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Cited by 573Interstitial Lung Diseases and Idiopathic Pulmonary FibrosisOccupational and environmental lung diseasesPulmonary Hypertension Research and Treatments

Idiopathic pulmonary fibrosis: pathogenesis and management

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Sgalla, Giacomo, Iovene, Bruno, Calvello, Mariarosaria, Ori, Margherita, Varone, Francesco, Richeldi, Luca·2018·Respiratory Research
DOI 10.1186/s12931-018-0730-2PMC PMC5824456
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Abstract

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive disease characterized by the aberrant accumulation of fibrotic tissue in the lungs parenchyma, associated with significant morbidity and poor prognosis. This review will present the substantial advances achieved in the understanding of IPF pathogenesis and in the therapeutic options that can be offered to patients, and will address the issues regarding diagnosis and management that are still open. MAIN BODY: Over the last two decades much has been clarified about the pathogenic pathways underlying the development and progression of the lung scarring in IPF. Sustained alveolar epithelial micro-injury and activation has been recognised as the trigger of several biological events of disordered repair occurring in genetically susceptible ageing individuals. Despite multidisciplinary team discussion has demonstrated to increase diagnostic accuracy, patients can still remain unclassified when the current diagnostic criteria are strictly applied, requiring the identification of a Usual Interstitial Pattern either on high-resolution computed tomography scan or lung biopsy. Outstanding achievements have been made in the management of these patients, as nintedanib and pirfenidone consistently proved to reduce the rate of progression of the fibrotic process. However, many uncertainties still lie in the correct use of these drugs, ranging from the initial choice of the drug, the appropriate timing for treatment and the benefit-risk ratio of a combined treatment regimen. Several novel compounds are being developed in the perspective of a more targeted therapeutic approach; in the meantime, the supportive care of these patients and their carers should be appropriately prioritized, and greater efforts should be made toward the prompt identification and management of relevant comorbidities. CONCLUSIONS: Building on the advances in the understanding of IPF pathobiology, the further investigation of the role of gene variants, epigenetic alterations and other molecular biomarkers reflecting disease activity and behaviour will hopefully enable earlier and more confident diagnosis, improve disease phenotyping and support the development of novel agents for personalized treatment of IPF.

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Cited by

Sorted by citations

Authors
Sgalla, G., Iovene, B., Calvello, M., Ori, M., Varone, F., & Richeldi, L.
Affiliations
  • Università Cattolica del Sacro Cuore (IT)
  • University of Modena and Reggio Emilia (IT)
Corresponding author
Sgalla, Giacomo
ORCID
Venue
Respiratory Research
Publisher
Springer Science and Business Media LLC
Published
2018-02-22
Volume
19
Issue
1
Pages
32-32
ISSN
  • 1465-9921 (ISSN-L)
  • 1465-993X
DOI
10.1186/s12931-018-0730-2
PMC
PMC5824456
OpenAlex
W2805771055
Cited by
573
References
15
Citations by year
2026: 33 · 2025: 70 · 2024: 108 · 2023: 84 · 2022: 93 · 2021: 87 · 2020: 55 · 2019: 31 · 2018: 10 · 2017: 1 · 2014: 1
FWCI
8.17
Type
journal article
Language
en
Open access
Licence
cc-by
Locations
  • Respiratory Research (publishedVersion, cc-by)
  • Europe PMC (PubMed Central) (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
Topics
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Occupational and environmental lung diseases
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
Keywords
idiopathic pulmonary fibrosis, pathogenesis, alveolar epithelial injury, pulmonary fibrosis, nintedanib, pirfenidone, antifibrotic therapy, high-resolution computed tomography, lung biopsy, comorbidities, supportive care, biomarkers, personalized treatment
MeSH terms
Anti-Inflammatory Agents, Non-Steroidal, Humans, Indoles, Pyridones, Tomography, X-Ray Computed, Disease Progression, Disease Management, Idiopathic Pulmonary Fibrosis

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