[Translated article] Diagnostic and Therapeutic Developments in Progressive Pulmonary Fibrosis |
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| Institution: | 1. Unidad Funcional de Intersticio Pulmonar (UFIP), Servicio de Neumología, Hospital Universitario de Bellvitge, UB, IDIBELL, Hospitalet de Llobregat, Barcelona, Spain;2. Centro de Investigación en Red de Enfermedades Respiratorias (CIBERES), Spain;3. Instituto Nacional de Enfermedades Respiratorias «Ismael Cosío Villegas», Ciudad de México, Mexico;4. Servicio de Neumología, Hospital de Sant Pau i Santa Creu, Barcelona, Spain;5. Enfermedades Pulmonares Intersticiales, Hospital de Rehabilitación Respiratoria María Ferrer, Buenos Aires, Argentina;6. Unidad de EPID, Servicio de Neumología, Hospital Universitario de la Princesa, Universidad Autónoma de Madrid, Madrid, Spain |
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| Abstract: | In addition to idiopathic pulmonary fibrosis (IPF), other diffuse interstitial lung diseases (ILD) are also associated with pulmonary fibrosis and occur in a variable proportion of patients, depending on the entity. The name given to this fibrotic component, that may progress despite treatment, is progressive pulmonary fibrosis (PPF). In this context, PPF is not an entity per se but a common clinical condition or behavior that may occur in association with different types of fibrosing diffuse ILDs, compromising patient prognosis. PPF is identified from worsening clinical, physiological, and/or radiological criteria during patient follow-up. Randomized clinical trials in patients with IPF or progressive non-IPF ILD have shown that treatment with antifibrotic drugs, either nintedanib or pirfenidone, slows progression. We are seeing the start of a new era in the clinical management of this subgroup of patients, offering the perfect opportunity for exploring still uncharted territories. |
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| Keywords: | Pulmonary fibrosis Progressive fibrosis Antifibrotics Fibrosis pulmonar Fibrosis progresiva Antifibrótico |
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