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cc-by-nc-nd (c) Molina Molina, María (et. al.), 2022
Si us plau utilitzeu sempre aquest identificador per citar o enllaçar aquest document: https://hdl.handle.net/2445/197433

Management of progressive pulmonary fibrosis associated with connective tissue disease

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Introduction: Fibrotic interstitial lung disease (ILD) is a frequent and severe complication of connective tissue disease (CTD). Areas covered: In this narrative review, we update the most relevant differential characteristics of fibrotic ILD associated with CTD (CTD-ILD) and propose a diagnostic and therapeutic approach based on a review of the articles published between 2002 and 2022 through PubMed. Expert opinion: The subset of ILD, mainly the radiological/histological pattern and the degree of fibrotic component, usually determines the prognosis and therapeutic strategy for these patients. Some patients with CTD-ILD can develop progressive pulmonary fibrosis (PPF) with severe deterioration of lung function, rapid progression to chronic respiratory failure, and high mortality. PPF has been described in many CTDs, mainly in systemic sclerosis and rheumatoid arthritis, and requires a multidisciplinary diagnostic and therapeutic approach to improve patient outcomes.

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MOLINA MOLINA, María, CASTELLVÍ, Ivan, VALENZUELA, Claudia, RAMÍREZ RUZ, J. (josé), RODRÍGUEZ PORTAL, Jose antonio, FRANQUET, Tomás, NARVÁEZ, Javier. Management of progressive pulmonary fibrosis associated with connective tissue disease. _Expert Review Of Respiratory Medicine_. 2022. Vol. 16, núm. 7, pàgs. 765-774. [consulta: 6 de febrer de 2026]. ISSN: 1747-6348. [Disponible a: https://hdl.handle.net/2445/197433]

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