Abstract Objective: To evaluate autoimmune hepatitis (AIH) in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients. Methods: This retrospective multicenter study included 847 patients with cSLE, performed in 10 Pediatric Rheumatology services of São Paulo state, Brazil. AIH was defined according to the International Autoimmune Hepatitis Group criteria (IAHGC). The statistical analysis was performed using the Bonferroni's correction (p < 0.0033). Results: AIH in cSLE patients confirmed by biopsy was observed in 7/847 (0.8%) and all were diagnosed during adolescence. The majority occurred before or at cSLE diagnosis [5/7 (71%)]. Antinuclear antibodies were a universal finding, 43% had concomitantly anti-smooth muscle antibodies and all were seronegative for anti-liver kidney microsomal antibodies. All patients with follow-up ≥18 months (4/7) had complete response to therapy according to IAHGC. None had severe hepatic manifestations such as hepatic failure, portal hypertension and cirrhosis at presentation or follow-up. Further comparison of 7 cSLE patients with AIH and 28 without this complication with same disease duration [0 (0–8.5) vs. 0.12 (0–8.5) years, p = 0.06] revealed that the frequency of hepatomegaly was significantly higher in cSLE patients in the former group (71% vs. 11%, p = 0.003) with a similar median SLEDAI-2 K score [6 (0–26) vs. 7 (0–41), p = 0.755]. No differences were evidenced regarding constitutional involvement, splenomegaly, serositis, musculoskeletal, neuropsychiatric and renal involvements, and treatments in cSLE patients with and without AIH (p > 0.0033). Conclusions: Overlap of AIH and cSLE was rarely observed in this large multicenter study and hepatomegaly was the distinctive clinical feature of these patients. AIH occurred during adolescence, mainly at the first years of lupus and it was associated with mild liver manifestations.

Abstract Objective: To evaluate prevalence, clinical manifestations, laboratory abnormalities and treatment in a multicenter cohort study including 847 childhood-onset systemic lupus erythematosus (cSLE) patients with and without diffuse alveolar hemorrhage (DAH), as well as concomitant parameters of severity. Methods: DAH was defined as the presence of at least three respiratory symptoms/signs associated with diffuse interstitial/alveolar infiltrates on chest x-ray or high-resolution computer tomography and sudden drop in hemoglobin levels. Statistical analysis was performed using Bonferroni correction (p < 0.0022). Results: DAH was observed in 19/847 (2.2%) cSLE patients. Cough/dyspnea/tachycardia/hypoxemia occurred in all cSLE patients with DAH. Concomitant parameters of severity observed were: mechanical ventilation in 14/19 (74%), hemoptysis 12/19 (63%), macrophage activation syndrome 2/19 (10%) and death 9/19 (47%). Further analysis of cSLE patients at DAH diagnosis compared to 76 cSLE control patients without DAH with same disease duration [3 (1–151) vs. 4 (1–151) months, p = 0.335], showed higher frequencies of constitutional involvement (74% vs. 10%, p < 0.0001), serositis (63% vs. 6%, p < 0.0001) and sepsis (53% vs. 9%, p < 0.0001) in the DAH group. The median of disease activity score(SLEDAI-2 K) was significantly higher in cSLE patients with DAH [18 (5–40) vs. 6 (0–44), p < 0.0001]. The frequencies of thrombocytopenia (53% vs. 12%, p < 0.0001), intravenous methylprednisolone (95% vs. 16%, p < 0.0001) and intravenous cyclophosphamide (47% vs. 8%, p < 0.0001) were also significantly higher in DAH patients. Conclusions: This was the first study to demonstrate that DAH, although not a disease activity score descriptor, occurred in the context of significant moderate/severe cSLE flare. Importantly, we identified that this condition was associated with serious disease flare complicated by sepsis with high mortality rate.

INTRODUÇÃO: A reumatologia pediátrica (RP) é uma especialidade emergente, com número restrito de especialistas, e ainda não conta com uma casuística brasileira sobre o perfil dos pacientes atendidos e as informações sobre a formação de profissionais capacitados. OBJETIVO: Estudar o perfil dos especialistas e dos serviços em RP e as características dos pacientes com doenças reumáticas nessa faixa etária a fim de estimar a situação atual no estado de São Paulo (ESP). PACIENTES E MÉTODOS: No ano de 2010 o departamento científico de RP da Sociedade de Pediatria de São Paulo encaminhou um questionário respondido por 24/31 especialistas com título de especialização em RP que atuam no ESP e por 8/12 instituições com atendimento nesta especialidade. RESULTADOS: A maioria (91%) dos profissionais exerce suas atividades em instituições públicas. Clínicas privadas (28,6%) e instituições (37,5%) relataram não ter acesso ao exame de capilaroscopia e 50% das clínicas privadas não tem acesso à acupuntura. A média de tempo de prática profissional na especialidade foi de 9,4 anos, sendo 67% deles pós-graduados. Sete (87,5%) instituições públicas atuam na área de ensino, formando novos reumatologistas pediátricos. Cinco (62,5%) delas têm pós-graduação. Dois terços dos especialistas utilizam imunossupressores e agentes biológicos de uso restrito pela Secretaria da Saúde. A doença mais atendida foi artrite idiopática juvenil (29,1%-34,5%), seguida de lúpus eritematoso sistêmico juvenil (LESJ) (11,6%-12,3%) e febre reumática (9,1%-15,9%). Vasculites (púrpura de Henoch Schönlein, Wegener, Takayasu) e síndromes autoinflamatórias foram mais incidentes nas instituições públicas (P = 0,03; P = 0,04; P = 0,002 e P < 0,0001, respectivamente). O LESJ foi a doença com maior mortalidade (68% dos óbitos), principalmente por infecção. CONCLUSÃO: A RP no ESP conta com um número expressivo de especialistas pós-graduados, que atuam especialmente em instituições de ensino, com infraestrutura adequada ao atendimento de pacientes de alta complexidade.

INTRODUCTION: Paediatric rheumatology (PR) is an emerging specialty, practised by a limited number of specialists. Currently, there is neither a record of the profile of rheumatology patients being treated in Brazil nor data on the training of qualified rheumatology professionals in the country. OBJECTIVE: To investigate the profile of PR specialists and services, as well as the characteristics of paediatric patients with rheumatic diseases, for estimating the current state of rheumatology in the state of São Paulo. PATIENTS AND METHODS: In 2010, the scientific department of PR of the Paediatric Society of São Paulo administered a questionnaire that was answered by 24/31 accredited specialists in PR practising in state of São Paulo and by 8/21 institutions that provide PR care. RESULTS: Most (91%) of the surveyed professionals practise in public institutions. Private clinics (28.6%) and public institutions (37.5%) reported not having access to nailfold capillaroscopy, and 50% of the private clinics reported not having access to acupuncture. The average duration of professional practise in PR was 9.4 years, and 67% of the physicians had attended postgraduate programmes. Seven (87.5%) public institutions perform teaching activities, in which new paediatric rheumatologists are trained, and five (62.5%) offer postgraduate programmes. Two-thirds of the surveyed specialists use immunosuppressants and biological agents classified as "restricted use" by the Health Secretariat. The disease most frequently reported was juvenile idiopathic arthritis (29.1-34.5%), followed by juvenile systemic lupus erythematosus (JSLE) (11.6-12.3%) and rheumatic fever (9.1-15.9%). The incidence of vasculitis (including Henoch-Schönlein purpura, Wegener's granulomatosis, and Takayasu's arteritis) and autoinflammatory syndromes was higher in public institutions compared to other institutions (P = 0.03, P = 0.04, P = 0.002, and P < 0.0001, respectively). Patients with JSLE had the highest mortality rate (68% of deaths), mainly due to infection. CONCLUSION: The field of PR in the state of São Paulo has a significant number of specialists with postgraduate degrees who mostly practise at teaching institutions with infrastructures appropriate for the care of high-complexity patients.

OBJETIVOS: Avaliar o consumo alimentar de crianças e adolescentes com artrite idiopática juvenil (AIJ) e lúpus eritematoso sistêmico (LES) por recordatório de 24 horas e relacioná-lo com características clínicas e antropométricas e com os medicamentos empregados. MÉTODOS: Em estudo transversal, avaliamos os recordatórios de 24 horas de pacientes ambulatoriais. O estado nutricional foi classificado pelo CDC, 2000. Para o cálculo da ingestão, utilizamos o software NutWin UNIFESP-EPM. Para a análise quantitativa e qualitativa, adotamos as Recommended Dietary Allowances e a pirâmide alimentar brasileira. RESULTADOS: A mediana de idade foi 12 na AIJ e 16,5 anos no LES. Na AIJ, 37,5% dos pacientes estavam em atividade de doença, e, no LES, 68,2% tinham Systemic Lupus Erythematosus Disease Activity Index > 4. Foi encontrada desnutrição em 8,3 e 4,5% dos pacientes com AIJ e com LES, respectivamente, e obesidade, em 16,7 e 18,2%. Na AIJ, o consumo excessivo de energia, proteína e lipídios foi de 12,5, 75 e 31,3%, respectivamente. No LES, o consumo excessivo de energia, proteína e lipídios foi de 13,6, 86,4 e 36,4%, respectivamente. Consumo deficiente de ferro, zinco e vitamina A foi observado em 29,2 e 50, 87,5 e 86,4 e 87,5 e 95,2% dos pacientes com AIJ e LES, respectivamente. Não houve relação significante entre consumo, atividade da doença e estado nutricional. CONCLUSÃO: Pacientes com doenças reumáticas apresentam inadequação do consumo alimentar. Ressaltamos a ingestão excessiva de lipídios e proteínas e a ingestão insuficiente de micronutrientes.

OBJECTIVES: To evaluate the dietary intake of children and adolescents with juvenile idiopathic arthritis (JIA) and juvenile systemic lupus erythematosus (JSLE) using a 24-hour diet recall and relating it to the patients clinical and anthropometric characteristics and to the drugs used in their treatment. METHODS: By means of a cross-sectional study, we assessed the 24-hour diet recalls of outpatients. Their nutritional status was classified according to the CDC (2000). The computer program NutWin UNIFESP-EPM was used for food intake calculation. The Recommended Dietary Allowances and the Brazilian food pyramid were used for quantitative and qualitative analysis. RESULTS: Median age was 12 years for JIA patients and 16.5 years for JSLE patients. Among the JIA patients, 37.5% had active disease, and among the JSLE patients, 68.2% showed Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) > 4. Malnutrition was found in 8.3 and 4.5% of the JIA and JSLE patients, respectively, and obesity was present in 16.7 and 18.2%. For JIA patients, the excessive intake of energy, protein, and lipids was 12.5, 75, and 31.3%, respectively. For JSLE patients, the excessive intake of energy, protein, and lipids was 13.6, 86.4, and 36.4%, respectively. Low intake of iron, zinc, and vitamin A was found in 29.2 and 50, 87.5 and 86.4, and 87.5 and 95.2% of the JIA and JSLE patients, respectively. There was not a significant association between intake, disease activity, and nutritional status. CONCLUSION: Patients with rheumatic diseases have inadequate dietary intake. There is excessive intake of lipids and proteins and low intake of micronutrients.

OBJETIVO: Nas doenças reumáticas, principalmente dermatomiosite e esclerodermia, a capilaroscopia periungueal é importante auxílio no diagnóstico e no seguimento dos pacientes. Em adultos, é descrita a relação entre a melhora das alterações capilaroscópicas e a melhora da atividade da doença. O nosso objetivo foi avaliar 13 pacientes com miopatias inflamatórias e correlacionar atividades clínica e laboratorial com as alterações da capilaroscopia periungueal. MÉTODOS: Foram incluídos 13 pacientes com dermatomiosite juvenil (critérios de Bohan e Peter) (média de idade de 8,8 anos) e cinco pacientes com síndrome de sobreposição (média de idade de 15,7 anos). Foram avaliadas as atividades clínicas (alterações cutâneas típicas e fraqueza muscular) e laboratoriais (aumento de enzimas musculares e provas inflamatórias) da doença e sua relação com as alterações da capilaroscopia periungueal (presença de ectasias capilares, megacapilares e áreas de deleção). Para a realização da capilaroscopia, foi utilizado um microscópio com iluminação especial com aumentos de 10 a 16 vezes. RESULTADOS: Foram avaliados 18 pacientes, sete deles em duas ou mais ocasiões, no total de 26 exames capilaroscópicos (13 realizados na fase ativa da doença e 13 na fase inativa). Dos 13 exames durante a fase ativa, 12 apresentavam padrão esclerodérmico; dos 13 realizados na fase inativa, oito não apresentavam alterações de capilaroscopia ou apresentavam melhora importante dos achados. Portanto, em 20 dos 26 exames houve correlação entre atividades clínica e laboratorial e achados capilaroscópicos (p = 0,01). CONCLUSÕES: Em resumo, a capilaroscopia periungueal é um exame não-invasivo que apresenta boa correlação com a atividade da doença e pode ser utilizada para auxiliar no diagnóstico, no seguimento e, principalmente, nas reagudizações da doença.

OBJECTIVE: Nailfold capillaroscopy is an important tool for the diagnosis and follow-up of patients with rheumatic diseases, in particular dermatomyositis and scleroderma. A relationship has been observed in adults between improved capillaroscopic findings and reduced disease activity. Our aim was to correlate disease activity (clinical and laboratory data) and nailfold capillaroscopy findings in 18 patients with inflammatory myopathies. METHODS: This prospective study included 13 juvenile dermatomyositis patients (Bohan and Peter criteria) (mean age of 8.8 years) and five patients with overlap syndrome (mean age of 15.7 years). We evaluated disease activity (skin abnormalities and muscle weakness, muscle enzymes and acute phase reactants) and its correlation with nailfold capillaroscopy findings (dilatation of isolated loops, dropout of surrounding vessels and giant capillary loops). We used a microscope with special light and magnification of 10 to 16X. RESULTS: Eighteen patients underwent a total of 26 capillaroscopic examinations, seven of them on two or more occasions (13 were performed during the active disease phase and 13 during remission). Twelve of the 13 examinations performed during the active phase exhibited scleroderma pattern and 8 of the 13 examinations performed during remission were normal. Therefore, in 20 of the 26 examinations clinical and laboratory data and nailfold capillaroscopy findings correlated (p = 0.01). CONCLUSIONS: Nailfold capillaroscopy is a non-invasive examination that offers satisfactory correlation with disease activity and could be a useful tool for the diagnosis and follow-up of inflammatory myopathies.