Idiopathic Pulmonary Arterial Hypertension

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art_SOUZA_Idiopathic_Pulmonary_Arterial_Hypertension_2013.PDF (223.6 KB)
Citações na Scopus
11
Tipo de produção
article
Data de publicação
2013
DOI
Scopus
Web of Science
PubMed
Título da Revista
ISSN da Revista
Título do Volume
Editora
THIEME MEDICAL PUBL INC
Autores
HUMBERT, Marc
Citação
SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE, v.34, n.5, p.560-567, 2013
Projetos de Pesquisa
Unidades Organizacionais
Fascículo
Resumo
Idiopathic pulmonary arterial hypertension (IPAH), formerly called primary pulmonary hypertension, is a rare disease (incidence and prevalence rates of approximately one and six cases per million inhabitants, respectively) with different clinical phenotypes. A group of diverse conditions manifest pulmonary arterial hypertension (PAH) and share similar pathological and/or clinical findings with IPAH. By definition, IPAH is diagnosed only after alternative diagnoses have been ruled out. Extensive investigation is needed to determine if PAH is associated with thyroid diseases, infectious diseases, autoimmune conditions, exposure to certain drugs (particularly anorexigens), certain genetic mutations, and so on. The presence of genetic abnormalities and risk factors (such as specific drug exposures) reinforces the ""multiple hit"" concept for the development of pulmonary hypertension. Fortunately, within the past two decades, therapeutic options have become available for IPAH, resulting in improved survival and clinical outcomes. At least seven different compounds have been registered for PAH treatment. However, even with aggressive PAH-specific therapy, mortality rates remain high (similar to 40% at 5 years). Given the high mortality rates, the use of combinations of agents that work by different pathways has been advocated (either as ""add-on"" therapy or initial ""up front"" therapy). Further, new therapeutic agents and treatment strategies are on the near horizon, aiming to further improve survival from the remarkable progress already seen.
Palavras-chave
idiopathic pulmonary arterial hypertension, pulmonary arterial hypertension, heritable PAH, epidemiology
URI
https://observatorio.fm.usp.br/handle/OPI/3957
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Coleções
Artigos e Materiais de Revistas Científicas - FM/MCP
Artigos e Materiais de Revistas Científicas - HC/ICHC
Artigos e Materiais de Revistas Científicas - HC/InCor
Artigos e Materiais de Revistas Científicas - LIM/09
Artigos e Materiais de Revistas Científicas - ODS/03