JULIANA CAIRES DE OLIVEIRA ACHILI FERREIRA
Projetos de Pesquisa
Unidades Organizacionais
Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina
14 resultados
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Agora exibindo 1 - 10 de 14
- Outcomes of 847 childhood-onset systemic lupus erythematosus patients in three age groups(2017) LOPES, S. R. M.; GORMEZANO, N. W. S.; GOMES, R. C.; AIKAWA, N. E.; PEREIRA, R. M. R.; TERRERI, M. T.; MAGALHAES, C. S.; FERREIRA, J. C.; OKUDA, E. M.; SAKAMOTO, A. P.; SALLUM, A. M. E.; APPENZELLER, S.; FERRIANI, V. P. L.; BARBOSA, C. M.; LOTUFO, S.; JESUS, A. A.; ANDRADE, L. E. C.; CAMPOS, L. M. A.; BONFA, E.; SILVA, C. A.Objective The objective of this study was to assess outcomes of childhood systemic lupus erythematosus (cSLE) in three different age groups evaluated at last visit: group A early-onset disease (<6 years), group B school age (6 and <12 years) and group C adolescent (12 and <18 years). Methods An observational cohort study was performed in ten pediatric rheumatology centers, including 847 cSLE patients. Results Group A had 39 (4%), B 395 (47%) and C 413 (49%). Median disease duration was significantly higher in group A compared to groups B and C (8.3 (0.1-23.4) vs 6.2 (0-17) vs 3.3 (0-14.6) years, p<0.0001). The median Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI) (0 (0-9) vs 0 (0-6) vs 0 (0-7), p=0.065) was comparable in the three groups. Further analysis of organ/system damage revealed that frequencies of neuropsychiatric (21% vs 10% vs 7%, p=0.007), skin (10% vs 1% vs 3%, p=0.002) and peripheral vascular involvements (5% vs 3% vs 0.3%, p=0.008) were more often observed in group A compared to groups B and C. Frequencies of severe cumulative lupus manifestations such as nephritis, thrombocytopenia, and autoimmune hemolytic anemia were similar in all groups (p>0.05). Mortality rate was significantly higher in group A compared to groups B and C (15% vs 10% vs 6%, p=0.028). Out of 69 deaths, 33/69 (48%) occurred within the first two years after diagnosis. Infections accounted for 54/69 (78%) of the deaths and 38/54 (70%) had concomitant disease activity. Conclusions This large multicenter study provided evidence that early-onset cSLE group had distinct outcomes. This group was characterized by higher mortality rate and neuropsychiatric/vascular/skin organ damage in spite of comparable frequencies of severe cumulative lupus manifestations. We also identified that overall death in cSLE patients was an early event mainly attributed to infection associated with disease activity.
- Anti-ribosomal P antibody: a multicenter study in childhood-onset systemic lupus erythematosus patients(2017) VALOES, C. C. M.; MOLINARI, B. C.; PITTA, A. C. G.; GORMEZANO, N. W. S.; FARHAT, S. C. L.; KOZU, K.; SALLUM, A. M. E.; APPENZELLER, S.; SAKAMOTO, A. P.; TERRERI, M. T.; PEREIRA, R. M. R.; MAGALHAES, C. S.; FERREIRA, J. C. O. A.; BARBOSA, C. M.; GOMES, F. H.; BONFA, E.; SILVA, C. A.Objectives: Anti-ribosomal P protein (anti-P) autoantibodies are highly specific for systemic lupus erythematosus (SLE). However, the evaluation of this autoantibody in childhood-onset SLE (cSLE) populations has been limited to a few small series, hampering the interpretation of the clinical and laboratorial associations. Therefore, the objective of this multicenter cohort study was to evaluate demographic, clinical/laboratorial features, and disease damage score in cSLE patients with and without the presence of anti-P antibody. Methods: This was a retrospective multicenter study performed in 10 pediatric rheumatology services of Sao Paulo state, Brazil. Anti-P antibodies were measured by ELISA in 228 cSLE patients. Results: Anti-P antibodies were observed in 61/228 (27%) cSLE patients. Frequencies of cumulative lymphadenopathy (29% vs. 15%, p = 0.014), acute confusional state (13% vs. 5%, p = 0.041), mood disorder (18% vs. 8%, p = 0.041), autoimmune hemolytic anemia (34% vs. 15%, p = 0.001), as well as presence of anti-Sm (67% vs. 40%, p = 0.001), anti-RNP (39% vs. 21%, p = 0.012) and anti-Ro/SSA antibodies (43% vs. 25%, p = 0.016) were significantly higher in cSLE patients with anti-P antibodies compared to those without these autoantibodies. A multiple regression model revealed that anti-P antibodies were associated with autoimmune hemolytic anemia (odds ratio (OR) = 2.758, 95% confidence interval (CI): 1.304-5.833, p = 0.008) and anti-Sm antibody (OR = 2.719, 95% CI: 1.365-5.418, p = 0.004). The SLICC/ACR damage index was comparable in patients with and without anti-P antibodies (p = 0.780). Conclusions: The novel association of anti-P antibodies and autoimmune hemolytic anemia was evidenced in cSLE patients and further studies are necessary to determine if anti-P titers may vary with this hematological manifestation.
- Echocardiographic study of juvenile dermatomyositis patients: new insights from speckle-tracking-derived strain(2021) DINIZ, Maria de Fatima Rodrigues; KOZU, Katia Tomie; ELIAS, Adriana Maluf; LIANZA, Alessandro Cavalcanti; SAWAMURA, Karen Saori Shiraishi; MENEZES, Carolina Rocha Brito; FERREIRA, Juliana Caires de Oliveira Achili; SILVA, Clovis Artur; LEAL, Gabriela NunesObjectives This study aimed to investigate subclinical left ventricle (LV) systolic dysfunction in juvenile dermatomyositis (JDM) using two-dimensional speckle-tracking echocardiography (2DST). Possible associations between LV deformation impairment and disease activity/cumulative damage were also evaluated. Methods Thirty-fiveconsecutive JDM patients without cardiac symptoms and 35 healthy volunteers were enrolled. Clinical data were collected from medical records, and echocardiograms were performed by a pediatric cardiologist, unaware of patients' conditions. Results Patients and controls had similar age (12.6 +/- 0.7 vs.12.5 +/- 0.6;p= 0.97) and gender (11F:24M vs.11F:24M;p= 1.0). Median of JDM duration was 4.6 (0.04-17.6) years, and only 6/35 (17%) had active disease (disease activity score (DAS > 3)). Conventional echocardiogram revealed preserved LV ejection fraction (EF) (>= 55%) in all individuals. In JDM, 2DST identified reduction of LV longitudinal [-22(-17.2 to -27.9) % vs. -23(-20.8 to -27.4) %;p= 0.028)] and circumferential -23.9 +/- 2.8% vs. -26.7 +/- 2.9%;p= 0.0002) strain. Lower longitudinal strain was associated with DAS >3 -19.9(-17.2 to -26.5)% vs. -22.1-18.9 to -27.9)%;p= 0.046], MDI extent > 0 [-19(-17.2 to -22.5)% vs. -22.1-19.2 to -27.9)%;p= 0.0008], MDI severity > 0 [-19(-17.2 to -22.1)% vs. -22.3(-20.3 to -27.9)%;p= 0.0001] and calcinosis[-20.6(-17.2 to -23)% vs. -22.3(-20.3 to -27.9)%;p= 0.03]. Lower circumferential strain was associated with MDI extent > 0 (-22.1 +/- 3.87% vs. -24.4 +/- 2.3%;p= 0.039), MDI severity > 0 (-21.7 +/- 3% vs. 24.7 +/- 2.3%;p= 0.004) and calcinosis (-22.5 +/- 3.3% vs. -24.8 +/- 2.1%;p= 0.02). There was a negative correlation between longitudinal strain and cumulative dose of prednisone (r= -0.44;p= 0.009) and methotrexate (r= -0.33;p= 0.0008). Conclusions LV 2DST detected early systolic myocardial compromise in asymptomatic pediatric JDM patients, with preserved EF. Longitudinal strain impairment was associated with disease activity and cumulative damage, whereas circumferential strain impairment was associated exclusively with cumulative damage.
conferenceObject EPIDEMIOLOGY AND MANAGEMENT PRACTICES IN CHILDHOOD-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS: A LATIN AMERICA SURVEY(2018) FERREIRA, J. C.; TRINDADE, V. C.; ESPADA, G.; MOREL, Z.; BONFA, E.; MAGALHAES, C. S.; SILVA, C. A.- Diffuse alveolar hemorrhage in childhood-onset systemic lupus erythematosus: a severe disease flare with serious outcome(2018) BLAY, Gabriela; RODRIGUES, Joaquim C.; FERREIRA, Juliana C. O.; LEAL, Gabriela N.; GORMEZANO, Natali W.; V, Glaucia Novak; PEREIRA, Rosa M. R.; TERRERI, Maria T.; MAGALHAES, Claudia S.; MOLINARI, Beatriz C.; SAKAMOTO, Ana P.; AIKAWA, Nadia E.; CAMPOS, Lucia M. A.; FERNANDES, Taciana A. P.; CLEMENTE, Gleice; PERACCHI, Octavio A. B.; BUGNI, Vanessa; MARINI, Roberto; SACCHETTI, Silvana B.; CARVALHO, Luciana M.; FRAGA, Melissa M.; CASTRO, Tania C. M.; RAMOS, Valeria C.; BONFA, Eloisa; SILVA, Clovis A.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.
- Panniculitis in childhood-onset systemic lupus erythematosus: a multicentric cohort study(2019) VERDIER, Monica; ANUARDO, Pedro; GORMEZANO, Natali Weniger Spelling; ROMITI, Ricardo; CAMPOS, Lucia Maria Arruda; AIKAWA, Nadia Emi; PEREIRA, Rosa Maria Rodrigues; TERRERI, Maria Teresa; MAGALHAES, Claudia Saad; FERREIRA, Juliana C. O. A.; SILVA, Marco Felipe Castro; FERRIANI, Mariana; SAKAMOTO, Ana Paula; FERRIANI, Virginia Paes Leme; CENTEVILLE, Maraisa; SATO, Juliana; SANTOS, Maria Carolina; BONFA, Eloisa; SILVA, Clovis ArturObjective: To evaluate prevalence, clinical manifestations, laboratory abnormalities, treatment and outcome in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients with and without panniculitis. Methods: Panniculitis was diagnosed due to painful subcutaneous nodules and/or plaques in deep dermis/subcutaneous tissues and lobular/mixed panniculitis with lymphocytic lobular inflammatory infiltrate in skin biopsy. Statistical analysis was performed using Bonferroni correction(p <0.004). Results: Panniculitis was observed in 6/847(0.7%) cSLE. Painful subcutaneous erythematosus and indurated nodules were observed in 6/6 panniculitis patients and painful subcutaneous plaques in 4/6. Generalized distribution was evidenced in 3/ 6 and localized in upper limbs in 2/6 and face in 1/6. Cutaneous hyperpigmentation and/or cutaneous atrophy occurred in 5/6. Histopathology features showed lobular panniculitis without vasculitis in 5/6(one of them had concomitant obliterative vasculopathy due to antiphospholipid syndrome) and panniculitis with vasculitis in 1/6. Comparison between cSLE with panniculitis and 60 cSLE without panniculitis with same disease duration [2.75(0-11.4) vs. 2.83(0-11.8) years,p = 0297], showed higher frequencies of constitutional involvement (67% vs. 1 0%,p = 0.003) and leukopenia (67% vs. 7%p = 0.002). Cutaneous atrophy and hyperpigmentation occurred in 83% of patients. Conclusions: Panniculitis is a rare skin manifestation of cSLE occurring in the first three years of disease with considerable sequelae. The majority of patients have concomitant mild lupus manifestations.
- Autoimmune hepatitis in 847 childhood- onset systemic lupus erythematosus population: a multicentric cohort study(2018) BALBI, Verena A.; MONTENEGRO, Barbara; PITTA, Ana C.; SCHMIDT, Ana R.; FARHAT, Sylvia C.; COELHO, Laila P.; FERREIRA, Juliana C. O.; PEREIRA, Rosa M. R.; TERRERI, Maria T.; SAAD-MAGALHAES, Claudia; AIKAWA, Nadia E.; SAKAMOTO, Ana P.; KOZU, Katia; CAMPOS, Lucia M.; SALLUM, Adriana M.; FERRIANI, Virginia P.; PIOTTO, Daniela P.; BONFA, Eloisa; SILVA, Clovis A.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 Sao 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 lAHGC 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.
- DIFFUSE ALVEOLAR HEMORRHAGE: A MULTICENTER STUDY IN 847 CHILDHOOD-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS(2017) SILVA, C. A.; BLAY, G.; RODRIGUES, J. C.; LEAL, G. N.; FERREIRA, J. C.; NOVAK, G.; PEREIRA, R. M. R.; TERRERI, M. T.; MAGALHAES, C. S.; MOLINARI, B. C.; SAKAMOTO, A. P.; AIKAWA, N. E.; CAMPOS, L. M. A.; FERNANDES, T. A. P.; CLEMENTE, G.; PERACCHI, O. A. B.; BUGNI, V.; MARINI, R.; SACCHETTI, S. B.; CARVALHO, L. M.; FRAGA, M. M.; CASTRO, T. C. M.; RAMOS, V. C.; BONFA, E.
- Epidemiology and management practices for childhood-onset systemic lupus erythematosus patients: a survey in Latin America(2018) FERREIRA, Juliana C. O. A.; TRINDADE, Vitor C.; ESPADA, Graciela; MOREL, Zoilo; BONFA, Eloisa; MAGALHAES, Claudia S.; SILVA, Clovis ArturTo assess epidemiology and management practices of Latin America Pediatric Rheumatologists (LAPR) about childhood-onset systemic lupus erythematosus (cSLE). A cross-sectional study was performed in 288 LAPR PANLAR members based on online survey about cSLE practices. The response rate of web-based survey by LAPR was 170/288(59%) and the majority worked in university hospitals (63%). The ACR and/or SLICC classification criteria (99%) and disease activity tools (97%) were almost universally used by LAPR, whereas damage index (70%) and CHAQ (58%) instruments were less frequently used. Laboratory exams, diagnostic imaging, and biopsies were generally available (>75%), however low availability for densitometry (66%). Drug access was excellent for the most common prescribed medications (>75%), except for belimumab (11%). Emerging mosquito-borne diseases were also reported: dengue (20%), chikungunya (11%), and Zika (8%). Groups were further divided in two, according to the median number of cSLE patients followed by LAPR in the last year: groups A and B (25 and <25, respectively). Frequencies of condom in combination with other contraceptive methods were significantly higher in group A than B (p=0.01). The frequencies of reported pregnancy (p<0.001) and non-adherence to therapy were significantly higher in group A (p=0.023). Alcohol intake (p=0.004) and illicit drug use (p=0.007) were also reported more frequently by LAPR of group A in at least one cSLE patient. This first large web-based survey demonstrated an overall excellent access for diagnosis and therapy by LAPR, probably related to their high rate of practices in tertiary care of university hospitals. Adherence to therapy, pregnancy, and substance abuse was identified as major challenges in this population, particularly in larger centers.
- 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.