LUCIA MARIA MATTEI DE ARRUDA CAMPOS
Projetos de Pesquisa
Unidades Organizacionais
Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina - Médico
LIM/36 - Laboratório de Pediatria Clínica, Hospital das Clínicas, Faculdade de Medicina
LIM/36 - Laboratório de Pediatria Clínica, Hospital das Clínicas, Faculdade de Medicina
17 resultados
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- Subclinical pulmonary abnormalities in childhood-onset systemic lupus erythematosus patients(2016) VEIGA, C. S.; COUTINHO, D. S.; NAKAIE, C. M. A.; CAMPOS, L. M. A.; SUZUKI, L.; CUNHA, M. T.; LEONE, C.; SILVA, C. A.; RODRIGUES, J. C.Objective The aims of this study were to analyze the pulmonary function of childhood-onset systemic lupus erythematosus (cSLE) patients and to identify possible correlations between the high-resolution computed chest tomography (HRCT) score, disease activity, disease cumulative damage, and the participants' quality of life. Methods Forty cSLE patients, median age: 14.1 years (range: 7.4-17.9), underwent spirometry and plethysmography. Carbon monoxide diffusing capacity (DLCO), HRCT, disease activity, disease cumulative damage, and quality of life were assessed. Results Pulmonary abnormalities were evident in 19/40 (47.5%) cSLE patients according to spirometry/DLCO. Forced expired volume in one second (FEV1%) was the parameter most affected (30%). The HRCT showed some abnormality in 22/30 patients (73%), which were minimal in 43%. Signs of airway affects were found in 50%. Twelve patients were hospitalized due to cSLE-related pulmonary complications before the study began (median discharge: 2.1 years earlier). Total lung capacity (TLC%), vital capacity (VC%), forced vital capacity (FVC%), and FEV1% were significantly lower in the group with hospitalization compared to the group without hospitalization (p=0.0025, p=0.0022, p=0.0032, and p=0.0004, respectively). Of note, DLCO was positively correlated with disease duration (r=+0.4; p=0.01). The HRCT-score was negatively correlated with FEV1/VC (r=-0.63; p=0.0002), FEV1 (r=-0.54; p=0.018), FEF25%-75% (r=-0.67; p<0.0001), and HRCT-score was positively correlated with resistance (r=+0.49; p=0.0056). Conclusions Almost half of patients with cSLE had subclinical pulmonary abnormalities, especially airway abnormalities. The cSLE-related pulmonary complications seem to determine long-term functional damage.
- Risk factors for mortality in 1528 Brazilian childhood-onset systemic lupus erythematosus patients(2023) SAKAMOTO, Ana P.; SILVA, Clovis A.; PITA, Ana C.; TRINDADE, Vitor C.; ISLABAO, Aline G.; FIOROT, Fernanda J.; LOPES, Sandra R. M.; PEREIRA, Rosa M. R.; SAAD-MAGALHAES, Claudia; RUSSO, Gleice C. S.; LEN, Claudio A.; PRADO, Rogerio do; CAMPOS, Lucia M. A.; AIKAWA, Nadia E.; APPENZELLER, Simone; FERRIANI, Virginia P. L.; SILVA, Marco F.; FELIX, Marta; FONSECA, Adriana R.; ASSAD, Ana P. L.; SZTAJNBOK, Flavio R.; SANTOS, Maria C.; BICA, Blanca E.; SENA, Evaldo G.; MORAES, Ana J.; FRAGA, Melissa M.; ROBAZZI, Teresa C.; SPELLING, Paulo F.; SCHEIBEL, Iloite M.; CAVALCANTI, Andre S.; MATOS, Erica N.; GUIMARAES, Luciano J.; SANTOS, Flavia P.; MOTA, Licia M. H.; BONFA, Eloisa; TERRERI, Maria T.Objectives: To identify associations between mortality in cSLE patients and their characteristics: clinical and laboratory features, disease activity and damage scores, and treatment; to evaluate risk factors associated with mortality in cSLE; and to determine the most frequent causes of death in this group of patients.Methods: We performed a multicenter retrospective cohort using data from 1,528 cSLE patients followed in 27 pediatric rheumatology tertiary centers in Brazil. Patients' medical records were reviewed according to a standardized protocol, in which information regarding demographic and clinical features, disease activity and damage scores, and treatment were collected and compared between deceased cSLE patients and survivors. Univariate and multivariate analyses by Cox regression model were used to calculate risk factors for mortality, whereas survival rates were analyzed by Kaplan-Meier plots.Results: A total of 63/1,528 (4.1%) patients deceased, 53/63 were female (84.1%), median age at death was 11.9 (9.4-13.1) years and median time interval between cSLE diagnosis and death was 3.2 (0.5-5.3) years. Sepsis was the main cause of death in 27/63 (42.8%) patients, followed by opportunistic infections in 7/63 (11.1%), and alveolar hemorrhage in 6/63 (9.5%) patients. The regression models resulted in neuropsychiatric lupus (NP-SLE) (HR = 2.56, 95% CI = 1.48-4.42) and chronic kidney disease (CKD) (HR = 4.33, 95% CI = 2.33-4.72), as risk factors significantly associated with mortality. Overall patient survival after cSLE diagnosis at 5, 10, and 15 years were 97%, 95.4%, and 93.8%, respectively.Conclusions: This study confirmed that the recent mortality rate in cSLE in Brazil is low, but still of concern. NP-SLE and CKD were the main risk factors for mortality, indicating that the magnitude of these manifestations was significantly high.
- Munchausen by proxy syndrome mimicking childhood-onset systemic lupus erythematosus(2019) KUHNE, A. C. A.; PITTA, A. C.; GALASSI, S. C.; GONCALVES, A. M. F.; CARDOSO, A. C. A.; PAZ, J. A.; CAMPOS, L. M. A.; SILVA, C. A.Childhood-onset systemic lupus erythematosus (cSLE) is a chronic inflammatory multisystem autoimmune disease that requires multiple differential diagnoses. Munchausen by proxy syndrome (MBPS) is a form of child abuse, where a caregiver intentionally creates a medical history and induces or fabricates signs or disease in a patient. To our knowledge, there is no case report of MBPS mimicking cSLE diagnosis. We reported herein a 9-year-old male patient, with a history of multiple hospitalizations due to seizures with altered levels of consciousness. The mother reported malar rash, photosensitivity, alopecia, arthralgia, arterial hypertension, macroscopic hematuria, seizure and positive antinuclear antibodies. In the other service, he was treated with intravenous methylprednisolone, prednisone and mycophenolate mofetil. At 8 years and 8 months, he was admitted to our tertiary center with history of fever and macroscopic hematuria. Laboratory examinations were normal, including negative for antinuclear antibodies, anti-double stranded DNA, anticardiolipin, anti-Ro/SSA, anti-La/SSB, anti-RNP and anti-Sm antibodies. Multiple urine cultures revealed the presence of Enterococcus faecium, Acinetobacter sp., Stenotrophomonas maltophilia and Serratia marcescens, without any association with pyuria. At 8 years and 9 months, he was readmitted at emergency room with history of severe fever, headache, vomiting, photophobia, phonophobia and dizziness. The physical examination showed agitation, confusion, ataxic gait, slurred speech, horizontal nystagmus, painful facial expressions, tachycardia and weight loss. Brain magnetic resonance angiography and cerebrospinal fluid analysis were normal. During hospitalization, he had an acute episode of epistaxis and otalgia with excoriation in the auditory canal. At that moment, the suspicion of MBPS mimicking cSLE was raised and phenytoin intoxication was confirmed (peak phenytoin concentration was 45.4 mcg/mL, therapeutic range 10-20 mcg/mL). The mother and the patient were immediately separated, and she was replaced by another legal guardian. One week later, the neurological and other signs and symptoms were completely resolved. The child was placed under paternal custody with a court order and moved to another state. After that, the mother reported phenytoin use for her child and was referred to psychiatric follow-up. In conclusion, the first case of MBPS mimicking cSLE, resulting in multiple unnecessary examinations and treatments with delayed diagnosis was reported.
- 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.
- Validation of the Portuguese Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY) in Brazil(2013) MOORTHY, L. N.; SAAD-MAGALHAES, C.; SATO, J. Oliveira; LEN, C. A.; VASCO, M. Brandao; APPENZELLER, S.; MARINI, R.; OLIVEIRA, S. K. Feitosa de; RODRIGUES, M.; SZTAJNBOK, F.; ALMEIDA, R. Gasparello de; JESUS, A. Almeida de; CAMPOS, L. M.; SILVA, C. A.; PETERSON, M. G. E.; HASSETT, A. L.; WEISS, E.; VERMA, S.; DAHODWALA, M. Q.; LEHMAN, T. J. A.Background and Objective: Simple Measure of the Impact of Lupus Erythematosus in Youngsters (SMILEY) is a health-related quality of life (HRQOL) assessment tool for pediatric systemic lupus erythematosus (SLE), which has been translated into Portuguese for Brazil. We are reporting preliminary data on cross-cultural validation and reliability of SMILEY in Portuguese (Brazil). Methods: In this multi-center cross-sectional study, Brazilian children and adolescents 5-18 years of age with SLE and parents participated. Children and parents completed child and parent reports of Portuguese SMILEY and Portuguese Pediatric Quality of Life Inventory (PedsQL (TM)) Generic and Rheumatology modules. Parents also completed the Childhood Health Assessment Questionnaire (CHAQ). Physicians completed the SLE disease activity index (SLEDAI), Physician's Global Assessment of disease activity (PGA) and Systemic Lupus Erythematosus International Collaborating Clinics ACR Damage Index (SDI). Results: 99 subjects (84 girls) were enrolled; 93 children and 97 parents filled out the SMILEY scale. Subjects found SMILEY relevant and easy to understand and completed SMILEY in 5-15 minutes. Brazilian SMILEY was found to have good psychometric properties (validity and reliability), and the child-parent agreement was moderate. Conclusion: SMILEY may eventually be used routinely as a research/clinical tool in Brazil. It may be also adapted for other Portuguese-speaking nations offering critical information regarding the effect of SLE on HRQOL for children with SLE. Lupus (2013) 22, 190-197.
- Laboratory-confirmed pediatric COVID-19 in patients with rheumatic diseases: A case series in a tertiary hospital(2021) IHARA, Bianca P.; STRABELLI, Claudia A. A.; SIMON, Juliana R.; VIANA, Vivianne S. L.; SALLUM, Adriana M. E.; KOZU, Katia T.; AIKAWA, Nadia E.; LEAL, Gabriela N.; PEREIRA, Maria F. B.; MARQUES, Heloisa H.; SILVA, Clovis A.; CAMPOS, Lucia M.
- Invasive aspergillosis: a severe infection in juvenile systemic lupus erythematosus patients(2012) SILVA, M. F.; RIBEIRO, A. S.; FIOROT, F. J.; AIKAWA, N. E.; LOTITO, A. P.; CAMPOS, L. M.; MAUAD, T.; SILVA, C. A.Infections are an important cause of morbidity and mortality in juvenile systemic lupus erythematosus (JSLE). Among them, invasive aspergillosis (IA), which is usually related to immunosuppressed patients, has been rarely reported in JSLE. From 1983 to 2011, 5604 patients were followed at our institution and 283 (5%) met the American College of Rheumatology (ACR) classification criteria for SLE. Six (2.1%) of our JSLE patients had IA. One of them was previously reported and five will be described herein. Four of them were female. The median age at JSLE diagnosis was 12 years (8-16) and the median interval between diagnosis of JSLE and IA was 6 months (1-38). All had pulmonary involvement and three of them had systemic involvement. The median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) was 19 (7-22). Diagnosis of IA was performed by isolation of Aspergillus spp., two in bronchoalveolar lavage culture and by way of autopsy in the others. All of them were treated with corticosteroids and/or immunosuppressive drugs at IA diagnosis (azathioprine and/or intravenous cyclophosphamide). They all required treatment in the pediatric intensive care unit with mechanical ventilation and antifungal therapy (fluconazole, amphotericin B, itraconazole and/or voriconazole); nonetheless, none of them survived. In conclusion, this was the first report that evaluated the prevalence of IA in a large population of JSLE patients from a tertiary pediatric hospital, and clearly showed the severity of the outcome, especially in patients with active disease and treated with immunosuppressive agents. This study reinforces the importance of early diagnosis and treatment with certain antifungals, especially in critically ill patients. Lupus (2012) 21, 1011-1016.
- Subclinical right ventricle systolic dysfunction in childhood-onset systemic lupus erythematosus: insights from two-dimensional speckle-tracking echocardiography(2015) LEAL, G. N.; SILVA, K. F.; FRANCA, C. M. P.; LIANZA, A. C.; ANDRADE, J. L.; CAMPOS, L. M. A.; BONFA, E.; SILVA, C. A.Objective The objective of this article is to evaluate right ventricle strain imaging by two-dimensional speckle-tracking (2DST) in childhood-onset systemic lupus erythematosus (c-SLE). Methods Thirty-five c-SLE patients with no signs or symptoms of heart failure and 33 healthy volunteers were evaluated by standard echocardiogram and 2DST. Conventional parameters included tricuspid annular plane systolic excursion (TAPSE), RV tissue-Doppler-derived Tei index and systolic pulmonary artery pressure. Global peak longitudinal systolic strain (PLSS) and strain rate (PLSSR) of RV were obtained by 2DST. Demographic/clinical features, SLEDAI-2K/SLICC/ACR-DI and treatment were also assessed. Results The median current age was similar in patients and controls (14.75 vs. 14.88 years, p=0.62). RV PLSS was significantly reduced in c-SLE (-24.55.09 vs. -27.62 +/- 3.02%, p=0.003). Similar findings were observed after excluding patients with pulmonary hypertension (-24.62 +/- 4.87% vs. -27.62 +/- 3.02%, p=0.0041). RV PLSS was positively correlated with TAPSE (r=+0.49, p=0.0027) and negatively correlated with Tei index (r=-0.34, p=0.04) in c-SLE. RV PLSSR was not different comparing patients and controls (-0.65s(-1)+/- 0.47 vs. -1.87 +/- 0.49s(-1), p=0.07). Further analysis of c-SLE patients revealed higher frequencies of neuropsychiatric manifestations (39% vs. 0%, p=0.007) and antiphospholipid antibodies (55% vs. 18%, p=0.035) in those with RV PLSS-23.7% vs >-23.7%. No differences were evidenced in demographic data, disease activity/damage or treatments (p>0.05). Conclusions The present study, using a new and more sensitive technique, revealed subclinical RV systolic dysfunction in c-SLE patients that may have future prognostic implications. The novel association of asymptomatic RV dysfunction with neuropsychiatric manifestations and antiphospholipid antibodies may suggest common physiopathological pathways.
- Immunogenicity and safety of two doses of a non-adjuvanted influenza A H1N1/2009 vaccine in young autoimmune rheumatic diseases patients(2013) AIKAWA, N. E.; TRUDES, G.; CAMPOS, L. M. A.; PEREIRA, R. M. R.; MORAES, J. C. B.; RIBEIRO, A. C.; MIRAGLIA, J.; TIMENETSKY, M. do Carmo S.; BONFA, E.; SILVA, C. A.Objectives The aim of this study was to evaluate the immunogenicity and safety of the influenza A H1N1/2009 vaccine in children under 9 years old with autoimmune rheumatic diseases (ARD). Methods Thirty-eight ARD patients and 11 healthy children received two doses of non-adjuvanted influenza A/California/7/2009 (H1N1) virus-like vaccine. Subjects were evaluated before and 21 days after vaccination. Seroprotection (SP) and seroconversion (SC) rates, geometric mean titers (GMT) and factor increases (FI) in GMT were calculated. Results Mean ages were comparable between patients and controls. Pre-vaccination SP and GMT were similar in patients and controls (p>0.05). Three weeks after immunization, SP (81.6% vs. 81.8%, p=1.0), SC (81.6% vs. 90.9%, p=0.66), GMT (151.5 vs. 282.1, p=0.26) and the FI in GMT (16.7 vs. 36.3, p=0.23) were similar in patients and controls, with both groups achieving an adequate response, according to the European Medicines Agency and Food and Drug Administration standards. Analysis of the possible factors influencing SC showed no difference in demographic data, leukocyte/lymphocyte counts or immunosuppressant use between seroconverted and non-seroconverted patients (p>0.05). The vaccine demonstrated a satisfactory safety profile in this population. Conclusions Two doses of influenza A H1N1/2009 vaccination induced an effective antibody response and caused adverse events in rare instances, suggesting this vaccine is appropriate and can be recommended for this age group.