RT Journal Article
T1 Management of progressive pulmonary fibrosis associated with connective tissue disease.
A1 Molina-Molina, María
A1 Castellví, Iván
A1 Valenzuela, Claudia
A1 Ramirez, José
A1 Rodríguez Portal, José Antonio
A1 Franquet, Tomás
A1 Narváez, Javier
K1 Interstitial lung disease
K1 connective tissue disease
K1 diagnosis
K1 multidisciplinary consultation
K1 progressive pulmonary fibrosis
K1 treatment
AB Fibrotic interstitial lung disease (ILD) is a frequent and severe complication of connective tissue disease (CTD). 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. 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.
YR 2022
FD 2022-08-05
LK http://hdl.handle.net/10668/19692
UL http://hdl.handle.net/10668/19692
LA en
DS RISalud
RD Apr 19, 2025