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.

Sleep and restless legs syndrome in female adolescents with idiopathic musculoskeletal pain / Sono e síndrome das pernas inquietas em adolescentes do sexo feminino com dor musculoesquelética idiopática

Abstract Objectives: To assess the presence of restless legs syndrome, periodic leg movement, and sleep disorders in female adolescents with idiopathic musculoskeletal pain through a sleep scale and polysomnography, and to compare these data in adolescents without pain history. Method: Twenty-six adolescents diagnosed with idiopathic musculoskeletal pain followed in a pain outpatient clinic and 25 healthy controls matched by age and education were recruited. The restless legs syndrome criteria were evaluated according to the International Restless Legs Syndrome Study Group, the Sleep Disturbance Scale for Children was completed, nocturnal polysomnography was performed, and anxiety symptoms were recorded. Results: The mean age of idiopathic musculoskeletal pain adolescents was 13.9 ± 1.6 years; in controls, it was 14.4 ± 1.4 years. One adolescent in the control group (4 %) and nine patients with idiopathic musculoskeletal pain (34.6 %) fulfilled the restless legs syndrome criteria (p = 0.011). The authors did not observe significant differences in Sleep Disturbance Scale for Children scores between the groups in all components: disorders of initiating and maintaining sleep (p = 0.290), sleep breathing disorders (p = 0.576), disorders of arousal (p = 0.162), sleep-wake transition disorders (p = 0.258), disorder of excessive daytime somnolence (p = 0.594), and sleep hyperhidrosis (p = 0.797). The neurophysiological, respiratory, and periodic leg movement parameters were similar in both groups. Having anxiety was not associated with restless legs syndrome (p = 0.11). Three patients with idiopathic musculoskeletal pain (11.5 %) presented restless legs syndrome and periodic leg movement simultaneously, which was absent in the control group. Conclusion: Female adolescents with idiopathic musculoskeletal pain present criteria for RLS more frequently than healthy adolescents. However, this study did not observe relevant changes in objective and subject sleep variables.

Resumo Objetivos: Avaliar a presença de síndrome das pernas inquietas, movimento periódico das pernas e distúrbios do sono em adolescentes do sexo feminino com dor musculoesquelética idiopática por meio da escala do sono e da polissonografia e comparar esses dados em adolescentes sem histórico de dor. Método: Foram recrutados 26 adolescentes diagnosticados com dor musculoesquelética idiopática acompanhados em um ambulatório de dor e 25 controles saudáveis pareados por idade e escolaridade. Avaliamos os critérios da síndrome das pernas inquietas de acordo com o Grupo Internacional de Estudos de Síndrome das Pernas Inquietas, a Escala de Distúrbios do Sono em Crianças, a polissonografia noturna e os sintomas de ansiedade. Resultados: A idade média dos adolescentes com dor musculoesquelética idiopática foi 13,9 ± 1,6 anos e dos controles foi 14,4 ± 1,4 anos. Um adolescente no grupo de controle (4%) e nove pacientes com dor musculoesquelética idiopática (34,6%) atenderam aos critérios da síndrome das pernas inquietas (p = 0,011). Não observamos diferenças significativas nos escores da Escala de Distúrbios do Sono em Crianças entre os grupos em todos os componentes: distúrbios do início e da manutenção do sono (p = 0,290), distúrbios respiratórios do sono (p = 0,576), distúrbios do despertar (p = 0,162), distúrbios da transição sono-vigília (p = 0,258), sonolência diurna excessiva (p = 0,594) e hiperidrose do sono (p = 0,797). Os parâmetros neurofisiológicos, respiratórios e o movimento periódico das pernas foram semelhantes nos dois grupos. Ansiedade não foi associada à síndrome das pernas inquietas (p = 0,11). Três pacientes com dor musculoesquelética idiopática (11,5%) apresentaram síndrome das pernas inquietas e movimento periódico das pernas simultaneamente, situação ausente no grupo de controle. Conclusão: As adolescentes do sexo feminino com dor musculoesquelética idiopática apresentaram critérios para síndrome das pernas inquietas com mais frequência do que as adolescentes saudáveis. Contudo, não observamos mudanças relevantes nas variáveis do sono objetivas e subjetivas.

Percepção e enfrentamento da dor em crianças e adolescentes com fibromialgia juvenil e artrite idiopática juvenil poliarticular / Pain perception and pain coping mechanisms in children and adolescents with juvenile fibromyalgia and polyarticular juvenile idiopathic arthritis

RESUMO Objetivo: Mensurar e comparar a dor musculoesquelética em pacientes com fibromialgia juvenil (FMJ) e em pacientes com artrite idiopática juvenil poliarticular (AIJ); e avaliar e comparar a percepção e o enfrentamento da dor. Métodos: Foram avaliados, em estudo transversal, 150 crianças e adolescentes (e seus respectivos pais), divididos em três grupos: FMJ, AIJ e controles saudáveis. A mensuração e o enfrentamento da dor foram realizados por meio de instrumentos específicos. Para a avaliação da percepção da dor, desenvolveram-se três vinhetas com simulação de situações que pudessem gerar dor: aplicação de injeção, queda de bicicleta e isolamento social. Os pais e os pacientes responderam individualmente quanto à percepção da dor em cada situação. Resultados: As maiores notas de dor, os menores escores de enfrentamento da dor, as maiores notas para a percepção da dor nas vinhetas e os piores índices de qualidade de vida relacionada à saúde foram observados nos pacientes com FMJ, quando comparados aos pacientes com AIJ e aos controles. O mesmo padrão foi observado com os respectivos pais. Conclusões: Pacientes com AIJ e FMJ se comportam diferentemente em relação à percepção da dor e ao desenvolvimento de técnicas para o enfrentamento da dor. A dor deve ser avaliada sob diferentes perspectivas para um planejamento mais individualizado e efetivo do tratamento desses pacientes.

ABSTRACT Objective: To measure and compare musculoskeletal pain in patients with juvenile fibromyalgia (JFM) and polyarticular juvenile idiopathic arthritis (JIA), and to evaluate and compare pain perception and pain coping mechanisms in these patients. Methods: In this cross sectional study, we evaluated 150 children and adolescents, and their respective parents, from 3 different groups: JFM, polyarticular JIA, and healthy controls. Pain intensity and pain coping mechanisms were measured using specific questionnaires. Pain perception was evaluated according to three illustrations simulating situations that might cause pain: a shot, a bicycle fall, and social isolation. The patients' parents also filled out the questionnaires and provided a pain score that matched their child's perception of pain for each illustration. Results: The highest pain scores, the lowest pain coping strategy scores, the highest pain perception scores for all three illustrations, and the worse health related to quality of life indicators were observed in the JFM group, when compared to the JIA and control groups. The same pattern was observed with their parents. Conclusions: Patients with JIA and JFM behave differently in relation to pain perception and the development pain coping mechanisms. Pain should be evaluated from different perspectives for an individualized and efficient treatment of patients.

The profile and clinical outcomes of patients with renal involvement due to IgA vasculitis: is azathioprine a good option for treatment?

Abstract Background: The Henoch-Schonlein Purpura (HSP) or IgA vasculitis is the most common vasculitis of childhood and may occur with renal involvement, with hematuria and / or proteinuria, and may cause severe and non-reversible sequelae. Objectives: To establish the profile of patients with renal involvement due to IgA vasculitisand to describe our experience with the use of azathioprine to treat patients with nephritis. Methods: Clinical data were retrospectively collected from medical records of patients with IgA vasculitiswho attended the pediatric rheumatology unit between 1995 and 2017. Patients were separated into two groups based on whether or notthey weretreated with non-glucocorticoid immunosuppressants. Results: From the178 patients with IgA vasculitis, nephritis was found in67 patients (37.6%), 13 of whom receivedtreatment with non-glucocorticoid immunosuppressants. Ten patients responded well to azathioprine and 1 patient to cyclosporine. Forty patients received oral glucocorticoids, whilst 16received intravenous glucocorticoids. Conclusion: Azathioprine may be beneficial in the treatment of IgA vasculitis with renal involvement.

Sleep, stress, neurocognitive profile and healthrelated quality of life in adolescents with idiopathic musculoskeletal pain

OBJECTIVES: The aims of this study were to measure levels of sleep, stress, and depression, as well as health-related quality of life, and to assess the neurocognitive profiles in a sample of adolescents with idiopathic musculoskeletal pain. METHODS: Nineteen adolescents with idiopathic musculoskeletal pain and 20 age-matched healthy control subjects were evaluated regarding their levels of sleep and stress, as well as quality of life, and underwent neurocognitive testing. RESULTS: The sample groups consisted predominantly of females (84%), and the socioeconomic status did not differ between the two groups. In addition, the occurrence of depressive symptoms was similar between the two groups; specifically, 26% of the idiopathic musculoskeletal pain patients and 30% of the control subjects had scores indicative of depression. Teenagers in the group with idiopathic musculoskeletal pain reported poorer quality of life and sleep scores than those in the control group. Regarding stress, patients had worse scores than the control group; whereas 79% of the adolescents with idiopathic musculoskeletal pain met the criteria for a diagnosis of stress, only 35% of the adolescents in the control group met the criteria. In both groups, we observed scores that classified adolescents as being in the resistance phase (intermediate) and exhaustion phase (pathological) of distress. However, the idiopathic musculoskeletal pain group more frequently reported symptomatic complaints of physical and emotional distress. The neurocognitive assessment showed no significant impairments in either group. CONCLUSION: Adolescents with idiopathic musculoskeletal pain did not exhibit cognitive impairments. However, adolescents with idiopathic musculoskeletal pain did experience intermediate to advanced psychological distress and lower health-related quality of life, which may increase their risk of cognitive dysfunction in the future.

Alterações oftalmológicas decorrentes do tratamento do lúpus eritematoso sistêmico juvenil / Ocular changes due to the treatment of juvenile systemic lupus erythematosus

OBJETIVO: Avaliar retrospectivamente as alterações oftalmológicas de crianças e adolescentes com lúpus eritematoso sistêmico juvenil (LESJ) em um serviço de reumatologia pediátrica terciário. MÉTODOS: Avaliamos 117 pacientes com LESJ (85,5% do gênero feminino, 60,7% não caucasoides) com média de idade de 10,4 anos e média de tempo de evolução da doença de 5,4 anos que preenchiam no mínimo quatro critérios de classificação do LES de acordo com o American College of Rheumatology de 1997. Aplicamos um protocolo que continha dados clínicos e demográficos, queixas e alterações oftalmológicas, idade do início, tempo de uso e dose cumulativa das medicações. RESULTADOS: Dos 117 pacientes, 24 (20,5%) apresentaram alterações oftalmológicas. Destes, 16 apresentaram alteração de fundo de olho associada a hipertensão arterial sistêmica e/ou uso de cloroquina, quatro apresentaram catarata, dois apresentaram glaucoma e dois apresentaram catarata e glaucoma. A média de idade do aparecimento das alterações oftalmológicas foi de 14,1 anos. Os pacientes com alterações oftalmológicas receberam, estatisticamente, maiores doses e tempos de pulsoterapia de glicocorticoide em relação aos pacientes sem alterações oftalmológicas [1,5 (0,4-1,6) versus 1 (0,2-1,6) mg/kg, P = 0,003; 25,7 (2-99) versus 17,8 (1-114) meses, P = 0,0001; respectivamente]. CONCLUSÃO: Verificamos alta prevalência de alterações oftalmológicas relacionadas principalmente ao tratamento do LESJ, o que demonstra a necessidade de avaliações regulares mesmo em pacientes assintomáticos, visando ao diagnóstico e intervenção precoces e à diminuição da morbidade ocular relacionada a essa doença.

OBJECTIVE: To assess retrospectively the ocular changes in children and adolescents with juvenile systemic lupus erythematosus (JSLE) in a tertiary pediatric rheumatology service. METHODS: This study assessed 117 JSLE patients (85.5% female, 60.7% non-Caucasian), who met at least four criteria of the 1997 SLE classification of the American College of Rheumatology. Their mean age was 10.4 years, and their mean time of disease progression was 5.4 years. A protocol containing clinical and demographic data, ophthalmologic complaints and changes, age of onset, duration of medication use, and cumulative medication dose was applied. RESULTS: Of the 117 patients, 24 (20.5%) had ocular changes. Sixteen of them had abnormal fundoscopy associated with systemic hypertension and/or use of chloroquine; four had cataract; two had glaucoma; and two had cataract and glaucoma. The mean age of ocular change onset was 14.1 years. Patients with ocular changes received statistically higher and longer doses of glucocorticoid pulse therapy as compared with patients without ocular changes [1.5 (0.4 to 1.6) versus 1 (0.2 to 1.6) mg/kg, P = 0.003; 25.7 (2-99) versus 17.8 (1-114) months, P = 0.0001, respectively]. CONCLUSION: A high prevalence of ocular changes relating mainly to the treatment of JSLE was observed. This demonstrates the need for regular ophthalmologic examinations even in asymptomatic patients, aiming at the early diagnosis and intervention, and at decreasing the ocular morbidity related to that disease.

Dor musculoesquelética idiopática difusa na infância e na adolescência / Diffuse idiopathic musculoskeletal pain in childhood and adolescence

OBJETIVO: A dor musculoesquelética difusa idiopática persistente ou intermitente é definida pela presença de dor com duração de três meses ou mais, sem etiologia aparente, e tem acometido cada vez mais crianças e adolescentes. Este estudo objetivou relatar dois pacientes com dor incapacitante submetidos à abordagem e ao tratamento multiprofissional, além de discutir o tratamento conduzido, por meio de revisão da literatura atual. DESCRIÇÃO DO CASO: O primeiro caso é de uma menina com nove anos de idade, com histórico de cefaleia, dor mus-culoesquelética e dor abdominal há dois anos, período no qual apresentou quatro episódios de ausência de marcha, sem causas orgânicas aparentes. O segundo caso refere-se a uma adolescente de 14 anos com lombalgia diária há 14 meses de forte intensidade, com irradiação para membros inferiores, acompanhada de cefaleia, fadiga e adinamia. Ambas apresentavam exame físico normal, exceto pela presença de alodínia (caso 1) e pontos de fibromialgia (caso 2); além de exames subsidiários normais, sem justificativas orgânicas para as queixas dolorosas. Houve melhora clínica significativa após diagnóstico e tratamento diferencial por meio de abordagem multiprofissional (médico, psicólogo, fisioterapeuta e nutricionista), com retomada das atividades regulares pelas pacientes. COMENTÁRIOS: A atuação integrada da equipe em um curto período de tempo proporcionou às pacientes a retomada de suas atividades normais, visto que fatores emocionais, físicos e nutricionais relacionam-se à dor.

OBJECTIVE: Persistent or intermittent diffuse idiopathic musculoskeletal pain is defined by the presence of pain over three months with no apparent etiology. The diagnosis of this condition in children and adolescents is increasing. The objective of this study was to report the cases of two patients with disabling pain that received multidiscipli-nary treatment and to review the current literature about this subject. CASE DESCRIPTION: The first patient is a nine-year old girl with history of headache, musculoskeletal pain and abdominal pain for two years. During this period she had four episodes of absence of gait with no apparent organic cause. The second patient is a 14-year girl with severe daily low back pain for 14 months, followed by headache and fatigue. Both patients presented a normal physical examina-tion, except for allodynia (case 1) and fibromyalgia tender points (case 2). All tests resulted negative regarding the search for organic reasons for pain complaints. Significant clinical improvement was observed with a multidisciplinary approach offered by pediatricians, psychologists, physical therapists, and nutritionists. Both patients resumed their daily regular activities. COMMENTS: Team integrated performance in a short-term period can provide the attenuation of pain symptoms since emotional, physical and nutritional factors are related to the pain expressed by the patients.