Epidemiology and Prognosis of Progressive Pulmonary Fibrosis: A Literature Review
Abstract The spectrum of interstitial lung diseases (ILDs) includes a wide range of clinical entities with variable disease courses and prognoses. Several ILDs other than idiopathic pulmonary fibrosis (IPF) may exhibit a progressive fibrotic phenotype, with diverse clinical presentation, histopathol...
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| Format: | Article |
| Language: | English |
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Adis, Springer Healthcare
2025-06-01
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| Series: | Pulmonary Therapy |
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| Online Access: | https://doi.org/10.1007/s41030-025-00302-5 |
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| author | Ignacio Español Montero Fernanda Hernandez-Gonzalez Jacobo Sellares |
| author_facet | Ignacio Español Montero Fernanda Hernandez-Gonzalez Jacobo Sellares |
| author_sort | Ignacio Español Montero |
| collection | DOAJ |
| description | Abstract The spectrum of interstitial lung diseases (ILDs) includes a wide range of clinical entities with variable disease courses and prognoses. Several ILDs other than idiopathic pulmonary fibrosis (IPF) may exhibit a progressive fibrotic phenotype, with diverse clinical presentation, histopathological and radiological patterns, as well as varying rates of disease progression and uncertain epidemiology, but with a similar prognosis of untreated idiopathic pulmonary fibrosis with irreversible lung function deterioration, substantial worsening of quality of life and early mortality. The recently defined term “progressive pulmonary fibrosis” (PPF) stands as an opportunity to better classify patients with progressive fibrotic disease and other IPF, irrespective of the underlying ILD. The definition of disease progression, including factors such as pulmonary function test decline, radiological progression, and symptomatic worsening, was not adopted until recently, thus significantly impacting the certainty of current estimates of incidence and prevalence and prognostic outcomes. Understanding disease progression in the broad spectrum of potentially progressive ILDs is key for developing standardized management algorithms irrespective of the ILD diagnosis. Current evidence points towards the potential beneficial effect of antifibrotic drugs in lung function decline and overall outcomes in several non-IPF progressive ILDs showing progression despite optimal management. |
| format | Article |
| id | doaj-art-df5d80fade9f414a9fa6424f47ef7a55 |
| institution | Kabale University |
| issn | 2364-1754 2364-1746 |
| language | English |
| publishDate | 2025-06-01 |
| publisher | Adis, Springer Healthcare |
| record_format | Article |
| series | Pulmonary Therapy |
| spelling | doaj-art-df5d80fade9f414a9fa6424f47ef7a552025-08-24T11:42:35ZengAdis, Springer HealthcarePulmonary Therapy2364-17542364-17462025-06-0111334736310.1007/s41030-025-00302-5Epidemiology and Prognosis of Progressive Pulmonary Fibrosis: A Literature ReviewIgnacio Español Montero0Fernanda Hernandez-Gonzalez1Jacobo Sellares2Pneumology and Respiratory Allergy Department, Hospital Clinic de Barcelona-IDIBAPSPneumology and Respiratory Allergy Department, Hospital Clinic de Barcelona-IDIBAPSPneumology and Respiratory Allergy Department, Hospital Clinic de Barcelona-IDIBAPSAbstract The spectrum of interstitial lung diseases (ILDs) includes a wide range of clinical entities with variable disease courses and prognoses. Several ILDs other than idiopathic pulmonary fibrosis (IPF) may exhibit a progressive fibrotic phenotype, with diverse clinical presentation, histopathological and radiological patterns, as well as varying rates of disease progression and uncertain epidemiology, but with a similar prognosis of untreated idiopathic pulmonary fibrosis with irreversible lung function deterioration, substantial worsening of quality of life and early mortality. The recently defined term “progressive pulmonary fibrosis” (PPF) stands as an opportunity to better classify patients with progressive fibrotic disease and other IPF, irrespective of the underlying ILD. The definition of disease progression, including factors such as pulmonary function test decline, radiological progression, and symptomatic worsening, was not adopted until recently, thus significantly impacting the certainty of current estimates of incidence and prevalence and prognostic outcomes. Understanding disease progression in the broad spectrum of potentially progressive ILDs is key for developing standardized management algorithms irrespective of the ILD diagnosis. Current evidence points towards the potential beneficial effect of antifibrotic drugs in lung function decline and overall outcomes in several non-IPF progressive ILDs showing progression despite optimal management.https://doi.org/10.1007/s41030-025-00302-5Progressive pulmonary fibrosisInterstitial lung diseasesAntifibrotic therapyDisease progression |
| spellingShingle | Ignacio Español Montero Fernanda Hernandez-Gonzalez Jacobo Sellares Epidemiology and Prognosis of Progressive Pulmonary Fibrosis: A Literature Review Pulmonary Therapy Progressive pulmonary fibrosis Interstitial lung diseases Antifibrotic therapy Disease progression |
| title | Epidemiology and Prognosis of Progressive Pulmonary Fibrosis: A Literature Review |
| title_full | Epidemiology and Prognosis of Progressive Pulmonary Fibrosis: A Literature Review |
| title_fullStr | Epidemiology and Prognosis of Progressive Pulmonary Fibrosis: A Literature Review |
| title_full_unstemmed | Epidemiology and Prognosis of Progressive Pulmonary Fibrosis: A Literature Review |
| title_short | Epidemiology and Prognosis of Progressive Pulmonary Fibrosis: A Literature Review |
| title_sort | epidemiology and prognosis of progressive pulmonary fibrosis a literature review |
| topic | Progressive pulmonary fibrosis Interstitial lung diseases Antifibrotic therapy Disease progression |
| url | https://doi.org/10.1007/s41030-025-00302-5 |
| work_keys_str_mv | AT ignacioespanolmontero epidemiologyandprognosisofprogressivepulmonaryfibrosisaliteraturereview AT fernandahernandezgonzalez epidemiologyandprognosisofprogressivepulmonaryfibrosisaliteraturereview AT jacobosellares epidemiologyandprognosisofprogressivepulmonaryfibrosisaliteraturereview |