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Made available in DSpace on 2019-10-06T15:31:10Z (GMT). No. of bitstreams: 0 Previous issue date: 2018-11-23. Added 1 bitstream(s) on 2021-07-15T15:23:32Z : No. of bitstreams: 1 S2523-31062018000100228.pdf: 598953 bytes, checksum: c510a60909892e9f6d7f26d4469f267b (MD5) Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) 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

Objetivo: Avaliar a prevalência das manifestações clinicas, alterações laboratoriais e tratamento em um estudo de coorte multicêntrico incluindo 847 pacientes com diagnóstico de lúpus eritematoso sistêmico juvenil (LESJ) com ou sem hemorragia pulmonar (HP) assim como parâmetros de gravidade. Métodos: HP foi definida de acordo com a presença de pelo menos três sintomas/sinais respiratórios associados à imagem de infiltrado intersticial/alveolar difuso em radiografia de tórax e/ou tomografia computadorizada e queda aguda dos níveis de hemoglobina. Analise estatística foi realizada utilizando-se correção de Bonferroni (p < 0,0022). Resultados: HP foi observada em 19/847 (2,2%) dos pacientes com LESJ. Tosse, dispneia, taquicardia e/ou hipoxemia ocorreram em todos os pacientes com HP. Parâmetros de gravidade associados observados foram: ventilação mecânica em 14/19 (74%), hemoptise 12/19 (63%), síndrome de ativação macrofágica 2/19 (10%) e óbito 9/19 (47%). Análises de pacientes com LESJ ao diagnóstico de HP comparando-os com 76 pacientes controles com LESJ sem hemorragia pulmonar e com mesmo tempo de doença [3 (1-151) vs. 4 (1-151) meses, p=0,335], revelaram maior frequência de envolvimento constitucional (74% vs. 10%, p < 0,0001), serosite (63% vs. 6%, p < 0,0001) e sepse (53% vs. 9%, p < 0,0001) no grupo com HP. A mediana de escore de atividade de doença (SLEDAI-2K) foi significantemente maior em pacientes com LESJ com HP [18 (5-40) vs. 6 (0-44), p < 0,0001] assim como a mediana da dose de corticoide em uso em mg/Kg [1,4 (0,3-2) vs. 05 (0,03-3), p < 0,0001]. A frequência de plaquetopenia (53% vs. 12%, p < 0,0001), uso de metilprednisolona endovenosa (95% vs. 16%, p < 0,0001) e ciclofosfamida endovenosa (47% vs. 8%, p < 0,0001) também foi significantemente maior em pacientes com HP. Conclusões: Pacientes com LESJ apresentaram baixa prevalência de HP. Pacientes com LESJ e HP tiveram uma doença ativa grave, sistêmica, associada a envolvimento constitucional, serosite, plaquetopenia, sepse, dose maior de manutenção de corticoide, e principalmente tratada com pulsoterapia de corticoide e ciclofosfamida endovenosa 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: cSLE patients have shown low prevalence of DAH. Patients with both cSLE and DAH had serious, active systemic disease, associated with constitutional involvement, serositis, thrombocytopenia, sepsis, higher doses of maintenance corticotherapy, and mostly treated with corticoid pulse therapy and intravenous cyclophosphamide

Descreve‐se aqui uma paciente que apresentou uma sindrome de sobreposicao de poliarterite nodosa sistemica de inicio na infância (PANS‐i) e lupus eritematoso sistemico de inicio na infância (LES‐i). Uma menina de nove anos apresentou nodulos subcutâneos dolorosos nos pes, hipertensao arterial, hemiplegia a direita e fala disartrica. Ela foi hospitalizada em decorrencia de um acidente vascular encefalico e queda do pe esquerdo. A tomografia computadorizada do encefalo mostrou um acidente vascular encefalico isquemico. A angiografia por ressonância magnetica revelou estenose nas arterias cerebral media e carotida interna. A eletroneuromiografia identificou uma mononeuropatia do nervo tibial posterior a esquerda e ela atendeu aos criterios validados de PANS‐i. Ela foi tratada com pulsoterapia endovenosa com metilprednisolona seguido de prednisona (que foi progressivamente diminuida), seis meses de ciclofosfamida intravenosa e entao azatioprina por 19 meses. Aos 14 anos e nove meses, apresentou eritema malar, fotossensibilidade, edema nos membros inferiores e hipertensao arterial. A proteinuria era de 1,7 g/dia. Os anticorpos antinucleares (ANA) eram de 1/1.280 (padrao nuclear homogeneo) e os anticorpos anti‐dsDNA eram positivos. A biopsia renal mostrou glomerulonefrite proliferativa focal e membranosa. Portanto, ela preencheu aos criterios para LES do American College of Rheumatology e foi tratada com prednisona, hidroxicloroquina e micofenolato de mofetil. Em conclusao, descreve‐se uma possivel sindrome de sobreposicao de duas doencas autoimunes, em que a PANS‐i ocorreu cinco anos antes do diagnostico LES‐i.

Pyomyositis is a pyogenic infection of skeletal muscle that arises from hematogenous spread and usually presents with localized abscess. This muscle infection has been rarely reported in adult-onset systemic lupus erythematous and, to the best of our knowledge, has not been diagnosed in pediatric lupus population. Among our childhood-onset systemic lupus erythematous population, including 289 patients, one presented pyomyositis. This patient was diagnosed with childhood-onset systemic lupus erythematous at the age of 10 years-old. After six years, while being treated with prednisone, azathioprine and hydroxychloroquine, she was hospitalized due to a 30-day history of insidious pain in the left thigh and no apparent trauma or fever were reported. Her physical examination showed muscle tenderness and woody induration. Laboratory tests revealed anemia, increased acute phase reactants and normal muscle enzymes. Computer tomography of the left thigh showed collection on the middle third of the vastus intermedius, suggesting purulent stage of pyomyositis. Treatment with broad-spectrum antibiotic was initiated, leading to a complete clinical resolution. In conclusion, we described the first case of pyomyositis during childhood in pediatric lupus population. This report reinforces that the presence of localized muscle pain in immunocompromised patients, even without elevation of muscle enzymes, should raise the suspicion of pyomyositis. A prompt antibiotic therapy is strongly recommended.

Background Data of diffuse alveolar hemorrhage (DAH) in childhood-onset systemic lupus erythematosus (cSLE) patients are limited due to the small representation of this complication in previous case series or the focus on the comparison to adult SLE, precluding an accurate analysis of associated factors and outcomes in patients with and without this severe complication. Objectives To evaluate prevalence, clinical manifestations, laboratory abnormalities and treatment in a multicenter cohort study including 847 cSLE patients with and without diffuse DAH, as well as concomitant parameters of severity. Methods DAH was defined as the presence of at least three respiratory symptoms or signs associated with diffuse interstitial/alveolar infiltrates on chest x-ray or high-resolution computer tomography and sudden drop in hemoglobin levels with no other source of bleeding. Holm-Bonferroni correction for multiple comparisons was performed adjusting the significance level (p Results DAH was evidenced 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 Conclusions This is the largest study to evaluate DAH. This complication, although not a disease activity score descriptor, occurs in the context of significant moderate/severe cSLE flare. Importantly, we identified that this condition is associated with serious disease flare complicated by sepsis and with high mortality rate. Acknowledgements This study was supported by research grants from Conselho Nacional de Desenvolvimento Cientifico e Tecnolόgico (CNPq 301805/2013-0 to RMRP, 303752/2015-7 to MTT, 301479/2015-1 to CSM, 305068/2014-8 to EB and 303422/2015-7 to CAS), Federico Foundation (to EB, RMRP and CAS) and by Nucleo de Apoio a Pesquisa “Saude da Crianca e do Adolescente” da USP (NAP-CriAd) to CAS. Disclosure of Interest : None declared

We described herein a patient who presented an overlap syndrome of childhood-onset systemic polyarteritis nodosa and childhood-onset systemic lupus erythematosus. A 9-year-old girl presented tender subcutaneous nodules on feet, arterial hypertension, right hemiplegia and dysarthric speech. She was hospitalized due to stroke and left foot drop. Brain computer tomography showed ischemic stroke. Magnetic resonance angiography revealed stenosis in the middle cerebral and internal carotid arteries. Electroneuromyography identified a mononeuropathy of left posterior tibial nerve and she fulfilled the childhood-onset systemic polyarteritis nodosa validated criteria. She was treated with intravenous methylprednisolone pulse therapy followed by prednisone, that was progressively tapered, six months of intravenous cyclophosphamide and after that she received azathioprine for 19 months. At the age of 14 years and 9 months, she presented malar rash, photosensitivity, edema in lower limbs and arterial hypertension. The proteinuria was 1.7 g/day. Antinuclear antibodies (ANA) were 1/1280 (homogeneous nuclear pattern) and anti-dsDNA antibodies were positive. Renal biopsy showed focal proliferative and membranous glomerulonephritis. Therefore, she fulfilled the American College of Rheumatology classification criteria for systemic lupus erythematosus and she was treated with prednisone, hydroxychloroquine and mycophenolate mofetil. In conclusion, we described herein a possible overlap syndrome of two autoimmune diseases, where childhood-onset systemic polyarteritis nodosa occurred five years before the childhood-onset systemic lupus erythematosus diagnosis.