Progression of fibrosing interstitial lung disease.
Alyson W WongChristopher J RyersonSabina A GulerPublished in: Respiratory research (2020)
Fibrotic interstitial lung diseases (ILDs) are often challenging to diagnose and classify, but an accurate diagnosis has significant implications for both treatment and prognosis. A subset of patients with fibrotic ILD experience progressive deterioration in lung function, physical performance, and quality of life. Several risk factors for ILD progression have been reported, such as male sex, older age, lower baseline pulmonary function, and a radiological or pathological pattern of usual interstitial pneumonia. Morphological similarities, common underlying pathobiologic mechanisms, and the consistently progressive worsening of these patients support the concept of a progressive fibrosing (PF)-ILD phenotype that can be applied to a variety of ILD subtypes. The conventional approach has been to use antifibrotic medications in patients with idiopathic pulmonary fibrosis (IPF) and immunosuppressive medications in patients with other fibrotic ILD subtypes; however, recent clinical trials have suggested a favourable treatment response to antifibrotic therapy in a wider variety of fibrotic ILDs. This review summarizes the literature on the evaluation and management of patients with PF-ILD, and discusses questions relevant to applying recent clinicial trial findings to real-world practice.
Keyphrases
- interstitial lung disease
- idiopathic pulmonary fibrosis
- systemic sclerosis
- lung function
- clinical trial
- multiple sclerosis
- rheumatoid arthritis
- physical activity
- healthcare
- cystic fibrosis
- chronic obstructive pulmonary disease
- air pollution
- systematic review
- randomized controlled trial
- ejection fraction
- phase ii
- mental health
- stem cells
- study protocol
- combination therapy
- replacement therapy
- mesenchymal stem cells
- mass spectrometry
- cell therapy
- mechanical ventilation
- double blind