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.

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.

Factors associated with adherence to treatment in children and adolescents with chronic rheumatic diseases.

OBJECTIVE: There are several factors that contribute to poor adherence to treatment in children and adolescents with chronic rheumatic diseases, worsening their quality of life and prognosis. Our aim was to assess the rates of adherence to treatment and to identify the socioeconomic and clinical factors associated. METHODS: The sample included 99 patients with juvenile idiopathic arthritis, systemic erythematosus lupus, dermatomyositis or juvenile scleroderma. All patients were followed at the outpatient pediatric rheumatology for a minimum period of 6 months. To assess adherence, a questionnaire was administered to the providers, which included three blocks: 1) demographic, clinical and laboratory data; 2) medication adherence; and 3) attending follow-up appointments, examinations and use of orthoses. A value lower than or equal to 80% of the prescribed was considered poor adherence. RESULTS: A total of 53% of patients showed good overall adherence, observed when the caregiver lived in a stable union marital status (p = 0.006); 20 patients (20.2%) presented poor medication adherence, related to the use of three or more medications daily (p = 0.047). The causes of poor adherence were forgetfulness, refusal, incorrect dose or lack of medication, personal problems, and financial difficulties. CONCLUSIONS: We observed good overall treatment adherence in patients whose providers lived in stable union and poor adherence to medication in patients who used more than three types of medication daily. There was no association between the adherence rates and sex, age, time since diagnosis and disease activity.

Fatores associados à adesão ao tratamento de crianças e adolescentes com doenças reumáticas crônicas / Factors associated with adherence to treatment in children and adolescents with chronic rheumatic diseases

OBJETIVO: São vários os fatores que contribuem para a má adesão ao tratamento de crianças e adolescentes com doenças reumáticas crônicas, gerando piora da qualidade de vida e do prognóstico. Nosso objetivo foi avaliar as taxas de adesão ao tratamento e identificar os fatores socioeconômicos e clínicos associados. MÉTODOS: Foram incluídos 99 pacientes com artrite idiopática juvenil, lúpus eritematoso sistêmico, dermatomiosite ou esclerodermia juvenil. Todos os pacientes eram acompanhados no ambulatório de reumatologia pediátrica por um período mínimo de 6 meses. Para avaliação da adesão, foi aplicado aos cuidadores um questionário composto por três blocos: 1) dados demográficos, clínicos e laboratoriais; 2) adesão ao tratamento medicamentoso; e 3) comparecimento às consultas, realização de exames e utilização de órteses. Foi considerada má adesão, quando realizado valor menor ou igual a 80% do prescrito. RESULTADOS: Um total de 53% dos pacientes apresentou boa adesão ao tratamento global, observada quando o cuidador possuía união estável (p = 0,006); 20 pacientes (20,2%) apresentaram má adesão ao tratamento medicamentoso, relacionada à utilização de mais que três medicamentos diários (p = 0,047). As causas de má adesão ao tratamento foram esquecimento, recusa, dose incorreta ou falta de medicamento, problemas pessoais e dificuldades financeiras. CONCLUSÕES: Observamos boa adesão ao tratamento global nos pacientes cujos cuidadores possuíam união estável e má adesão ao tratamento medicamentoso nos pacientes que utilizavam mais que três tipos de medicamentos diariamente. Não houve associação entre as taxas de adesão ao tratamento e sexo, idade, tempo de diagnóstico e atividade da doença.

OBJECTIVE: There are several factors that contribute to poor adherence to treatment in children and adolescents with chronic rheumatic diseases, worsening their quality of life and prognosis. Our aim was to assess the rates of adherence to treatment and to identify the socioeconomic and clinical factors associated. METHODS: The sample included 99 patients with juvenile idiopathic arthritis, systemic erythematosus lupus, dermatomyositis or juvenile scleroderma. All patients were followed at the outpatient pediatric rheumatology for a minimum period of 6 months. To assess adherence, a questionnaire was administered to the providers, which included three blocks: 1) demographic, clinical and laboratory data; 2) medication adherence; and 3) attending follow-up appointments, examinations and use of orthoses. A value lower than or equal to 80% of the prescribed was considered poor adherence. RESULTS: A total of 53% of patients showed good overall adherence, observed when the caregiver lived in a stable union marital status (p = 0.006); 20 patients (20.2%) presented poor medication adherence, related to the use of three or more medications daily (p = 0.047). The causes of poor adherence were forgetfulness, refusal, incorrect dose or lack of medication, personal problems, and financial difficulties. CONCLUSIONS: We observed good overall treatment adherence in patients whose providers lived in stable union and poor adherence to medication in patients who used more than three types of medication daily. There was no association between the adherence rates and sex, age, time since diagnosis and disease activity.

Henoch-Schõnlein purpura: recurrence and chronicity

OBJETIVOS: Descrever um grupo de pacientes com púrpura de Henoch-Schõnlein seguido em nosso serviço, com ênfase nos casos recorrentes e crônicos, e comparar pacientes com doença monocíclica ou recorrente segundo características clínicas e demográficas. MÉTODOS: Foram avaliados dados de 67 pacientes atendidos desde o início da doença. Doze pacientes foram excluídos, pois abandonaram o seguimento antes de 3 meses de acompanhamento, sendo incluídas 55 crianças no estudo. Recorrência foi definida pela presença de novo quadro após um período de 3 meses sem sintomas, e cronicidade como persistência de manifestações cutâneas, abdominais e renais por um período ≥ 12 meses. RESULTADOS: Foi observada recorrência em 8/55 pacientes (14,4 por cento) e cronicidade em quatro (7,2 por cento). Em 29/55 pacientes (52,7 por cento), foi constatada infecção como fator desencadeante. Curso monocíclico esteve presente em 43 pacientes (26 meninas, média de idade de 5,4 anos). Acometimento gastrointestinal e renal foi observado em 55,8 e 20,9 por cento dos pacientes, respectivamente. Dentre os 12 pacientes com púrpura de Henoch-Schõnlein recorrente ou crônica, três apresentaram artrite, quatro tiveram sinais e sintomas de comprometimento abdominal e sete de doença renal: hematúria microscópica em cinco, hematúria macroscópica em um e hematúria com proteinúria em um paciente. Idade de início tardia foi a única variável relacionada com a presença de recorrência (p < 0,05). CONCLUSÕES: Como observado na literatura médica, o curso monocíclico é mais freqüente nas crianças com início da doença em idade mais precoce. Pacientes com púrpura de Henoch-Schõnlein devem ser seguidos a longo prazo, uma vez que recorrências e/ou cronicidade são observadas em mais de 20 por cento dos casos.

OBJECTIVES: To describe a group of patients treated at our service for Henoch-Schõnlein 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 percent) and four cases were chronic (7.2 percent). In 29/55 patients (52.7 percent), 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 percent of patients, respectively. Among the 12 patients with recurrent or chronic Henoch-Schõnlein 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-Schõnlein purpura should be followed over the long term, since recurrent and chronic cases account for more than 20 percent of the total.