Details

Autor(en) / Beteiligte

• Koudstaal, Thomas
• Funke-Chambour, Manuela
• Kreuter, Michael
• Molyneaux, Philip L.
• Wijsenbeek, Marlies S.

Titel

Pulmonary fibrosis: from pathogenesis to clinical decision-making

Ist Teil von

• Trends in molecular medicine, 2023-12, Vol.29 (12), p.1076-1087

Ort / Verlag

Elsevier Ltd

Erscheinungsjahr

2023

Quelle

Access via ScienceDirect (Elsevier)

Beschreibungen/Notizen

• Pulmonary fibrosis (PF) is a basket term for a spectrum of fibrotic lung diseases.Idiopathic pulmonary fibrosis (IPF) is the most prevalent and progressive form of PF with high symptom burden and poor prognosis.Other forms of PF often occur in the context of autoimmune diseases or exposures. It is estimated that 30–50% of these patients manifest progressive pulmonary fibrosis (PPF), which has similar disease burden and outcome as IPF.Novel diagnostic methods are currently being developed for the diagnosis of PF, including positron emission tomography (PET) tracer fluorescence imaging, exhaling breath analysis using eNose, and novel blood biomarkers.In IPF, antifibrotics should be recommended to patients at diagnosis. In PPF, the timing and balancing of immunosuppression and antifibrotics is unclear.Various promising studies are underway to target key molecular pathways involved in PF pathogenesis. Pulmonary fibrosis (PF) encompasses a spectrum of chronic lung diseases that progressively impact the interstitium, resulting in compromised gas exchange, breathlessness, diminished quality of life (QoL), and ultimately respiratory failure and mortality. Various diseases can cause PF, with their underlying causes primarily affecting the lung interstitium, leading to their referral as interstitial lung diseases (ILDs). The current understanding is that PF arises from abnormal wound healing processes triggered by various factors specific to each disease, leading to excessive inflammation and fibrosis. While significant progress has been made in understanding the molecular mechanisms of PF, its pathogenesis remains elusive. This review provides an in-depth exploration of the latest insights into PF pathophysiology, diagnosis, treatment, and future perspectives. Pulmonary fibrosis (PF) encompasses a spectrum of chronic lung diseases that progressively impact the interstitium, resulting in compromised gas exchange, breathlessness, diminished quality of life (QoL), and ultimately respiratory failure and mortality. Various diseases can cause PF, with their underlying causes primarily affecting the lung interstitium, leading to their referral as interstitial lung diseases (ILDs). The current understanding is that PF arises from abnormal wound healing processes triggered by various factors specific to each disease, leading to excessive inflammation and fibrosis. While significant progress has been made in understanding the molecular mechanisms of PF, its pathogenesis remains elusive. This review provides an in-depth exploration of the latest insights into PF pathophysiology, diagnosis, treatment, and future perspectives.