NATALI WENIGER SPELLING GORMEZANO
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5 resultados
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- Subclinical Pulmonary Hypertension in Childhood Systemic Lupus Erythematosus Associated with Minor Disease Manifestations(2017) ANUARDO, Pedro; VERDIER, Monica; GORMEZANO, Natali W. S.; FERREIRA, Gabriela R. V.; LEAL, Gabriela N.; LIANZA, Alessandro; FERREIRA, Juliana C. O. A.; PEREIRA, Rosa M. R.; AIKAWA, Nadia E.; TERRERI, Maria Teresa; MAGALHES, Claudia S.; APPENZELLER, Simone; SANTOS, Maria Carolina dos; SACHETTI, Silvana B.; LEN, Claudio A.; PILLEGGI, Gecilmara S.; LOTUFO, Simone; BONFA, Eloisa; SILVA, Clovis A.The aim of this study was to evaluate pulmonary hypertension (PH) in 852 childhood-onset systemic lupus erythematosus (cSLE) patients. This was a large multicenter study conducted in 10 Pediatric Rheumatology Services of So Paulo state, Brazil. PH was defined as systolic pulmonary artery pressure > 35 mmHg and/or measurement of the mean pulmonary artery pressure > 25 mmHg and/or diastolic pressure > 15 mmHg by transthoracic echocardiogram. Demographic data, clinical manifestations, disease activity score (SLEDAI-2K), disease damage score (SLICC/ACR-DI) and treatments were also evaluated. Statistical analysis was performed using Bonferroni correction (p < 0.002). PH was observed in 17/852 (2%) cSLE patients. Effort dyspnea occurred in 3/17, chest pain in 1/17 and right ventricle dysfunction in 3/17 cSLE patients. None had pulmonary thromboembolism or antiphospholipid syndrome. Further comparison between 17 cSLE with PH and 85 cSLE control patients without PH with similar disease duration [15 (0-151) vs. 15 (0-153) months, p = 0.448], evaluated at the last visit, revealed higher frequencies of fever (47 vs. 9%, p < 0.001), reticuloendothelial manifestations (41 vs. 7%, p < 0.001) and serositis (35 vs. 5%, p = 0.001) in the former group. Frequencies of renal and neuropsychiatric involvements and antiphospholipid syndrome, as well as the median of SLEDAI-2K and SLICC/ACR-DI scores, were comparable in both groups (p > 0.002). Normal transthoracic echocardiography was evidenced in 9/17 (53%), with median cSLE duration of 17.5 months (1-40) after PH standard treatment. PH was a rare manifestation of cSLE occurring in the first two years of disease. The majority of patients were asymptomatic with mild lupus manifestations. The underlying mechanism seemed not to be related to pulmonary thromboembolism and/or antiphospholipid syndrome.
- 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.
- 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.
- Macrophage activation syndrome: A severe and frequent manifestation of acute pancreatitis in 362 childhood-onset compared to 1830 adult-onset systemic lupus erythematosus patients(2016) GORMEZANO, Natali W. S.; OTSUZI, Carini I.; BARROS, Diego L.; SILVA, Mariana A. da; PEREIRA, Rosa M. R.; CAMPOS, Lucia M. A.; BORBA, Eduardo F.; BONFA, Eloisa; SILVA, Clovis A.Objective: We previously reported a case series of acute pancreatitis (AP) and macrophage activation syndrome (MAS) in childhood (cSLE) patients; however, there are no data regarding the comparison of AP and MAS in large populations of cSLE and adult SLE (aSLE). Methods: A study included 362 cSLE and 1830 aSLE patients. MAS was diagnosed according to preliminary diagnostic guidelines and AP according to the presence of abdominal pain or vomiting associated to an increase of pancreatic enzymes and/or pancreatic radiological abnormalities. Demographic data, clinical features, SLEDAI-2K, SLICC/ACR-DI, and treatment were assessed. Results: Age in MAS patients was significantly lower compared with those without this complication [15 (8.8-55) vs. 33.5 (10.2-45.7) years, p = 0.0071. The frequencies of fever (94% vs. 37%, p = 0.001), leucopenia (82% vs. 19%, p = 0.0001), thrombocytopenia (65% vs. 19%, p = 0.013), hypertriglyceridemia (87% vs. 42%, p = 0.037), and hyperferritinemia (93% vs. 37%, p = 0.011) were also more frequently observed in AP patients with MAS compared in AP patients without MAS. Fever and hyperferritinemia concomitantly were more frequent in the former group (86% vs. 12%, p = 0.0015). Higher and significant frequency of AP in cSLE compared to aSLE patients [12/362 (3.3%) vs. 20/1830 (1.1%), p = 0.003], with similar AP duration [22 (6-60) vs. 15 (4-90) days, p = 0.534]. MAS (85% vs. 30%, p = 0.003) and death by MAS complication (31% vs. 0%, p = 0.017) were significantly higher in children compared with aSLE. Conclusions: This study provides novel data demonstrating that MAS occur in the majority of cSLE with AP with a higher mortality compared to aSLE. In addition, we identified in AP patients, a cluster of MAS clinical and laboratorial parameters more associated with this complication.