Esclerose sistémica - perspectivas actuais
Palavras-chave:
Esclerose sistémica, classificação, diagnóstico, tratamento, prognóstico, esclerodermiaResumo
A esclerose sistémica (ES) é uma doença relativamente rara, caracterizada por diferentes graus de fibrose da pele e dos órgãos internos. Pode ser dividida em dois grupos principais: ES limitada e ES difusa. Estes diferenciam-se, sobretudo, pela extensão do envolvimento cutâneo e caracterizam-se por evoluções e prognósticos diferentes. Ao longo dos anos ocorreram várias alterações a nível de conceitos relacionados com a ES. A uniformização destes é de grande importância, já que permite a realização de estudos em que se comparam grupos de doentes de centros diferentes, facilitando assim o melhor conhecimento desta entidade patológica. Apesar de a ES ser conhecida desde o século XVIII e ser reconhecida como patologia multissistémica desde o século XIX (antes considerava-se a esclerose cutânea – esclerodermia – como uma entidade patológica, a que se associavam outras doenças gastrointestinais, pulmonares, etc.), a sua etiologia continua a ser desconhecida e a sua patogénese não totalmente esclarecida. Isto leva a que não exista um tratamento específico e, portanto, curativo. Apesar disso, hoje já é possível controlar muitas das manifestações da ES. O aparecimento dos inibidores do enzima de conversão da angiotensina (IECA) na década de oitenta, por exemplo, permitiu controlar aquela que era até então a maior causa de morte na ES, a crise renal esclerodérmica. Este e outros novos avanços têm permitido melhorar a qualidade de vida destes doentes, bem como a sua esperança de vida. Neste trabalho abordam-se as perspectivas actuais de tratamento desta patologia e dos seus diferentes aspectos.
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