CLOVIS ARTUR ALMEIDA DA SILVA
Projetos de Pesquisa
Unidades Organizacionais
Departamento de Pediatria, Faculdade de Medicina - Docente
Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina
LIM/36 - Laboratório de Pediatria Clínica, Hospital das Clínicas, Faculdade de Medicina - Líder
Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina
LIM/36 - Laboratório de Pediatria Clínica, Hospital das Clínicas, Faculdade de Medicina - Líder
40 resultados
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- Health related quality of life measure in systemic pediatric rheumatic diseases and its translation to different languages: an international collaboration(2014) MOORTHY, Lakshmi Nandini; ROY, Elizabeth; KURRA, Vamsi; PETERSON, Margaret G. E.; HASSETT, Afton L.; LEHMAN, Thomas J. A.; SCOT, Christiaan; EL-GHONEIMY, Dalia; SAAD, Shereen; FEKY, Reem El; AL-MAYOUF, Sulaiman; DOLEZALOVA, Pavla; MALCOVA, Hana; HERLIN, Troels; NIELSEN, Susan; WULFFRAAT, Nico; ROYEN, Annet van; MARKS, Stephen D.; BELOT, Alexandre; BRUNNER, Jurgen; HUEMER, Christian; FOELDVARI, Ivan; HORNEFF, Gerd; SAURENMAN, Traudel; SCHROEDER, Silke; PRATSIDOU-GERTSI, Polyxeni; TRACHANA, Maria; UZIEL, Yosef; AGGARWAL, Amita; CONSTANTIN, Tamas; CIMAZ, Rolando; GIANI, Theresa; CANTARINI, Luca; FALCINI, Fernanda; MANZONI, Silvia Magni; RAVELLI, Angelo; RIGANTE, Donato; ZULIAN, Fracnceso; MIYAMAE, Takako; YOKOTA, Shumpei; SATO, Juliana; MAGALHAES, Claudia S.; LEN, Claudio A.; APPENZELLER, Simone; KNUPP, Sheila Oliveira; RODRIGUES, Marta Cristine; SZTAJNBOK, Flavio; ALMEIDA, Rozana Gasparello de; JESUS, Adriana Almeida de; CAMPOS, Lucia Maria de Arruda; SILVA, Clovis; LAZAR, Calin; SUSIC, Gordana; AVCIN, Tadej; CUTTICA, Ruben; BURGOS-VARGAS, Ruben; FAUGIER, Enrique; ANTON, Jordi; MODESTO, Consuelo; VAZQUEZ, Liza; BARILLAS, Lilliana; BARINSTEIN, Laura; STERBA, Gary; MALDONADO, Irama; OZEN, Seza; KASAPCOPUR, Ozgur; DEMIRKAYA, Erkan; BENSELER, SusaBackground: Rheumatic diseases in children are associated with significant morbidity and poor health-related quality of life (HRQOL). There is no health-related quality of life (HRQOL) scale available specifically for children with less common rheumatic diseases. These diseases share several features with systemic lupus erythematosus (SLE) such as their chronic episodic nature, multi-systemic involvement, and the need for immunosuppressive medications. HRQOL scale developed for pediatric SLE will likely be applicable to children with systemic inflammatory diseases. Findings: We adapted Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY (c)) to Simple Measure of Impact of Illness in Youngsters (SMILY (c)-Illness) and had it reviewed by pediatric rheumatologists for its appropriateness and cultural suitability. We tested SMILY (c)-Illness in patients with inflammatory rheumatic diseases and then translated it into 28 languages. Nineteen children (79% female, n= 15) and 17 parents participated. The mean age was 12 +/- 4 years, with median disease duration of 21 months (1-172 months). We translated SMILY (c)-Illness into the following 28 languages: Danish, Dutch, French (France), English (UK), German (Germany), German (Austria), German (Switzerland), Hebrew, Italian, Portuguese (Brazil), Slovene, Spanish (USA and Puerto Rico), Spanish (Spain), Spanish (Argentina), Spanish (Mexico), Spanish (Venezuela), Turkish, Afrikaans, Arabic (Saudi Arabia), Arabic (Egypt), Czech, Greek, Hindi, Hungarian, Japanese, Romanian, Serbian and Xhosa. Conclusion: SMILY (c)-Illness is a brief, easy to administer and score HRQOL scale for children with systemic rheumatic diseases. It is suitable for use across different age groups and literacy levels. SMILY (c)-Illness with its available translations may be used as useful adjuncts to clinical practice and research.
conferenceObject Identification of ""autoinflammatory interferonopathies""? a New Class of Autoinflammatory Conditions?(2014) JESUS, Adriana Almeida de; DENG, Zuoming; BROOKS, Stephen; LIU, Yin; KIM, Hanna; SANCHEZ, Gina A. Montealegre; CHAPELLE, Dawn C.; HUANG, Yan; HASHKES, Philip; NASRULLAYEVA, Gulnara; TERRERI, Maria Teresa; ARABSHAHI, Bita; PUNARO, Marilynn G.; MOORTHY, Lakshmi N.; REINHARDT, Adam; SILVA, Clovis A.; SATO, Emilia I.; LILLEBY, Vibke; FLEISHER, Thomas; GOLDBACH-MANSKY, Raphaela- A novel mutation of IL1RN in the deficiency of interleukin-1 receptor antagonist syndrome: Description of two unrelated cases from Brazil(2011) JESUS, Adriana A.; OSMAN, Mazen; SILVA, Clovis A.; KIM, Peter W.; Tuyet-Hang Pham; GADINA, Massimo; YANG, Barbara; BERTOLA, Debora R.; CARNEIRO-SAMPAIO, Magda; FERGUSON, Polly J.; RENSHAW, Blair R.; SCHOOLEY, Ken; BROWN, Michael; AL-DOSARI, Asma; AL-ALAMI, Jamil; SIMS, John E.; GOLDBACH-MANSKY, Raphaela; EL-SHANTI, HatemObjective Monogenic autoinflammatory diseases are disorders of Mendelian inheritance that are characterized by mutations in genes that regulate innate immunity and whose typical features are systemic inflammation without high-titer autoantibodies or antigen-specific T cells. Skin and bone inflammation in the newborn period have been described in 3 of these autoinflammatory disorders: neonatal-onset multisystem inflammatory disease, Majeed syndrome, and deficiency of interleukin-1 (IL-1) receptor antagonist (DIRA) syndrome. This study was undertaken to present the characteristics of the DIRA syndrome in 2 cases from Brazil, and describe a novel mutation in IL1RN. Methods. Two unrelated Brazilian patients were evaluated for the clinical signs and symptoms of these 3 disorders, and peripheral blood samples were assessed for mutations in NLRP3, LPIN2, and IL1RN by DNA resequencing analysis. A mutation in IL1RN that encodes a mutant protein was identified, and the expression and function of this mutant protein were compared to those of the wild-type protein. Results. Both patients presented with pustular dermatitis resembling generalized pustular psoriasis, recurrent multifocal aseptic osteomyelitis, and elevation in the levels of acute-phase reactants, all of which are features most consistent with the DIRA syndrome. Chronic lung disease was observed in 1 of the patients, and jugular venous thrombosis was observed in the other patient. Both patients showed a partial response to corticosteroid therapy, and 1 patient experienced an initial improvement of dermatitis with the use of acitretin. Both patients were homozygous for a novel 15-bp (in-frame) deletion on the IL1RN gene. The mutated protein expressed in vitro had no affinity with the IL-1 receptor, and stimulation of the patients' cells with recombinant human IL-1 alpha or IL-1 alpha led to oversecretion of proinflammatory cytokines, similar to the findings obtained in previously reported patients. Conclusion. The presence of the same homozygous novel mutation in IL1RN in 2 unrelated Brazilian patients suggests that this genetic variant may be a founder mutation that has been introduced in the Brazilian population.
- International Consensus for the Dosing of Corticosteroids in Childhood-Onset Systemic Lupus Erythematosus With Proliferative Lupus Nephritis(2022) CHALHOUB, Nathalie E.; WENDERFER, Scott E.; LEVY, Deborah M.; ROUSTER-STEVENS, Kelly; AGGARWAL, Amita; I, Sonia Savani; RUTH, Natasha M.; ARKACHAISRI, Thaschawee; QIU, Tingting; MERRITT, Angela; ONEL, Karen; GOILAV, Beatrice; KHUBCHANDANI, Raju P.; DENG, Jianghong; FONSECA, Adriana R.; ARDOIN, Stacy P.; CIURTIN, Coziana; KASAPCOPUR, Ozgur; JELUSIC, Marija; HUBER, Adam M.; OZEN, Seza; KLEIN-GITELMAN, Marisa S.; APPENZELLER, Simone; CAVALCANTI, Andre; FOTIS, Lampros; LIM, Sern Chin; SILVA, Rodrigo M.; RAMIREZ-MIRAMONTES, Julia; ROSENWASSER, Natalie L.; SAAD-MAGALHAES, Claudia; SCHONENBERG-MEINEMA, Dieneke; SCOTT, Christiaan; SILVA, Clovis A.; ENCISO, Sandra; TERRERI, Maria T.; TORRES-JIMENEZ, Alfonso-Ragnar; TRACHANA, Maria; AL-MAYOUF, Sulaiman M.; DEVARAJAN, Prasad; HUANG, Bin; I, Hermine BrunnerObjective To develop a standardized steroid dosing regimen (SSR) for physicians treating childhood-onset systemic lupus erythematosus (SLE) complicated by lupus nephritis (LN), using consensus formation methodology. Methods Parameters influencing corticosteroid (CS) dosing were identified (step 1). Data from children with proliferative LN were used to generate patient profiles (step 2). Physicians rated changes in renal and extrarenal childhood-onset SLE activity between 2 consecutive visits and proposed CS dosing (step 3). The SSR was developed using patient profile ratings (step 4), with refinements achieved in a physician focus group (step 5). A second type of patient profile describing the course of childhood-onset SLE for >= 4 months since kidney biopsy was rated to validate the SSR-recommended oral and intravenous (IV) CS dosages (step 6). Patient profile adjudication was based on majority ratings for both renal and extrarenal disease courses, and consensus level was set at 80%. Results Degree of proteinuria, estimated glomerular filtration rate, changes in renal and extrarenal disease activity, and time since kidney biopsy influenced CS dosing (steps 1 and 2). Considering these parameters in 5,056 patient profile ratings from 103 raters, and renal and extrarenal course definitions, CS dosing rules of the SSR were developed (steps 3-5). Validation of the SSR for up to 6 months post-kidney biopsy was achieved with 1,838 patient profile ratings from 60 raters who achieved consensus for oral and IV CS dosage in accordance with the SSR (step 6). Conclusion The SSR represents an international consensus on CS dosing for use in patients with childhood-onset SLE and proliferative LN. The SSR is anticipated to be used for clinical care and to standardize CS dosage during clinical trials.
- 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.
conferenceObject Ultrasonography of major salivary glands in juvenile Sjogren's syndrome: an international multicentre study(2018) HAMMENFORS, Daniel S.; VALIM, Valeria; BICA, Blanca E. R. G.; PASOTO, Sandra G.; LILLEBY, Vibke; NIETO-GONZALEZ, Juan Carlos; SILVA, Clovis A.; MOSSEL, Esther; PEREIRA, Rosa M. R.; COELHO, Aline; BOOTSMA, Hendrika; THATAYATIKOM, Akaluck; BRUN, Johan G.; JONSSON, Roland; JONSSON, Malin V.conferenceObject Development and Initial Validation of the MS Score for Diagnosis of Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis(2019) MINOIA, Francesca; BOVIS, Francesca; DAVI, Sergio; HORNE, AnnaCarin; FISCHBACH, Michel; FROSCH, Michael; HUBER, Adam; JELUSIC, Marija; SAWHNEY, Sujata; MCCURDY, Deborah; SILVA, Clovis Artur; RIGANTE, Donato; UNSAL, Erbil; RUPERTO, Nicolino; MARTINI, Alberto; CRON, Randy; RAVELLI, Angelo- Hereditary Autoinflammatory Syndromes: A Brazilian Multicenter Study(2012) JESUS, Adriana A.; FUJIHIRA, Erika; WATASE, Mariana; TERRERI, Maria T.; HILARIO, Maria O.; CARNEIRO-SAMPAIO, Magda; LEN, Claudio A.; OLIVEIRA, Sheila K.; RODRIGUES, Marta C.; PEREIRA, Rosa M.; BICA, Blanca; SILVA, Nilzio A.; CAVALCANTI, Andre; MARINI, Roberto; SZTAJNBOK, Flavio; QUINTERO, Maria V.; FERRIANI, Virginia P.; MORAES-VASCONCELOS, Dewton; SILVA, Clovis A.; OLIVEIRA, Joao B.To evaluate the prevalence of genetic defects in clinically suspected autoinflammatory syndromes (AIS) in a Brazilian multicenter study. The study included 102 patients with a clinical diagnosis of Cryopyrin Associated Periodic Syndromes (CAPS), TNF Receptor Associated Periodic Syndrome (TRAPS), Familial Mediterranean Fever (FMF), Mevalonate Kinase Deficiency (MKD) and Pediatric Granulomatous Arthritis (PGA). One of the five AIS-related genes (NLRP3, TNFRSF1A, MEFV, MVK and NOD2) was evaluated in each patient by direct DNA sequencing, based on the most probable clinical suspect. Clinical diagnoses of the 102 patients were: CAPS (n = 28), TRAPS (n = 31), FMF (n = 17), MKD (n = 17) and PGA (n = 9). Of them, 27/102 (26 %) had a confirmed genetic diagnosis: 6/28 (21 %) CAPS patients, 7/31 (23 %) TRAPS, 3/17 (18 %) FMF, 3/17 (18 %) MKD and 8/9 (89 %) PGA. We have found that approximately one third of the Brazilian patients with a clinical suspicion of AIS have a confirmed genetic diagnosis.
- Pregnancy and reproduction in autoimmune rheumatic diseases(2011) OSTENSEN, Monika; BRUCATO, Antonio; CARP, Howard; CHAMBERS, Christina; DOLHAIN, Radboud J. E. M.; DORIA, Andrea; FOERGER, Frauke; GORDON, Caroline; HAHN, Sinuhe; KHAMASHTA, Munther; LOCKSHIN, Michael D.; MATUCCI-CERINIC, Marco; MERONI, Pierluigi; NELSON, J. Lee; PARKE, Ann; PETRI, Michelle; RAIO, Luigi; RUIZ-IRASTORZA, Guillermo; SILVA, Clovis A.; TINCANI, Angela; VILLIGER, Peter M.; WUNDER, Dorothea; CUTOLO, MaurizioDespite evidence for the important role of oestrogens in the aetiology and pathophysiology of chronic immune/inflammatory diseases, the previous view of an unequivocal beneficial effect of oestrogens on RA compared with a detrimental effect on SLE has to be reconsidered. Likewise, the long-held belief that RA remits in the majority of pregnant patients has been challenged, and shows that only half of the patients experience significant improvement when objective disease activity measurements are applied. Pregnancies in patients with SLE are mostly successful when well planned and monitored interdisciplinarily, whereas a small proportion of women with APS still have adverse pregnancy outcomes in spite of the standard treatment. New prospective studies indicate better outcomes for pregnancies in women with rare diseases such as SSc and vasculitis. Fertility problems are not uncommon in patients with rheumatic disease and need to be considered in both genders. Necessary therapy, shortly before or during the pregnancy, demands taking into account the health of both mother and fetus. Long-term effects of drugs on offspring exposed in utero or during lactation is a new area under study as well as late effects of maternal rheumatic disease on children.
- CANDLE syndrome: chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature-a rare case with a novel mutation(2016) CAVALCANTE, Miria Paula V.; BRUNELLI, Juliana B.; MIRANDA, Clarissa C.; NOVAK, Glaucia V.; MALLE, Louise; AIKAWA, Nadia E.; JESUS, Adriana A.; SILVA, Clovis ArturWe described herein a patient with chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome and a novel mutation in PSMB8 gene. This patient had multiple visceral inflammatory involvements, including rare manifestations, such as Sweet syndrome and pericarditis. A 3-year-old male, Caucasian, was born to consanguineous healthy parents. At the age of 11 months, he presented daily fever (temperature > 40 A degrees C), irritability, hepatomegaly, splenomegaly; and tender and itching, erythematous papular and edematous plaque lesions. Echocardiogram showed mild pericarditis. Skin biopsy revealed a neutrophil infiltrate without vasculitis suggesting Sweet syndrome. Mutational screening of PSMB8 gene revealed homozygous c.280G > C, p.A94P mutation. He responded partially to high doses of oral glucorticoid and intravenous methylprednisolone. Colchicine, azathioprine, methotrexate, cyclosporine, and intravenous immunoglobulin were not efficacious. At the age of 3 years and 1 month, tocilizumab was administered resulting in remission of daily fever and irritability. However, there was no improvement of the skin tenderness and itching lesions. Conclusion: A new mutation in a CANDLE syndrome patient was reported with pericarditis and mimicking Sweet syndrome. The disease manifestations were refractory to immunosuppressive agents and partially responsive to tocilizumab therapy.