ROBERTA CUNHA GOMES
Índice h a partir de 2011
4
Projetos de Pesquisa
Unidades Organizacionais
Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina - Médico
6 resultados
Resultados de Busca
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- Childhood-onset bullous systemic lupus erythematosus(2014) LOURENCO, D. M. R.; GOMES, R. Cunha; AIKAWA, N. E.; CAMPOS, L. M. A.; ROMITI, R.; SILVA, C. A.Bullous systemic lupus erythematosus has rarely been described in pediatric lupus population and the real prevalence of childhood-onset bullous systemic lupus erythematosus has not been reported. From January 1983 to November 2013, 303 childhood-onset SLE (c-SLE) patients were followed at the Pediatric Rheumatology Unit of the Childres Institute of Hospital das Clinicas da Faculdade de Medicina Universidade da Universidade de SAo Paulo, three of them (1%) diagnosed as childhood-onset bullous systemic lupus erythematosus. All three cases presented tense vesiculobullous lesions unassociated with lupus erythematosus lesions, with the median duration of 60 days (30-60). All patients fulfilled bullous systemic lupus erythematosus criteria. Two had nephritis and serositis and presented specific autoantibodies. The histological pattern demonstrated subepidermal blisters with neutrophils-predominant infiltrates within the upper dermis. Direct immunofluorescence (DIF) showed deposits of IgG and complement along the epidermal basement membrane, in the presence or absence of IgA and/or IgM. A positive indirect immunofluorescence on salt-split skin demonstrating dermal binding was observed in two cases. All of them had moderate/severe disease activity at diagnosis with median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) of 18 (14-24). Two patients received dapsone and one with severe nephritis received immunosuppressive drugs. In conclusion, in the last 30 years the prevalence of bullous lupus in childhood-onset lupus population was low (1%) in our tertiary University Hospital. A diagnosis of SLE should always be considered in children with recurrent tense vesiculobullous lesions with or without systemic manifestations.
- 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.
- Features of 847 Childhood-Onset Systemic Lupus Erythematosus Patients in Three Age Groups at Diagnosis: A Brazilian Multicenter Study(2016) GOMES, Roberta C.; SILVA, Marco F.; KOZU, Katia; BONFA, Eloisa; PEREIRA, Rosa M.; TERRERI, Maria T.; MAGALHAES, Claudia S.; SACCHETTI, Silvana B.; MARINI, Roberto; FRAGA, Melissa; CARVALHO, Luciana M.; BARBOSA, Cassia M.; CARNEIRO-SAMPAIO, Magda; SILVA, Clovis A.ObjectiveTo evaluate demographic data and clinical and laboratory features at disease diagnosis in 3 different age groups of childhood-onset systemic lupus erythematosus (SLE): group A, early-onset (<6 years); group B, school age (6 to <12 years); and group C, adolescent (12 to <18 years). MethodsThis was a Brazilian multicenter cohort retrospective study in 10 pediatric rheumatology centers, including 847 childhood-onset SLE patients. ResultsPatients were divided into 3 groups: group A with 39 patients (4%), group B with 395 patients (47%), and group C with 413 patients (49%). Of 39 childhood-onset SLE patients in group A, 3 (8%) were ages <2 years, 4 (10%) were 2 to <3 years, and 32 (82%) were 3 and <6 years. A total of 74 childhood-onset SLE patients were analyzed for C1q levels, and complete C1q deficiency was observed in 3 of 74 patients (4%), all in group A. Groups were similar regarding high frequencies of female sex, nephritis, neuropsychiatric involvement, Systemic Lupus Erythematosus Disease Activity Index 2000 score 8, autoantibody profile, elevated acute phase proteins, and low complement levels (P>0.05). However, the frequency of fever (78% versus 61% versus 47%; P<0.0001), hepatomegaly (42% versus 29% versus 14%; P<0.0001), splenomegaly (28% versus 12% versus 4%; P<0.0001), and discoid lupus (13% versus 4% versus 4%; P=0.020) was significantly higher in group A compared to groups B and C. The frequency of weight loss >2 kg (19% versus 28% versus 36%; P=0.017), photosensitivity (34% versus 41% versus 51%; P=0.006), leukopenia <4,000/mm(3) (14% versus 25% versus 30%; P=0.048), and lymphopenia <1,500/mm(3) (22% versus 41% versus 47%; P=0.011) was significantly lower in group A. ConclusionOur large multicenter study identified the finding that the initial appearance of childhood-onset SLE is characterized by comparable high frequency of internal organ involvement and some distinct clinical and laboratory features in early-onset and adolescent groups.
- Severe intestinal involvement as initial manifestation of systemic childhood polyarteritis nodosa: Report of two cases(2013) GOMES, Roberta C.; MARQUES, Victor L. S.; CAVALCANTE, Erica G. N.; CAMPOS, Lucia M. A.; SALLUM, Adriana M.; TANNURI, Uenis; SILVA, Clovis A. daSystemic childhood polyarteritis nodosa (C-PAN) is a rare primary vasculitis involving medium or small sized arteries. Abdominal angina is an important and serious complication of PAN, occurring usually 15 to 30 min after food intake, and particularly in adult patients. However, to our knowledge, this involvement as the first manifestation of C-PAN was not described. Therefore, we reported herein two C-PAN cases that fulfilled the new criteria for this vasculitis. These patients were young boys that had malignant arterial hypertension and recurrent post-prandial cramping with acute abdomen. Both of them were submitted to laparotomy that revealed multiple and diffuse intestinal necrosis. One of our cases had a severe post-prandial cramping, even after drinking water, and the laparotomy evidenced multiple intestinal perforations. In spite of use of antihypertensive therapies, immunosuppressive agents (corticosteroids, cyclophosphamide and/or methotrexate) and intravenous immunoglobulin, they died possibly due to severe and disseminated activity disease. In conclusion, we described herein the first two fatal cases of C-PAN that presented severe abdominal pain as initial manifestation. We suggest that the diagnosis of PAN should be considered in patients under acute abdominal angina with no apparent etiology.
conferenceObject Features of 847 Childhood-Onset Systemic Lupus Erythematousus Patients in Three Age-Related Groups at Diagnosis: A Brazilian Multicenter Study(2015) SILVA, Clovis A.; SR., Roberta C. Gomes; SILVA, Marco F.; KOZU, Katia T.; BONFA, Eloisa; PEREIRA, Rosa M. R.; TERRERI, Maria Teresa; SR., Claudia S. Magalhaes; SR., Silvana B. Sacchetti; SR., Roberto Marini; FRAGA, Melissa; SR., Luciana M. Carvalho; BARBOSA, Cassia M.; SR., Magda Carneiro-SampaioconferenceObject Outcome of 847 Childhood-Onset Systemic LUPUS Erythematousus Patients in Three Age Groups(2016) LOPES, Sandra R. M.; GORMEZANO, Natali W.; SR., Roberta C. Gomes; AIKAWA, Nadia E.; PEREIRA, Rosa M. R.; TERRERI, Maria Teresa; MAGALHAES, Claudia S.; OKUDA, Eunice M.; SAKAMOTO, Ana Paula; SALLUM, Adriana M. E.; APPENZELLER, Simone; FERRIANI, Virginia; BARBOSA, Cassia M.; LOTUFO, Simone; JESUS, Adriana A.; ANDRADE, Luis E. C.; CAMPOS, Lucia M. A.; BONFA, Eloisa; SILVA, Clovis A.