Management of progressive pulmonary fibrosis associated with connective tissue disease
Por:
Molina-Molina, M, Castellvi, I, Valenzuela, C, Ramirez, J, Portal, JAR, Franquet, T, Narvaez, J
Publicada:
1 ago 2022
Ahead of Print:
1 ago 2022
Resumen:
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.
Filiaciones:
Molina-Molina, M:
Hosp Bellvitge Princeps Espanya, Serv Neumol, IDIBELL, Barcelona, Spain
Castellvi, I:
Hosp Santa Creu & Sant Pau, Serv Reumatol, Barcelona, Spain
Valenzuela, C:
Hosp La Princesa, Serv Neumol, Madrid, Spain
Ramirez, J:
Univ Barcelona, Hosp Clin, Serv Anat Patol, CIBERES,IDIBAPS, Barcelona, Spain
Portal, JAR:
Hosp Univ Virgen del Rocio, Serv Neumol, Seville, Spain
Franquet, T:
Univ Autonoma Barcelona, Hosp Santa Creu & St Pau, Serv Radiol, Barcelona, Spain
Narvaez, J:
Hosp Bellvitge Princeps Espanya, Serv Reumatol, IDIBELL, Red Invest Inflamac & Enfermedades Reumat, Barcelona, Spain
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