients was included, with a mean age of 11.12 years, and 65.1% (n = 28) were female. Applying the questionnaire, it was found “highest” adherence in 46.5% (n = 20) patients, “moderate” adherence in 48.8% (n = 21), and “low” adherence in 4.7% (n = 2). Through an analysis of the medication dispensing registers, a lower level of adherence was observed: only 25.6% (n = 11) of the participants received “regularly” the medications. Twenty-six DRP was identified, and 84.6% (n = 22) were classified as real. There were no significant associations between socio-demographic variables and adherence, although some caregivers have reported difficulty in accessing the medicines and in understanding the treatment. Conclusion: Our findings showed problems in the adherence process related to inattention, forgetfulness and irregularity in getting medicines, reinforcing the need for the development of strategies to facilitate a better understanding of treatment and to ensure adherence.

RESUMO Objetivo: Investigar a adesão ao tratamento farmacológico de pacientes com artrite idiopática juvenil, atendidos na farmácia ambulatorial de hospital terciário do Nordeste do Brasil. Métodos: A análise da adesão foi feita junto aos cuidadores, por meio de questionário estruturado com base no teste de Morisky, Green e Levine, que viabilizou a categorização da adesão em “máxima”, “moderada” ou “baixa”, e da avaliação dos registros de dispensação dos medicamentos, que classificou a retirada de medicamentos na farmácia como “regular” ou “irregular”. Os problemas relacionados com medicamentos (PRM) foram identificados por meio da narrativa dos cuidadores e classificados conforme o Segundo Consenso de Granada. Em seguida, aplicou-se uma tabela de orientação farmacêutica, que contém informações sobre o esquema terapêutico, de forma a esclarecer questões que influenciavam a adesão. Resultados: Participaram 43 pacientes, com média de 11,12 anos, 65,1%, (n = 28) do sexo feminino. Por meio do questionário, verificou-se adesão “máxima” em 46,5% (n = 20) dos pacientes, “moderada” em 48,8% (n = 21) e “baixa” em 4,7% (n = 2). Pelo registro de dispensação, observou-se um nível de adesão menor: apenas 25,6% (n = 11) dos participantes receberam os medicamentos “regularmente”. Identificaram-se 26 PRM, 84,6% (n = 22) classificados como “reais”. Não foram observadas associações significativas entre as variáveis sociodemográficas e a adesão, embora alguns cuidadores tenham relatado dificuldade de acesso ao medicamento e de compreensão do tratamento. Conclusão: Nossos achados demonstraram falhas no processo de adesão, relacionadas ao descuido, esquecimento e à irregularidade para receber os medicamentos, o que reforça a necessidade de estratégias para facilitar a compreensão do tratamento e garantir a adesão

Medication adherence in patients with juvenile idiopathic arthritis. / Adesão ao tratamento farmacológico em pacientes com artrite idiopática juvenil por meio de questionários.

OBJECTIVE: The aim of this study was to investigate pharmacological treatment adherence of patients with juvenile idiopathic arthritis, attended in an outpatient pharmacy at a tertiary hospital in northeastern Brazil. METHODS: The analysis of adherence was performed along with caregivers, through a structured questionnaire based on Morisky, Green and Levine, which enabled the categorization of adherence in "highest", "moderate" or "low" grades, and through evaluating medication dispensing registers, which classified the act of getting medications at the pharmacy as "regular" or "irregular". Drug Related Problems (DRP) were identified through the narrative of caregivers and classified according to the Second Granada Consensus. Then, a pharmaceutical orientation chart with information about the therapeutic regimen was applied, in order to function as a guide for issues that influenced adherence. RESULTS: A total of 43 patients was included, with a mean age of 11.12 years, and 65.1% (n=28) were female. Applying the questionnaire, it was found "highest" adherence in 46.5% (n=20) patients, "moderate" adherence in 48.8% (n=21), and "low" adherence in 4.7% (n=2). Through an analysis of the medication dispensing registers, a lower level of adherence was observed: only 25.6% (n=11) of the participants received "regularly" the medications. Twenty-six DRP was identified, and 84.6% (n=22) were classified as real. There were no significant associations between socio-demographic variables and adherence, although some caregivers have reported difficulty in accessing the medicines and in understanding the treatment. CONCLUSION: Our findings showed problems in the adherence process related to inattention, forgetfulness and irregularity in getting medicines, reinforcing the need for the development of strategies to facilitate a better understanding of treatment and to ensure adherence.