Systemic Sclerosis – Actual perspectives
Autor(a) principal: | |
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Data de Publicação: | 2002 |
Outros Autores: | , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | https://revista.spmi.pt/index.php/rpmi/article/view/1939 |
Resumo: | ystemic sclerosis is a relatively rare disease, characterized by variable degrees of fibrosis within the skin and internal organs. There are two major subsets, limited and diffuse systemic sclerosis, which are differentiated primarily by the extent of skin involvement, and are characterized by different disease evolution and prognosis. Concepts regarding this disease have changed over the years. Uniform concepts are extremely important, as they will allow comparison of groups of patients from different centres, and therefore facilitate a better knowledge of this disease.Although systemic sclerosis was first described in the 18th century and is recognized as a multi-systemic disease since the 19th century (before, cutaneous sclerosis – scleroderma – was considered as one disease and other diseases, such as gastrointestinal, pulmonary, etc., were considered to be associated with it), its’ aetiology remains unknown and its’ pathogenesis not entirely understood. This has resulted in the non-existence of a specific and therefore curative treatment. Despite this, advances have been made regarding the treatment of several of the disease manifestations. The discovery that angiotensin-converting enzyme (ACE) inhibitors, for instance are effective in the treatment of sclero-derma renal crisis, the most fatal complication of systemic sclerosis up till then. Such new advances have allowed improvement in the quality of life and the rate of survival of these patients. In this article, the new perspectives of treatment of this disease and its’ different aspects are discussed. |
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Systemic Sclerosis – Actual perspectivesEsclerose sistémica - perspectivas actuaisEsclerose sistémicaclassificaçãodiagnósticotratamentoprognósticoesclerodermiaSystemic sclerosissclerodermaclassificationdiagnosistreatmentprognosisystemic sclerosis is a relatively rare disease, characterized by variable degrees of fibrosis within the skin and internal organs. There are two major subsets, limited and diffuse systemic sclerosis, which are differentiated primarily by the extent of skin involvement, and are characterized by different disease evolution and prognosis. Concepts regarding this disease have changed over the years. Uniform concepts are extremely important, as they will allow comparison of groups of patients from different centres, and therefore facilitate a better knowledge of this disease.Although systemic sclerosis was first described in the 18th century and is recognized as a multi-systemic disease since the 19th century (before, cutaneous sclerosis – scleroderma – was considered as one disease and other diseases, such as gastrointestinal, pulmonary, etc., were considered to be associated with it), its’ aetiology remains unknown and its’ pathogenesis not entirely understood. This has resulted in the non-existence of a specific and therefore curative treatment. Despite this, advances have been made regarding the treatment of several of the disease manifestations. The discovery that angiotensin-converting enzyme (ACE) inhibitors, for instance are effective in the treatment of sclero-derma renal crisis, the most fatal complication of systemic sclerosis up till then. Such new advances have allowed improvement in the quality of life and the rate of survival of these patients. In this article, the new perspectives of treatment of this disease and its’ different aspects are discussed.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.Sociedade Portuguesa de Medicina Interna2002-06-28info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttps://revista.spmi.pt/index.php/rpmi/article/view/1939Internal Medicine; Vol. 9 No. 2 (2002): Abril/ Junho; 111-120Medicina Interna; Vol. 9 N.º 2 (2002): Abril/ Junho; 111-1202183-99800872-671Xreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPporhttps://revista.spmi.pt/index.php/rpmi/article/view/1939https://revista.spmi.pt/index.php/rpmi/article/view/1939/1355Vilas, Ana PaulaVeiga, Manuela ZitaAbecasis, Pedroinfo:eu-repo/semantics/openAccess2023-05-27T06:11:02Zoai:oai.revista.spmi.pt:article/1939Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T17:56:25.339551Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Systemic Sclerosis – Actual perspectives Esclerose sistémica - perspectivas actuais |
title |
Systemic Sclerosis – Actual perspectives |
spellingShingle |
Systemic Sclerosis – Actual perspectives Vilas, Ana Paula Esclerose sistémica classificação diagnóstico tratamento prognóstico esclerodermia Systemic sclerosis scleroderma classification diagnosis treatment prognosis |
title_short |
Systemic Sclerosis – Actual perspectives |
title_full |
Systemic Sclerosis – Actual perspectives |
title_fullStr |
Systemic Sclerosis – Actual perspectives |
title_full_unstemmed |
Systemic Sclerosis – Actual perspectives |
title_sort |
Systemic Sclerosis – Actual perspectives |
author |
Vilas, Ana Paula |
author_facet |
Vilas, Ana Paula Veiga, Manuela Zita Abecasis, Pedro |
author_role |
author |
author2 |
Veiga, Manuela Zita Abecasis, Pedro |
author2_role |
author author |
dc.contributor.author.fl_str_mv |
Vilas, Ana Paula Veiga, Manuela Zita Abecasis, Pedro |
dc.subject.por.fl_str_mv |
Esclerose sistémica classificação diagnóstico tratamento prognóstico esclerodermia Systemic sclerosis scleroderma classification diagnosis treatment prognosis |
topic |
Esclerose sistémica classificação diagnóstico tratamento prognóstico esclerodermia Systemic sclerosis scleroderma classification diagnosis treatment prognosis |
description |
ystemic sclerosis is a relatively rare disease, characterized by variable degrees of fibrosis within the skin and internal organs. There are two major subsets, limited and diffuse systemic sclerosis, which are differentiated primarily by the extent of skin involvement, and are characterized by different disease evolution and prognosis. Concepts regarding this disease have changed over the years. Uniform concepts are extremely important, as they will allow comparison of groups of patients from different centres, and therefore facilitate a better knowledge of this disease.Although systemic sclerosis was first described in the 18th century and is recognized as a multi-systemic disease since the 19th century (before, cutaneous sclerosis – scleroderma – was considered as one disease and other diseases, such as gastrointestinal, pulmonary, etc., were considered to be associated with it), its’ aetiology remains unknown and its’ pathogenesis not entirely understood. This has resulted in the non-existence of a specific and therefore curative treatment. Despite this, advances have been made regarding the treatment of several of the disease manifestations. The discovery that angiotensin-converting enzyme (ACE) inhibitors, for instance are effective in the treatment of sclero-derma renal crisis, the most fatal complication of systemic sclerosis up till then. Such new advances have allowed improvement in the quality of life and the rate of survival of these patients. In this article, the new perspectives of treatment of this disease and its’ different aspects are discussed. |
publishDate |
2002 |
dc.date.none.fl_str_mv |
2002-06-28 |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://revista.spmi.pt/index.php/rpmi/article/view/1939 |
url |
https://revista.spmi.pt/index.php/rpmi/article/view/1939 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
https://revista.spmi.pt/index.php/rpmi/article/view/1939 https://revista.spmi.pt/index.php/rpmi/article/view/1939/1355 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
Sociedade Portuguesa de Medicina Interna |
publisher.none.fl_str_mv |
Sociedade Portuguesa de Medicina Interna |
dc.source.none.fl_str_mv |
Internal Medicine; Vol. 9 No. 2 (2002): Abril/ Junho; 111-120 Medicina Interna; Vol. 9 N.º 2 (2002): Abril/ Junho; 111-120 2183-9980 0872-671X reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
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RCAAP |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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