Idiopathic Pulmonary Fibrosis, Today and Tomorrow: Certainties and New Therapeutic Horizons
Abstract Idiopathic pulmonary fibrosis (IPF) represents a clinical and therapeutic challenge characterized by progressive fibrosis and destruction of the lung architecture. The pathogenesis of IPF has been long debated; while it is generally believed that repeated lung injury and abnormal wound repa...
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| Format: | Article |
| Language: | English |
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Adis, Springer Healthcare
2025-05-01
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| Series: | Pulmonary Therapy |
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| Online Access: | https://doi.org/10.1007/s41030-025-00296-0 |
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| author | Giacomo Giulianelli Elisabetta Cocconcelli Giordano Fiorentù Nicol Bernardinello Elisabetta Balestro Paolo Spagnolo |
| author_facet | Giacomo Giulianelli Elisabetta Cocconcelli Giordano Fiorentù Nicol Bernardinello Elisabetta Balestro Paolo Spagnolo |
| author_sort | Giacomo Giulianelli |
| collection | DOAJ |
| description | Abstract Idiopathic pulmonary fibrosis (IPF) represents a clinical and therapeutic challenge characterized by progressive fibrosis and destruction of the lung architecture. The pathogenesis of IPF has been long debated; while it is generally believed that repeated lung injury and abnormal wound repair are the main pathogenetic mechanisms, clear understanding of disease development and efficacious treatment remain important unmet needs. Indeed, current standard of care (i.e., the antifibrotic drugs pirfenidone and nintedanib) can slow down lung function decline and disease progression without halting the disease. In the last 2 decades, several clinical trials in IPF have been completed mostly with negative results. Yet, unprecedented numbers of clinical trials of pharmacological interventions are currently being conducted. In this review, we summarize and critically discuss the current and future treatment landscape of IPF, with emphasis on the most promising developmental molecules. |
| format | Article |
| id | doaj-art-a7bff060c2eb4802a4435ed80bc1a48c |
| institution | Kabale University |
| issn | 2364-1754 2364-1746 |
| language | English |
| publishDate | 2025-05-01 |
| publisher | Adis, Springer Healthcare |
| record_format | Article |
| series | Pulmonary Therapy |
| spelling | doaj-art-a7bff060c2eb4802a4435ed80bc1a48c2025-08-20T03:48:18ZengAdis, Springer HealthcarePulmonary Therapy2364-17542364-17462025-05-0111219523410.1007/s41030-025-00296-0Idiopathic Pulmonary Fibrosis, Today and Tomorrow: Certainties and New Therapeutic HorizonsGiacomo Giulianelli0Elisabetta Cocconcelli1Giordano Fiorentù2Nicol Bernardinello3Elisabetta Balestro4Paolo Spagnolo5Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of PadovaRespiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of PadovaRespiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of PadovaRespiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of PadovaRespiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of PadovaRespiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of PadovaAbstract Idiopathic pulmonary fibrosis (IPF) represents a clinical and therapeutic challenge characterized by progressive fibrosis and destruction of the lung architecture. The pathogenesis of IPF has been long debated; while it is generally believed that repeated lung injury and abnormal wound repair are the main pathogenetic mechanisms, clear understanding of disease development and efficacious treatment remain important unmet needs. Indeed, current standard of care (i.e., the antifibrotic drugs pirfenidone and nintedanib) can slow down lung function decline and disease progression without halting the disease. In the last 2 decades, several clinical trials in IPF have been completed mostly with negative results. Yet, unprecedented numbers of clinical trials of pharmacological interventions are currently being conducted. In this review, we summarize and critically discuss the current and future treatment landscape of IPF, with emphasis on the most promising developmental molecules.https://doi.org/10.1007/s41030-025-00296-0Idiopathic pulmonary fibrosisIPFLung fibrosisInterstitial lung diseaseILDClinical trials |
| spellingShingle | Giacomo Giulianelli Elisabetta Cocconcelli Giordano Fiorentù Nicol Bernardinello Elisabetta Balestro Paolo Spagnolo Idiopathic Pulmonary Fibrosis, Today and Tomorrow: Certainties and New Therapeutic Horizons Pulmonary Therapy Idiopathic pulmonary fibrosis IPF Lung fibrosis Interstitial lung disease ILD Clinical trials |
| title | Idiopathic Pulmonary Fibrosis, Today and Tomorrow: Certainties and New Therapeutic Horizons |
| title_full | Idiopathic Pulmonary Fibrosis, Today and Tomorrow: Certainties and New Therapeutic Horizons |
| title_fullStr | Idiopathic Pulmonary Fibrosis, Today and Tomorrow: Certainties and New Therapeutic Horizons |
| title_full_unstemmed | Idiopathic Pulmonary Fibrosis, Today and Tomorrow: Certainties and New Therapeutic Horizons |
| title_short | Idiopathic Pulmonary Fibrosis, Today and Tomorrow: Certainties and New Therapeutic Horizons |
| title_sort | idiopathic pulmonary fibrosis today and tomorrow certainties and new therapeutic horizons |
| topic | Idiopathic pulmonary fibrosis IPF Lung fibrosis Interstitial lung disease ILD Clinical trials |
| url | https://doi.org/10.1007/s41030-025-00296-0 |
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