NATALI WENIGER SPELLING GORMEZANO

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  • article 24 Citação(ões) na Scopus
    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.
  • article 23 Citação(ões) na Scopus
    Chronic arthritis in systemic lupus erythematosus: distinct features in 336 paediatric and 1830 adult patients
    (2016) GORMEZANO, Natali W. S.; SILVA, Clovis A.; AIKAWA, Nadia E.; BARROS, Diego L.; SILVA, Mariana A. da; OTSUZI, Carini I.; KOZU, Katia; SEGURO, Luciana Parente; PEREIRA, Rosa M. R.; BONFA, Eloisa
    The objectives of this study are to assess the frequency of chronic arthritis and compare the clinical and laboratory features in a large population of childhood-onset systemic lupus erythematosus (cSLE) and adult-onset (aSLE) patients. This historical study evaluated 336 cSLE and 1830 aSLE patients. Chronic arthritis was defined as synovitis of at least 6 weeks of duration. Rhupus was characterised as the association of SLE and chronic inflammatory arthritis with erosion and positive rheumatoid factor. Jaccoud's arthropathy is a non-erosive subluxation leading to severe deformity of the hands and feet. Data were compared using Student's t test or the Mann-Whitney test for continuous variables. For categorical variables, differences were assessed by Fisher's exact test and Pearson chi-square. Frequencies of chronic arthritis were similar in cSLE and aSLE (2.4 vs. 3.8 %, p=0.261). The median time from disease onset to appearance of chronic arthritis was shorter in cSLE (0 vs. 10 years, p<0.001), and the median of age at chronic arthritis diagnosis was [10.8 (4.2-14.6) vs. 40 (21-67), p<0.001]. The children presented with more chronic polyarthritis than the adults (75 vs. 32 %, p=0.024), a higher median number of joints with arthritis [8.5 (118) vs. 3 (1-9), p=0.017] and a higher number of joints with limitation [1.5(0-24) vs. 0(0-4), p=0.004]. The chronic arthritis diagnosis frequencies of hepatomegaly (25 vs. 0 %, p=0.009), splenomegaly (25 vs. 0 %, p=0.009), pericarditis (25 vs. 0 %, p=0.009), nephritis (37 vs. 3 %, p=0.006), haematuria (37 vs. 1.4 %, p=0.002), lupus anticoagulant (40 vs. 1.6 %, p=0.012), anticardiolipin IgM (40 vs. 1.5 %, p=0.012) and median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) [10.5(1-20) vs. 6(4-16), p=0.029] were higher in cSLE. Frequency of rhupus, (12 vs. 17 %, p=1.0), Jaccoud's arthropathy (0 vs. 17 %, p=0.343) and treatments were similar in cSLE and aSLE. We determined that chronic arthritis in SLE has distinct features in children, with very early onset, polyarticular involvement and association with active disease. We further demonstrated in this series that a proportion of chronic arthritis involvement in SLE is manifested as rhupus and Jaccoud's arthropathy.
  • article 15 Citação(ões) na Scopus
    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, Eloisa
    Herpes 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).
  • article 38 Citação(ões) na Scopus
    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.
  • article 13 Citação(ões) na Scopus
    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.
  • article 12 Citação(ões) na Scopus
    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.
  • conferenceObject
    Distinct Clinical Correlates of Immune Thrombocytopenic Purpura at Diagnosis of Childhood-Onset and Adult SLE
    (2016) ESTEVES, Gladys; GORMEZANO, Natali W.; PEREIRA, Oriany; KERN, David; KOZU, Katia T.; PEREIRA, Rosa M. R.; SILVA, Clovis A.; BONFA, Eloisa; AIKAWA, Nadia E.
  • article 17 Citação(ões) na Scopus
    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.
  • article 11 Citação(ões) na Scopus
    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.
  • article 32 Citação(ões) na Scopus
    Autoimmune hemolytic anemia in systemic lupus erythematosus at diagnosis: differences between pediatric and adult patients
    (2017) GORMEZANO, N. W. S.; KERN, D.; PEREIRA, O. L.; ESTEVES, G. C. X.; SALLUM, A. M. E.; AIKAWA, N. E.; PEREIRA, R. M. R.; SILVA, C. A.; BONFA, E.
    Objective To determine the overall prevalence of autoimmune hemolytic anemia (AIHA), and to compare clinical and laboratory features in a large population of children and adult lupus patients at diagnosis. Methods This retrospective study evaluated the medical charts of 336 childhood-onset systemic lupus erythematosus (cSLE) and 1830 adult SLE (aSLE) patients followed in the same tertiary hospital. Demographic data, clinical features and disease activity were recorded. AIHA was defined according to the presence of anemia (hemoglobin <10g/dL) and evidence of hemolysis (reticulocytosis and positive direct antiglobulin test (DAT)/Coombs test) at SLE diagnosis. Evans syndrome (ES) was defined by the combination of immune thrombocytopenia (platelet count <100,000/mm(3)) and AIHA. Results The frequency of AIHA at diagnosis was significantly higher in cSLE patients compared to aSLE (49/336 (14%) vs 49/1830 (3%), p=0.0001), with similar frequency of ES (3/336 (0.9%) vs 10/1830 (0.5%), p=0.438). The median of hemoglobin levels was reduced in cSLE vs aSLE patients (8.3 (2.2-10) vs 9.5 (6.6-10) g/dL, p=0.002) with a higher frequency of multiple hemorrhagic manifestations (41% vs 7%, p=0.041) and erythrocyte transfusion due to bleeding (24% vs 5%, p=0.025). cSLE patients also had more often constitutional involvement (84% vs 31%, p<0.001), fever (65% vs 26%, p<0.001), weight loss>2kg (39% vs 6%, p<0.001), reticuloendothelial manifestations (48% vs 8%, p<0.001), hepatomegaly (25% vs 2%, p<0.001) and splenomegaly (21% vs 2%, p=0.004). Other major organ involvements were common but with similar frequencies in cSLE and aSLE (p>0.05). Median systemic lupus erythematosus disease activity index 2000 (SLEDAI-2K) was comparable in cSLE and aSLE (p=0.161). Conclusions We identified that AIHA was not a common condition in cSLE and aSLE, with distinct features characterized by a higher prevalence/severity in children and concomitant constitutional symptoms in the majority of them.