GLAUCIA VANESSA NOVAK
Projetos de Pesquisa
Unidades Organizacionais
LIM/36 - Laboratório de Pediatria Clínica, Hospital das Clínicas, Faculdade de Medicina
5 resultados
Resultados de Busca
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- Symptomatic polyautoimmunity at diagnosis of 1463 childhood-onset lupus: A Brazilian multicenter study(2018) SETOUE, Debora N.; PITTA, Ana C.; FIOROT, Fernanda J.; NASTRI, Mariana M.; NOVAK, Glaucia V.; MOLINARI, Beatriz C.; OLIVEIRA, Juliana C.; GORMEZANO, Natali W.; SAKAMOTO, Ana P.; TERRERI, Maria T.; PEREIRA, Rosa M.; SAAD-MAGALHAES, Claudia; SALLUM, Adriana M.; KOZU, Katia; FRAGA, Melissa M.; PIOTTO, Daniela P.; CLEMENTE, Gleice; MARINI, Roberto; GOMES, Hugo R.; RABELO-JUNIOR, Carlos N.; FELIX, Marta M.; RIBEIRO, Maria C.; ALMEIDA, Rozana G.; ASSAD, Ana P.; SACCHETTI, Silvana B.; BARROS, Leandra C.; BONFA, Eloisa; SILVA, Clovis A.Objective: To evaluate symptomatic polyautoimmunity (PA) at childhood-onset systemic lupus erythematosus (cSLE) diagnosis, and its association with demographic data, disease activity, clinical manifestations and laboratorial abnormalities in a large Brazilian cSLE population. Methods: A multicenter retrospective study was performed in 1463 cSLE(ACR criteria) patients from 27 Pediatric Rheumatology services. Symptomatic PA was defined according to the presence of more than one concomitant autoimmune disease(AD) and symptomatic multiple autoimmune syndrome(MAS) was defined as three or more AD. An investigator meeting was held to define the protocol. Demographic data, SLICC classification criteria and SLEDAI-2K were evaluated. Results: At cSLE diagnosis symptomatic PA was observed in 144/1463(9.8%) and symptomatic MAS occurred in solely 10/1463(0.7%). In the former group the more frequently observed associated AD were Hashimoto thyroiditis n = 42/144(29%), antiphospholipid syndrome n = 42/144(29%), autoimmune hepatitis n = 26/144(18%) and type 1 diabetes mellitus n = 23/144(15.9%). Further comparisons between cSLE patients with and without PA showed a higher median age(p = 0.016) and lower mean SLICC criteria (p = 0.039) in those with PA. Additionally, these cSLE patients had less renal involvement(35% vs. 44%, p = 0.038) and red blood cell cast(6% vs. 12%, p = 0.042) and more antiphospholipid antibodies(29% vs. 15%, p < 0.0001). Conclusions: Approximately 10% of cSLE had symptomatic PA at diagnosis, particularly endocrine autoimmune disorders and antiphospholipid syndrome. Lupus was characterized by a mild disease onset and MAS was infrequently evidenced. Further studies are necessary to determine if this subgroup of cSLE patients have a distinct genetic background with a less severe disease and a better long-term outcome.
- Disease presentation of 1312 childhood-onset systemic lupus erythematosus: influence of ethnicity(2019) FIOROT, Fernanda J.; ISLABAO, Aline G.; PEREIRA, Rosa M.; TERRERI, Maria T.; SAAD-MAGALHAES, Claudia; V, Glaucia Novak; MOLINARI, Beatriz C.; SAKAMOTO, Ana P.; AIKAWA, Nadia E.; CAMPOS, Lucia M.; PERACCHI, Octavio A.; APPENZELLER, Simone; FERRIANI, Virginia P.; SILVA, Marco F.; FONSECA, Adriana R.; SZTAJNBOK, Flavio R.; PAIM, Luciana B.; FRAGA, Melissa M.; OKUDA, Eunice M.; BICA, Blanca E.; SENA, Evaldo G.; MORAES, Ana J.; ROLIM, Ana M.; SPELLING, Paulo F.; SCHEIBEL, Iloite M.; CAVALCANTI, Andre S.; MATOS, Erica N.; ROBAZZI, Teresa C.; GUIMARAES, Luciano J.; SANTOS, Flavia P.; RAMOS, Valeria C.; CARNEIRO-SAMPAIO, Magda; BONFA, Eloisa; SILVA, Clovis A.Objective To evaluate the influence of ethnicity in presentation of childhood-onset systemic lupus erythematosus (cSLE) patients. Methods This multicenter study included cSLE patients (American College of Rheumatology criteria) followed in 27 Pediatric Rheumatology services of Brazil. Ethnicities were classified in four groups according to the parents' and all four grandparents' self-reported ethnicity. The statistical analysis was performed using the Bonferroni's correction (p < 0.0027). Results According to ethnic groups, 1537 cSLE patients were classified in Caucasian (n = 786), African-Latin American (n = 526), Asian (n = 8), and others/unknown (n = 217). Comparisons between 1312 African-Latin American and Caucasian revealed similar median age at cSLE diagnosis [12.2(2.6-18) vs. 12.1(0.3-18) years, p = 0.234], time interval to diagnosis [0.25(0-12) vs. 0.3(0-10) years, p = 0.034], and SLEDAI-2K score [14(0-55) vs. 14(0-63), p = 0.781] in both groups. The mean number of diagnostic criteria according to SLICC (6.47 +/- 1.911 vs. 5.81 +/- 1.631, p < 0.0001) and frequencies of maculopapular lupus rash (8% vs. 3%, p < 0.0001), palate oral ulcers (17% vs. 11%, p = 0.001), tongue oral ulcers (4% vs. 1%, p = 0.001), and nonscarring alopecia (29% vs. 16%, p < 0.0001) were significantly higher in African-Latin American, whereas malar rash (45% vs. 58%, p < 0.0001) was more frequent in Caucasian. The presence of anti-phospholipid antibody (23% vs. 12%, p < 0.0001), low complement levels (58% vs. 41%, p < 0.0001), and isolated direct Coombs test (10% vs. 5%, p = 0.001) was also significantly higher in the former group. Conclusions Our study demonstrated that disease presentation severity of African-Latin American cSLE patients is comparable with Caucasian. Mucocutaneous manifestations and autoantibodies profile were the only distinctive features of the former group. The unique mixed background of Brazilian patients probably minimized race diversity spectrum of these patients.
- Childhood-onset systemic lupus erythematosus-related antiphospholipid syndrome: A multicenter study with 1519 patients(2020) ISLABA, Aline G.; MOTA, Licia M. H.; RIBEIRO, Maria Custodia M.; ARABI, Tamima M.; CIVIDATTI, Georgiana N.; QUEIROZ, Ligia B.; ANDRADE, Danieli C.; SAKAMOTO, Ana P.; TRINDADE, Vitor C.; NOVAK, Glaucia V.; MOLINARI, Beatriz C.; CAMPOS, Lucia M.; AIKAWA, Nadia E.; PEREIRA, Rosa M. R.; TERRERI, Maria T.; MAGALHA, Claudia S.; MARINI, Roberto; GOMES, Hugo R.; SILVA, Marco F.; OLIVEIRA, Sheila K.; SZTAJNBOK, Flavio R.; SACCHETTI, Silvana B.; BICA, Blanca E.; SENA, Evaldo G.; MORAES, Ana P.; SANTOS, Maria C.; ROBAZZI, Teresa C.; SPELLING, Paulo F.; SCHEIBEL, Iloite M.; CAVALCANTI, Andre S.; NAKA, Erica N.; GUIMARAES, Luciano J.; SANTOS, Flavia P.; SAMPAIO, Magda C.; BONFA, Eloisa; SILVA, Clovis A.Objective: To assess childhood-onset systemic lupus erythematosus-related antiphospholipid syndrome(cSLE-APS) in a large Brazilian population. Methods: A retrospective observational cohort study was carried-out in 27 Pediatric Rheumatology university centers, including 1519 cSLE patients. Results: cSLE-APS was observed in 67/1519 (4%) and was diagnosed at disease onset in 39/67 (58%). The median disease duration was 4.9 (0-17) years. Thrombosis recurrences were evidenced in 18/67 (27%) cSLE-APS patients. The most frequent thrombosis sites in cSLE-APS patients were: venous thrombosis in 40/67 (60%), especially deep vein thrombosis in 29/40 (72%); arterial thrombosis in 35/67 (52%), particularly stroke; small vessels thrombosis in 9/67 (13%) and mixed thrombosis in 3/67 (4%). Pregnancy morbidity was observed in 1/67 (1%). Non-thrombotic manifestation associated to cSLE-APS occurred in 21/67 (31%), mainly livedo reticularis in 14/67 (21%), valvar thickening in 4/67 (6%) and valvar vegetations not related to infections in 2/67 (3%). None of them had catastrophic APS. Further analysis demonstrated that the median of SLICC/ACR-DI [1 (0-5) vs. 0(0-7),p < 0.0001] was significantly higher in cSLE-APS patients compared to cSLE without APS. The frequencies of cerebrovascular disease (40% vs. 1%,p < 0.0001), polyneuropathy (9% vs. 1%,p < 0.0001), SLICC/ACR-DI >= 1 (57% vs. 27%, p < 0.0001) and intravenous cyclophosphamide use (59% vs. 37%, p < 0.0001) were significantly higher in the former group. Conclusions: Our large multicenter study demonstrated that cSLE-APS was a rare condition, occurring during disease course with a high accrual damage. Central and peripheral neuropsychiatric involvements were distinctive features of this autoimmune thrombosis.
- Chronic kidney disease in patients with childhood-onset systemic lupus erythematosus(2023) SAKAMOTO, Ana P.; SILVA, Clovis A.; ISLABAO, Aline G.; V, Glaucia Novak; MOLINARI, Beatriz; NOGUEIRA, Paulo K.; PEREIRA, Rosa M. R.; SAAD-MAGALHAES, Claudia; CLEMENTE, Gleice; PIOTTO, Daniela P.; AIKAWA, Nadia E.; PITTA, Ana C.; TRINDADE, Vitor C.; APPENZELLER, Simone; CARVALHO, Luciana M.; RABELO-JUNIOR, Carlos N.; FONSECA, Adriana R.; SZTAJNBOK, Flavio R.; SANTOS, Maria C.; BICA, Blanca E.; SENA, Evaldo G.; MORAES, Ana J.; FRAGA, Melissa M.; ROBAZZI, Teresa C.; SPELLING, Paulo F.; SCHEIBEL, Iloite M.; CAVALCANTI, Andre S.; MATOS, Erica N.; GUIMARAES, Luciano J.; SANTOS, Flavia P.; MOTA, Licia M. H.; BONFA, Eloisa; TERRERI, Maria T.Background Lupus nephritis (LN) is a frequent manifestation of childhood-onset systemic lupus erythematosus (cSLE) with a potential risk for kidney failure and poor outcomes. This study aimed to evaluate stages III, IV, and V of chronic kidney disease (CKD) and investigate risk factors for CKD in cSLE patients. Methods We performed a nationwide observational cohort study in 27 pediatric rheumatology centers, including medical charts of 1528 cSLE patients. Data were collected at cSLE diagnosis, during follow-up, and at last visit or death, between September 2016 and May 2019. Results Of 1077 patients with LN, 59 (5.4%) presented with CKD, 36/59 (61%) needed dialysis, and 7/59 (11.8%) were submitted for kidney transplantation. After Bonferroni's correction for multiple comparisons (p < 0.0013), determinants associated with CKD were higher age at last visit, urinary biomarker abnormalities, neuropsychiatric involvement, higher scores of disease activity at last visit and damage index, and more frequent use of methylprednisolone, cyclosporine, cyclophosphamide, and rituximab. In the regression model analysis, arterial hypertension (HR = 15.42, 95% CI = 6.12-38.83, p < 0.001) and biopsy-proven proliferative nephritis (HR = 2.83, 95%CI = 1.70-4.72, p <= 0.001) increased the risk of CKD, while children using antimalarials had 71.0% lower CKD risk ((1.00-0.29) x 100%) than children not using them. The Kaplan-Meier comparison showed lower survival in cSLE patients with biopsy-proven proliferative nephritis (p = 0.02) and CKD (p <= 0.001). Conclusions A small number of patients manifested CKD; however, frequencies of dialysis and kidney transplantation were relevant. This study reveals that patients with cSLE with hypertension, proliferative nephritis, and absence of use of antimalarials exhibited higher hazard rates of progression to CKD.
- Characteristics of 1555 childhood-onset lupus in three groups based on distinct time intervals to disease diagnosis: a Brazilian multicenter study(2018) V, G. Novak; MOLINARI, B. C.; FERREIRA, J. C.; SAKAMOTO, A. P.; TERRERI, M. T.; PEREIRA, R. M. R.; SAAD-MAGALHAES, C.; AIKAWA, N. E.; CAMPOS, L. M.; LEN, C. A.; APPENZELLER, S.; FERRIANI, V. P.; SILVA, M. F.; OLIVEIRA, S. K.; ISLABDO, A. G.; SZTAJNBOK, F. R.; PAIM, L. B.; BARBOSA, C. M.; SANTOS, M. C.; BICA, B. E.; SENA, E. G.; MORAES, A. J.; ROLIM, A. M.; SPELLING, P. F.; SCHEIBEL, I. M.; CAVALCANTI, A. S.; MATOS, E. N.; ROBAZZI, T. C.; GUIMARACS, L. J.; SANTOS, F. P.; SILVA, C. T.; BONFA, E.; SILVA, C. A.Objective The objective of this study was to compare demographic data, clinical/laboratorial features and disease activity at diagnosis in three different groups with distinct time intervals between onset of signs/symptoms and disease diagnosis. Methods A multicenter study was performed in 1555 childhood-onset systemic lupus erythematosus (American College of Rheumatology criteria) patients from 27 pediatric rheumatology services. Patients were divided into three childhood-onset systemic lupus erythematosus groups: A: short time interval to diagnosis (<1 month); B: intermediate time interval (1 and <3 months); and C: long time interval (3 months). An investigator meeting was held to define the protocol. Demographic data, SLICC classification criteria and SLEDAI-2K were evaluated. Results The number of patients in each group was: A=60 (4%); B=522 (33.5%); and C=973 (62.5%). The median age at diagnosis (11.1 (4.2-17) vs. 12 (1.9-17.7) vs. 12.5 (3-18) years, P=0.025) was significantly lower in group A compared with groups B and C. The median number of diagnostic criteria according to SLICC (7 (4-12) vs. 6 (4-13) vs. 6 (4-12), P<0.0001) and SLEDAI-2K (18 (6-57) vs. 16 (2-63) vs. 13 (1-49), P<0.0001) were significantly higher in group A than the other two groups. The frequency of oral ulcers in the palate (25% vs. 15% vs. 11%, P=0.003), pleuritis (25% vs. 24% vs. 14%, P<0.0001), nephritis (52% vs. 47% vs. 40%, P=0.009), neuropsychiatric manifestations (22% vs. 13% vs. 10%, P=0.008), thrombocytopenia (32% vs. 18% vs. 19%, P=0.037), leucopenia/lymphopenia (65% vs. 46% vs. 40%, P<0.0001) and anti-dsDNA antibodies (79% vs. 66% vs. 61%, P=0.01) were significantly higher in group A compared with the other groups. In contrast, group C had a less severe disease characterized by higher frequencies of synovitis (61% vs. 66% vs. 71%, P=0.032) and lower frequencies of serositis (37% vs. 33% vs. 25%, P=0.002), proteinuria >500mg/day (48% vs. 45% vs. 36%, P=0.002) and low complement levels (81% vs. 81% vs. 71%, P<0.0001) compared with groups A or B. Conclusions Our large Brazilian multicenter study demonstrated that for most childhood-onset systemic lupus erythematosus patients, diagnosis is delayed probably due to mild disease onset. Conversely, the minority has a very short time interval to diagnosis and a presentation with a more severe and active multisystemic condition.