Please use this identifier to cite or link to this item: http://repositorio.unicamp.br/jspui/handle/REPOSIP/313858
Type: TESE
Title: Miastenia grave autoimune do adulto : experiencia de 20 anos do HC - FCM - UNICAMP
Author: Carandina-Maffeis, Rosana
Advisor: Nucci, Anamarli, 1947-
Abstract: Resumo: A miastenia grave (MG) é a doença autoimune melhor compreendida. Assume grande importância por suas manifestações clínicas de morbi-mortalidade, as quais declinaram com o maior conhecimento fisiopatológico da doença nas últimas décadas . Analisou-se retrospectivamente os prontuários dos pacientes adultos com MG autoimune do Hospital de Clínicas da Faculdade de Ciências Médicas da Universidade Estadual de Campinas ( HC - FCM - UNICAMP ), acompanhados no período de 1982 a 2001. Enfocou-se dados de apresentação clínica, exames complementares, tratamento e evolução. Medidas de significância estatística foram realizadas por teste do Qui-Quadrado ou teste exato de Fisher quando indicado, com significância quando p < = 0,05; e por análise de regressão logística multivariada para resposta dicotômica. Foram estudados 127 casos, 71% mulheres e 29% homens, com média de idade de início da MG de 36,95 e mediana de 32 anos. Doenças autoimunes associadas estavam presentes em 19,68%. Na classificação de Osserman e Genkins ( O e G ) 17,3% foram grau I, 42,5% IIa, 29,9% IIb, 7,1% III e 3,1% IV . Um primeiro teste de estimulação nervosa repetitiva ( TER ) evidenciou decremento > 10% em 69,57%, e em 25% dos casos grau I. Anticorpos anti receptor de acetilcolina (AcARACo ), fração ligador, foram positivos em 67,24% dos casos submetidos ao exame. A tomografia computadorizada ( TC ) de mediastino mostrou uma sensibilidade de no máximo 75% para detecção de timoma e especificidade de 67,92%. O exame anátomo patológico evidenciou 16% de timoma. A timectomia como parte da terapêutica foi realizada em 59% dos casos. Anti-colinesterásico foi utilizado em 96,9%, prednisona em 86%, outros imunossupressores em 35%, plasmaférese aguda em 20% e a imunoglobulina (IgIV) aguda em 3,93%. Na evolução 29,13% apresentaram remissão completa, 7,09% remissão farmacológica, 53,54% melhora, 4,72% ficaram inalterados, 0,79% (um caso) piora, 2,36% exacerbação e 2,36% óbito relacionado a MG. Entre os fatores associados com remissão completa mostrou-se significante a realização de timectomia (p=0,0002) e com evolução não favorável o sexo masculino (p=0,0469). Óbitos pela MG foram associados a uma pior classificação de O e G ( graus III e IV )

Abstract: Introduction: Myasthenia gravis (MG) is the best known autoimmune diseased. There is very importance due to its morbidity and mortality, with improvement in the treatment in the last years. Objectives: to analyze retrospectively the experience of UNICAMP University Hospital about MGAA. Casuistics and Methods: to review retrospectively the charts of adults myasthenic patients attended in Unicamp University Hospital and that were followed in a regular bases during the period 1982-2001. It was analysed the clinical classification, laboratory examinations (repetitive nerve stimulation; acetylcholine receptor antibodies; CT or MRI images of mediastinum and histopathological exams of surgical removed material), treatment and outcome. Variables were analyzed by Chi ¿ square or Fisher' s exact test ( probability ¿ p < = 0,05 was considered significant ), and by multivariance regression analysis. Results: 127 patients fulfilled criteria for MGAA , 71% were females and 29% males; 87,4% white and 11,81% black. The mean age at the beginning of MG was 36,95 years, median 32 years, extremes 19 and 71 years. Another autoimmune disease was present in 19,68%. Osserman-Genkins scale classified 17,3% as grade I, 42,5% as IIa, 29,9% as IIb, 7,1% as III and 3,1% as grade IV. Repetitive nerve stimulation was positive in 69,57%, and only in 25% grade I. Acetylcholine receptor antibodies was positive in 67,24% from the cases that performed the exam. CT images of mediastinum showed 75% sensibility for diagnosis of thymoma and 67,92% specificity. Thymic histopathology revealed thymoma in 16%. Seventy five patients (59%) were submitted to thymectomy. The treatment with anticholinesterasic agents was prescribed for 96,9%, prednisone for 86%, and another immunossupressor for 35%. Plasmapheresis was indicated in 20% and intravenous immunoglobulin in 3,93%. Evolution: 29,13% was in complete remission, 7,09% in pharmacologic remission, 53,54% were considered improved, 4,72% had unchanged status, 0,79% (one case) worse, 2,36% exacerbation and 2,36% died of MG. Thymectomy associated to complete remission was considered significant (p=0,0002), and males were associated to unfavorable evolution (p=0,0469). Death of MG was associated to a worse clinical classification on Osserman-Genkins scale. Conclusions: One hundred and Twenty seven pacients with MGAA were followed; and clinical presentation range from ocular MG to severe MG, but mild generalized MG predominated. The treatment considered drugs and thymectomy. A favorable evolution was observed in 90% of cases
Subject: Junção neuromuscular
Language: Português
Editor: [s.n.]
Date Issue: 2003
Appears in Collections:FCM - Tese e Dissertação

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