Identification, course, and management of progressive pulmonary fibrosis. Academic Article uri icon

Overview

abstract

  • The term "progressive pulmonary fibrosis" or "PPF" is generally used to describe progressive lung fibrosis in an individual with an interstitial lung disease (ILD) other than idiopathic pulmonary fibrosis (IPF). Several sets of criteria have been proposed for the identification of PPF, most of which are based on a combination of a decline in forced vital capacity, worsening of respiratory symptoms, and increase in the extent of fibrosis on radiology. Although some risk factors for faster progression of fibrosing ILD have been identified, it remains challenging to predict which individuals will develop PPF. Close monitoring, including regular pulmonary function tests, is required to detect the earliest signs of worsening disease. PPF is associated with high rates of hospitalization and death. Management of PPF requires a multidisciplinary and multimodal approach, including pharmacological therapy and supportive care. Discussions about palliative care should begin at an early stage, individualized to the needs of the patient.

publication date

  • October 1, 2024

Research

keywords

  • Disease Progression
  • Pulmonary Fibrosis

Identity

Scopus Document Identifier

  • 85208503256

Digital Object Identifier (DOI)

  • 10.37765/ajmc.2024.89634

PubMed ID

  • 39495032

Additional Document Info

volume

  • 30

issue

  • 7 Suppl