Idiopathic pulmonary fibrosis: Pathogenesis and management

Giacomo Sgalla, Bruno Iovene, Mariarosaria Calvello, Margherita Ori, Francesco Varone, Luca Richeldi

Research output: Contribution to journalReview article

30 Citations (Scopus)

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.

Original languageEnglish
Article number32
JournalRespiratory Research
Volume19
Issue number1
DOIs
Publication statusPublished - Feb 22 2018

Fingerprint

Idiopathic Pulmonary Fibrosis
Lung
Therapeutics
Epigenomics
Pharmaceutical Preparations
Caregivers
Uncertainty
Cicatrix
Comorbidity
Patient Care
Chronic Disease
Biomarkers
Odds Ratio
Tomography
Morbidity
Biopsy
Wounds and Injuries
Genes

Keywords

  • Diagnosis
  • Idiopathic pulmonary fibrosis
  • Interstitial lung disease
  • Management
  • Nintedanib
  • Pathogenesis
  • Pirfenidone
  • Treatment

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Idiopathic pulmonary fibrosis : Pathogenesis and management. / Sgalla, Giacomo; Iovene, Bruno; Calvello, Mariarosaria; Ori, Margherita; Varone, Francesco; Richeldi, Luca.

In: Respiratory Research, Vol. 19, No. 1, 32, 22.02.2018.

Research output: Contribution to journalReview article

Sgalla, Giacomo ; Iovene, Bruno ; Calvello, Mariarosaria ; Ori, Margherita ; Varone, Francesco ; Richeldi, Luca. / Idiopathic pulmonary fibrosis : Pathogenesis and management. In: Respiratory Research. 2018 ; Vol. 19, No. 1.
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