Management of progressive pulmonary fibrosis associated with connective tissue disease
Molina-Molina, María (Institut d'Investigació Biomèdica de Bellvitge)
Castellvi, Ivan (Institut d'Investigació Biomèdica Sant Pau)
Valenzuela, Claudia (Hospital La Princesa. Servicio de Neumología)
Ramirez, José (Institut d'Investigacions Biomèdiques August Pi i Sunyer)
Rodríguez Portal, José Antonio (Hospital Universitario Virgen del Rocío (Sevilla, Andalusia))
Franquet, Tomas (Institut d'Investigació Biomèdica Sant Pau)
Narváez, Javier (Institut d'Investigació Biomèdica de Bellvitge)
Universitat Autònoma de Barcelona

Date:	2022
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.
Note:	Altres ajuts: Roche; Boehringer Ingelheim; Pfizer.
Rights:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, i la comunicació pública de l'obra, sempre que no sigui amb finalitats comercials, i sempre que es reconegui l'autoria de l'obra original. No es permet la creació d'obres derivades.
Language:	Anglès
Document:	Article de revisió ; recerca ; Versió publicada
Subject:	Interstitial lung disease ; Connective tissue disease ; Progressive pulmonary fibrosis ; Multidisciplinary consultation ; Diagnosis ; Treatment
Published in:	Expert Review of Respiratory Medicine, Vol. 16 Núm. 7 (2022) , p. 765-774, ISSN 1747-6356

DOI: 10.1080/17476348.2022.2107508
PMID: 35912842

11 p, 1.5 MB

The record appears in these collections:
Research literature > UAB research groups literature > Research Centres and Groups (research output) > Health sciences and biosciences > Institut de Recerca Sant Pau
Articles > Research articles
Articles > Published articles