Henoch-Schönlein purpura nephritis: initial risk factors and outcomes in a tertiary center in Latin America

Objetivo: Avaliar a prevalência, fatores de risco iniciais e a evolução da nefrite da púrpura de Henoch-Schönlein (NPHS) em crianças e adolescentes em um serviço terciário da América Latina. Métodos: O prontuário de 296 pacientes, que preencheram o critério do EULAR/PRINTO/PRES para PHS, foram retrospectivamente analisados. Dados referentes as características demográficas, manifestações clínicas, exames laboratoriais e tratamentos foram obtidos nos primeiros 3 meses após o diagnóstico. Os pacientes foram seguidos em um serviço terciário da América Latina e foram divididos em dois grupos: com e sem nefrite. Proteinúria não-nefrótica persistente, proteinúria nefrótica e insuficiência renal aguda e crônica foram avaliadas em até 1, 5, 10 e 15 anos após o diagnóstico. Resultados: Nefrite foi observada em 139/296 (47%) nos primeiros 3 meses de doença. A mediana da idade ao diagnóstico foi significantemente maior em pacientes com acometimento renal em comparação aos sem nefrite [6,6 (1,5-17,7) vs. 5,7 (0,9-13,5) anos, p=0,022]. As frequências de púrpura persistente (31% vs. 10%, p < 0,0001), dor abdominal recorrente (16% vs. 7%, p=0,011), sangramento gastrointestinal (GI) (25% vs. 10%, p < 0,0001) e uso de corticosteroides (54% vs. 41%, p=0,023) foram significantemente maiores nos pacientes com nefrite. Na análise multivariada, a regressão logística demonstrou que variáveis independentes preditoras de NPHS foram púrpura persistente (OR=3,601; IC 95% 1,605-8,079; p=0,002) e sangramento GI (OR=2,991; IC 95% 1,245-7,183; p=0,014). Dos 139 pacientes com nefrite inicial, proteinúria não-nefrótica persistente, proteinúria nefrótica e insuficiência renal ocorreram respectivamente em até 1 ano de seguimento [46/88 (52%), 1/88 (1%) e 2/88 (2%)], 5 anos [25/47 (53%), 1/47 (2%) e 1/47 (2%)], 10 anos [9/20 (45%), 1/20 (5%) e 1/20 (5%)] e em até 15 anos [1/6 (17%), 0/6 (0%) e 0/6 (0%)]. Dos 157 pacientes sem NPHS inicial, 118/157 (75%) se mantiveram em acompanhamento por pelo menos um ano e 33/118 (28%) apresentaram envolvimento renal (proteinúria não-nefrótica persistente e/ou hematúria) em algum momento do seguimento. Conclusões: O presente estudo verificou NPHS inicial em aproximadamente metade dos pacientes e foi associada com púrpura persistente e sangramento GI. Nefrite tardia foi evidenciada em cerca de um terço dos pacientes que não tiveram NPHS no início da doença, sem manifestações renais gravesObjective: To evaluate prevalence, initial risk factors and outcome of Henoch-Schönlein purpura nephritis (HSPN) in children and adolescents in a tertiary center in Latin America. Methods: 296 patients (validated EULAR/PRINTO/PRES HSP criteria) were assessed by demographic data, clinical/laboratorial involvements and treatments in the first three months after diagnosis. They were followed-up in a Latin American tertiary center and were divided in two groups: with and without nephritis. Persistent non- nephrotic proteinuria, nephrotic proteinuria and acute/chronic kidney injury were also systematically evaluated up to 1, 5, 10 and 15 years after diagnosis. Results: HSPN was evidenced in 139/296 (47%) in the first 3 months. The median age at diagnosis was significantly higher in HSPN patients compared without renal involvement [6.6 (1.5-17.7) vs. 5.7 (0.9-13.5) years, p=0.022]. The frequencies of persistent purpura (31% vs. 10%, p < 0.0001), recurrent abdominal pain (16% vs. 7%, p=0.011), gastrointestinal (GI) bleeding (25% vs. 10%, p < 0.0001) and corticosteroid use (54% vs. 41%, p=0.023) were significantly higher in the former group. Logistic regression demonstrated that the independent variables associated with HSNP were persistent purpura (OR=3.601; 95% CI 1.605-8.079; p=0.002) and GI bleeding (OR=2.991; 95% CI 1.245-7.183; p=0.014). Of 139 patients with initial HSPN, persistent non-nephrotic proteinuria, nephrotic proteinuria and renal insufficiency were respectively observed in up to 1 year of follow-up [46/88 (52%), 1/88 (1%) and 2/88 (2%)], 5 years [25/47 (53%), 1/47 (2%) and 1/47 (2%)], 10 years [9/20 (45%), 1/20 (5%) and 1/20 (5%)] and in up to 15 years [1/6 (17%), 0/6 (0%) and 0/6 (0%)]. Further analysis of 157 patients without initial HSPN, 118/157 (75%) were followed-up for at least 1 year and 33/118 (28%) showed renal involvement (persistent non-nephrotic proteinuria and/or hematuria) at any time in the follow-up. Conclusions: The present study observed initial HSPN in almost one half of patients and was associated with persistent purpura and GI bleeding. Almost one third of patients had nephritis only evidenced during follow-up without severe renal manifestation

Safety and immunogenicity of influenza A(H3N2) component vaccine in juvenile systemic lupus erythematosus

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  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)