NATALI WENIGER SPELLING GORMEZANO
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- Pancreatitis Subtypes Survey in 852 Childhood-Onset Systemic Lupus Erythematosus Patients(2016) MARQUES, Victor L. S.; GORMEZANO, Natali W. S.; BONFA, Eloisa; AIKAWA, Nadia E.; TERRERI, Maria T.; PEREIRA, Rosa M.; MAGALHAES, Claudia S.; GUARIENTO, Andressa; APPENZELLER, Simone; FERRIANI, Virginia P.; BARBOSA, Cassia M.; RAMOS, Valeria C.; LOTUFO, Simone; SILVA, Clovis A.Objective:Pancreatitis is a rare and a life-threatening systemic lupus erythematosus (SLE) manifestation in childhood-onset SLE (cSLE). The objective of this study was to systematically classify pancreatitis in cSLE according to the International Study Group of Pediatric Pancreatitis and determine the overall prevalence, clinical features, laboratory, and first episode outcomes.Methods:A multicenter cohort study in 10 pediatric rheumatology centers, including 852 patients with cSLE.Results:Pancreatitis was diagnosed in 22 of 852 (2.6%) patients with cSLE. It was classified as acute pancreatitis in 20 (91%), acute recurrent pancreatitis in 2 (9%), and none of them had chronic pancreatitis. None of them had gallstones, traumatic pancreatitis, or reported alcohol/tobacco use. The comparison of patients with pancreatitis (first episode) and without this complication revealed a shorter disease duration (1 [0-10] vs 4 [0-23] years, P<0.0001) and higher median of Systemic Lupus Erythematosus Disease Activity Index 2000 (21 [0-41] vs 2 [0-45], P<0.0001). The frequencies of fever (P<0.0001), weight loss (P<0.0001), serositis (P<0.0001), nephritis (P<0.0001), arterial hypertension (P<0.0001), acute renal failure (P<0.0001), macrophage activation syndrome (P<0.0001), and death (P=0.001) were also higher in patients with pancreatitis. The frequencies of intravenous methylprednisolone use (P<0.0001) and the median of prednisone dose (55 [15-60] vs 11 [1-90] mg/day, P<0.0001) were significantly higher in patients with pancreatitis. Of note, the 2 patients with acute recurrent pancreatitis had 2 episodes, with pain-free interval of 1 and 4 years.Conclusions:This was the first study characterizing pancreatitis using the International Study Group of Pediatric Pancreatitis standardized definitions in patients with cSLE showing that the predominant form is acute pancreatitis seen in association with glucocorticoid treatment and active severe disease.
- HIGHER PREVALENCE AND DISTINCT FEATURES OF HERPES ZOSTER INFECTION IN CHILDREN THAN ADULTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS(2015) GORMEZANO, Natali W. S.; SILVA, Clovis A.; OTSUZI, Carini I.; BARROS, Diego L.; SILVA, Mariana A. da; SALLUM, Adriana M. E.; PASOTO, Sandra; PEREIRA, Rosa M. R.; BONFA, EloisaHerpes zoster infection was significantly more often observed in children (10%, N = 362) than in adults (4%, N = 1830). At herpes zoster infection diagnosis, disease activity score (8 vs. 3, P = 0.002) was higher in children, and fever (43% vs. 12%, P < 0.0001), nephritis (45% vs. 25%, P = 0.038), anti-double-stranded DNA autoantibodies (76% vs. 15%, P < 0.0001) and low C4 (48% vs. 22%, P = 0.017) were more often observed in children versus adults. Post herpetic neuralgia was less common in children than adults (3% vs. 24%, P =0.005).
- 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.
- 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.
- Herpes zoster infection in childhood-onset systemic lupus erythematosus patients: a large multicenter study(2016) FERREIRA, J. C. O. A.; MARQUES, H. H.; FERRIANI, M. P. L.; GORMEZANO, N. W. S.; TERRERI, M. T.; PEREIRA, R. M.; MAGALHAES, C. S.; CAMPOS, L. M.; BUGNI, V.; OKUDA, E. M.; MARINI, R.; PILEGGI, G. S.; BARBOSA, C. M.; BONFA, E.; SILVA, C. A.Objective The aim of this multicenter study in a large childhood-onset systemic lupus erythematosus (cSLE) population was to assess the herpes zoster infection (HZI) prevalence, demographic data, clinical manifestations, laboratory findings, treatment, and outcome. Methods A retrospective multicenter cohort study (Brazilian cSLE group) was performed in ten Pediatric Rheumatology services in SAo Paulo State, Brazil, and included 852 cSLE patients. HZI was defined according to the presence of acute vesicular-bullous lesions on erythematous/edematous base, in a dermatomal distribution. Post-herpetic neuralgia was defined as persistent pain after one month of resolution of lesions in the same dermatome. Patients were divided in two groups for the assessment of current lupus manifestations, laboratory findings, and treatment: patients with HZI (evaluated at the first HZI) and patients without HZI (evaluated at the last visit). Results The frequency of HZI in cSLE patients was 120/852 (14%). Hospitalization occurred in 73 (61%) and overlap bacterial infection in 16 (13%). Intravenous or oral aciclovir was administered in 113/120 (94%) cSLE patients at HZI diagnosis. None of them had ophthalmic complication or death. Post-herpetic neuralgia occurred in 6/120 (5%). After Holm-Bonferroni correction for multiple comparisons, disease duration (1.58 vs 4.41 years, p<0.0001) was significantly lower in HZI cSLE patients compared to those without HZI. Nephritis (37% vs 18%, p<0.0001), lymphopenia (32% vs 17%, p<0.0001) prednisone (97% vs 77%, p<0.0001), cyclophosphamide (20% vs 5%, p<0.0001) and SLE Disease Activity Index 2000 (6.0 (0-35) vs 2 (0-45), p<0.0001) were significantly higher in the former group. The logistic regression model showed that four independent variables were associated with HZI: disease duration<1 year (OR 2.893 (CI 1.821-4.597), p<0.0001), lymphopenia <1500/mm(3) (OR 1.931 (CI 1.183-3.153), p=0.009), prednisone (OR 6.723 (CI 2.072-21.815), p=0.002), and cyclophosphamide use (OR 4.060 (CI 2.174-7.583), p<0.0001). Conclusion HZI is an early viral infection in cSLE with a typical dermatomal distribution. Lymphopenia and immunosuppressive treatment seem to be major factors underlying this complication in spite of a benign course.