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2022-08-05

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cc-by-nc-nd (c) Molina Molina, María (et. al.), 2022
Please use this identifier to cite or link to this item: https://hdl.handle.net/2445/197433

Management of progressive pulmonary fibrosis associated with connective tissue disease

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https://doi.org/10.1080/17476348.2022.2107508

Abstract

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.

Subject

Càncer de pulmó, Malalties del teixit connectiu, Diagnòstic, Consulta mèdica, Fibrosi pulmonar, Tractament pal·liatiu

Subject (English)

Lung cancer, Connective tissues diseases, Diagnosis, Medical consultation, Pulmonary fibrosis, Palliative treatment

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Articles publicats en revistes (Fonaments Clínics)
Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer)
Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))

Citation

MOLINA MOLINA, María, et al. Management of progressive pulmonary fibrosis associated with connective tissue disease. Expert Review Of Respiratory Medicine. 2022. Vol. 16, num. 7, pags. 765-774. ISSN 1747-6348. [consulted: 8 of June of 2026]. Available at: https://hdl.handle.net/2445/197433

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