Abstract Introduction Seasonal influenza A (H3N2) virus is an important cause of morbidity and mortality in the last 50 years in population that is greater than the impact of H1N1. Data assessing immunogenicity and safety of this virus component in juvenile systemic lupus erythematosus (JSLE) is lacking in the literature. Objective To evaluate short-term immunogenicity and safety of influenza A/Singapore (H3N2) vaccine in JSLE. Methods 24 consecutive JSLE patients and 29 healthy controls (HC) were vaccinated with influenza A/Singapore/ INFIMH-16-0019/2016(H3N2)-like virus. Influenza A (H3N2) seroprotection (SP), seroconversion (SC), geometric mean titers (GMT), factor increase in GMT (FI-GMT) titers were assessed before and 4 weeks post-vaccination. Disease activity, therapies and adverse events (AE) were also evaluated. Results JSLE patients and controls were comparable in current age [14.5 (10.1–18.3) vs. 14 (9–18.4) years, p = 0.448] and female sex [21 (87.5%) vs. 19 (65.5%), p = 0.108]. Before vaccination, JSLE and HC had comparable SP rates [22 (91.7%) vs. 25 (86.2%), p = 0.678] and GMT titers [102.3 (95% CI 75.0–139.4) vs. 109.6 (95% CI 68.2–176.2), p = 0.231]. At D30, JSLE and HC had similar immune response, since no differences were observed in SP [24 (100%) vs. 28 (96.6%), p = 1.000)], SC [4 (16.7%) vs. 9 (31.0%), p = 0.338), GMT [162.3 (132.9–198.3) vs. 208.1 (150.5–287.8), p = 0.143] and factor increase in GMT [1.6 (1.2–2.1) vs. 1.9 (1.4–2.5), p = 0.574]. SLEDAI-2K scores [2 (0–17) vs. 2 (0–17), p = 0.765] and therapies remained stable throughout the study. Further analysis of possible factors influencing vaccine immune response among JSLE patients demonstrated similar GMT between patients with SLEDAI < 4 compared to SLEDAI ≥ 4 ( p = 0.713), as well as between patients with and without current use of prednisone ( p = 0.420), azathioprine ( p = 1.0), mycophenolate mofetil ( p = 0.185), and methotrexate ( p = 0.095). No serious AE were reported in both groups and most of them were asymptomatic (58.3% vs. 44.8%, p = 0.958). Local and systemic AE were alike in both groups ( p > 0.05). Conclusion This is the first study that identified adequate immune protection against H3N2-influenza strain with additional vaccine-induced increment of immune response and an adequate safety profile in JSLE. ( www.clinicaltrials.gov , NCT03540823).

INTRODUÇÃO/OBJETIVO: Avaliar a frequência do comprometimento da habilidade verbal e possíveis fatores associados em pacientes portadores de Lúpus Eritematoso Sistêmico Juvenil (LESJ). PACIENTES E MÉTODOS: Estudo transversal de 36 crianças e adolescentes com LESJ, de um grupo de 57 pacientes, do Ambulatório de Reumatologia do Departamento de Pediatria e Clínica Médica da Santa Casa de Misericórdia de São Paulo. Por ocasião do diagnóstico e do estudo, foram analisados aspectos epidemiológicos, clínicos, socioeconômicos e de escolaridade. Os pacientes foram submetidos a testes cognitivos e exames laboratoriais, e foram avaliadas medidas de atividade da doença (SLEDAI) e do dano cumulativo (SLICC-DI) e o tratamento com corticoesteroide. Os pacientes foram submetidos a testes cognitivos (escalas Weschler de inteligência: WISC III e WAISS III), e os resultados foram avaliados de acordo com os aspectos epidemiológicos, clínicos, laboratoriais e terapêuticos. RESULTADOS: A média de idade ao diagnóstico foi de 11,2 ± 2 anos, a idade na época do estudo de 15,4 ± 4,7 anos, com 89% do sexo feminino. Houve predomínio de pacientes da classe socioeconômica C (61,1%). O comprometimento cognitivo detectado nesses pacientes foi frequente (58,3%), sendo o comprometimento da habilidade verbal um dos domínios cognitivos mais constantes. Encontrou-se associação do comprometimento da habilidade verbal com baixa condição socioeconômica e dano cumulativo (P < 0,05), mas não com atividade da doença, presença de autoanticorpos e dose de corticoesteroide (P > 0,05). CONCLUSÕES: Alteração da habilidade verbal é frequente no LESJ e está associada à condição socioeconômica e ao dano cumulativo, devendo ser suspeitada e investigada, principalmente por se tratar de pacientes pediátricos, para que não haja comprometimento da qualidade de vida na fase adulta. Como não está relacionado à atividade da doença ou à presença de autoanticorpos, deve ser sempre avaliado na presença ou não desses fatores. Da mesma forma, deve-se avaliar independentemente das doses de corticoesteroide por não haver associação.

INTRODUCTION/OBJECTIVE: Evaluate the frequency of verbal ability impairment and associated factors in patients with juvenile systemic lupus erythematosus (JSLE). PATIENTS AND METHODS: Cross sectional study of 36 children and adolescents with JSLE of a group of 57 patients at the Clinic of Rheumatology, Department of Pediatrics and Medical Clinic of Santa Casa de Misericórdia de São Paulo. At the time of diagnosis and study, we analyzed the following epidemiological features: clinical, socioeconomic, and educational level. Patients underwent cognitive and laboratory tests and we assessed disease activity (SLEDAI), cumulative damage (SLICC-DI), and treatment with corticosteroids. The patients underwent cognitive tests (Wechsler Intelligence Scales: WISC III and Waiss III), and the results were evaluated according to epidemiological, clinical, laboratory and treatment features. RESULTS: The mean age at diagnosis was 11.2 ± 2 years and at the time of the study the mean age was 15.4 ± 4.7 years. There was predominance of women (89%) and of socioeconomic class C patients (61.1%). The cognitive impairment found in these patients was frequent (58.3%), affecting more often the verbal ability. We found association of verbal ability impairment with low socioeconomic status and cumulative damage (P < 0.05), but not with disease activity, presence of autoantibodies, and dose of corticosteroids (P > 0.05). CONCLUSIONS: Change in verbal ability is frequent in JSLE and is associated with socioeconomic status and cumulative damage, and should be suspected and investigated, particularly in pediatric patients to avoid quality of life impairment in adulthood. As it is not related with disease activity or presence of autoantibodies, it should always be assessed in the presence or absence of these factors. Likewise, the doses of corticosteroids should be independently evaluated.

INTRODUÇÃO: O Lúpus Eritematoso Sistêmico (LES) se caracteriza por períodos de exacerbação e remissão clínica que frequentemente são acompanhados por alterações nos níveis séricos de anticorpos específicos, como o anti-dsDNA, que está presente em 40% dos casos, associado principalmente à atividade renal. Recentemente houve a descrição de duas subpopulações de anticorpos antilipoproteína lipase (anti-LPL) no LES: uma com e a outra sem atividade anti-dsDNA. A possível relação desse último grupo de anticorpos com a atividade inflamatória de doença ainda não foi analisada no LES. OBJETIVOS: Avaliar longitudinalmente a associação dos níveis séricos dos anticorpos anti-LPL com atividade do LES em pacientes com anti-dsDNA persistentemente negativo. PACIENTES E MÉTODOS: Cinco pacientes com LES com anti-dsDNA persistentemente negativo mensurado por ELISA e por imunofluorescência indireta em crithidia luciliae e altos títulos de anti-LPL por ELISA (> 5 desvios-padrão (DP) da média de 20 controles normais) foram selecionados e acompanhados longitudinalmente durante um período mínimo de dois anos. RESULTADOS: Caso 1: Homem, 24 anos com LES desde 2001 apresentou hemorragia alveolar, proteinúria, hipertensão arterial sistêmica, eritema malar, aftas, artrite, FAN+, com SLEDAI (systemic lupus erythematosus disease activity index) = 16 e anti-LPL = 144UA. Tratado com pulso de metilprednisolona e prednisona com melhora clínica e SLEDAI = 0 e redução do anti-LPL (109UA). Nova atividade com acometimento renal em abril de 2002, SLEDAI = 10 e aumento de anti-LPL (150UA). Iniciada pulsoterapia de ciclofosfamida e metilprednisolona com boa resposta, SLEDAI = 0 e diminuição de anti-LPL (77UA) até a sua total negativação acompanhando a remissão do quadro no ano de 2003. Caso 2: Mulher, 32 anos, com LES desde 1997. Em setembro de 2001 iniciou vasculite cutânea, febre e rash, SLEDAI = 10, anti-LPL = 80UA. Em janeiro de 2002, teve atividade renal e HAS, SLEDAI = 8 e anti-LPL = 25UA, mas com a introdução de CE e ciclofosfamida evoluiu com melhora importante. Em 2003, assintomática, apresentava SLEDAI = 2 e anti-LPL = 12UA. Caso 3: Homem, 39 anos, com LES desde 1997. Estável em uso de cloroquina, sem atividade durante 3 anos, SLEDAI = 0 em todas as ocasiões e sem variação dos títulos de anti-LPL no período do estudo: 85UA (2001),100UA (2002) e 86UA (2003). Caso 4: Mulher, 58 anos com LES desde 1996. Remissão do LES desde agosto de 2001 e com SLEDAI = 0 no período do estudo. Títulos de anti-LPL sem flutuações significativas: 71UA (2001), 42UA (2002) e 61UA (2003). Caso 5: Mulher, 55 anos com LES desde 1989. Estável desde 2000 em uso de cloroquina, SLEDAI = 0, e títulos de anti-LPL também sem variações: 71UA (1999), 92UA (2001), 71UA (2002) e 90UA (2003). CONCLUSÃO: A avaliação longitudinal dos pacientes selecionados mostrou-se estar associada à flutuação dos títulos de anti-LPL com o SLEDAI, sugerindo ser essa variação um novo marcador de atividade do LES, na ausência de anticorpos anti-dsDNA.

INTRODUCTION: Systemic lupus erythematosus (SLE) is characterized by periods of clinical flares and remission that are followed by alterations of sera specific autoantibodies such as anti-dsDNA, present in 40% of the cases and strongly associated with renal involvement. Recently, there was a description of two subpopulations of anti-lipoprotein lipase antibodies (anti-LPL) in SLE: with and without anti-dsDNA activity. A possible relationship between these antibodies with inflammatory activity of SLE was not analyzed. OBJECTIVES: To evaluate longitudinally the association between anti-LPL with lupus activity in patients persistently negatives for anti-dsDNA antibodies. PATIENTS AND METHODS: Five SLE patients with persistently negative anti-dsDNA measured by ELISA and indirect immunofluorescence using crithidia luciliae and high titers of anti-LPL by ELISA (> 5 SD) were selected and followed for at least 2 years. RESULTS: Case 1: A 24-year-old male with SLE since 2001, presented with alveolar hemorrhage, proteinuria, systemic hypertension, malar rash, oral ulcers, polyarthritis, positive ANA, SLEDAI=16 and anti-LPL=144U. He was treated with intravenous (IV) methylprednisolone followed by prednisone and had an excellent response. SLEDAI=0, anti-LPL decreased to 109U. New renal flare in April 2002, SLEDAI=10 and a new increment of anti-LPL (150U). IV Cyclophosphamide and methylprednisolone were started and he achieved remission, SLEDAI=0 and a decrease of anti-LPL (77U) until become negative in 2003. Case 2: A 32-year-old female had SLE since 1997. In September 2001 began cutaneous vasculitis, fever and rash, SLEDAI=10, anti-LPL=80U. In January 2002, she had renal involvement and systemic hypertension, SLEDAI=8 and anti-LPL= 25U. She received corticosteroid and cyclophosphamide and improved. In 2003, she was asymptomatic, SLEDAI=2 and anti-LPL=12U. Case 3: A 39-year-old male has SLE since 1997. He was stable, under chloroquine use, without disease activity for 3 years, SLEDAI=0 in all period studied and no fluctuation of anti-LPL titers: 85U (2001),100U (2002) e 86U (2003). Case 4: A 58-year-old female had SLE since 1996. She was in remission since August 2001 with a SLEDAI=0 during all this study. Anti-LPL titers did not significantly change: 71U (2001), 42U (2002) e 61U (2003). Case 5: A 55-year-old female had SLE since 1989. She was stable since 2000 using chloroquine, SLEDAI=0, and anti-LPL titers without variations: 71U (1999), 92U (2001), 71U (2002) e 90U (2003). CONCLUSION: The longitudinal evaluation of the selected patients showed a positive correlation between the fluctuations of the titers of anti-LPL with the SLEDAI score. These findings suggest that this variation may be a new marker for lupus activity in patients without anti-dsDNA antibodies.