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About Rare DiseasesRare Disease Wiki

Progressive Fibrosing Interstitial Lung Disease (PF-ILD)

Name of disease:

Progressive Fibrosing Interstitial Lung Disease (PF-ILD)

ICD-10 diagnosis code:

J84.1

Causes:

Interstitial lung disease (ILD) encompasses a group of >200 parenchymal pulmonary disorders, including idiopathic pulmonary fibrosis, autoimmune ILDs and hypersensitivity pneumonitis. A proportion of patients with interstitial lung diseases (ILD) may develop a progressive fibrosing phenotype, associated with worsening respiratory symptoms, lung function decline, and potentially early death.

Pattern of inheritance:

The pathogenesis is not well understood. Although genetic variants contribute to the risk of developing IPF, their clinical relevance needs to be demonstrated in future studies.

Prevalence:

18-32% of patients with designated ILD develop a progressive fibrosing phenotype. Risk factors of PF-ILD includes male sex, older age, and lower lung function.

Diagnosis:

Evaluations include clinical presentation, specific history assessment, smoking status, lung function evolution, serological test results, imaging and, if required, lung biopsy.

Age of onset:

ILD can happen in all age groups. Age of onset depends on the ILD subtypes. For instance, peak age of onset of idiopathic pulmonary fibrosis is 50-60, while it may occur in young age groups for other ILD subtypes.

Common signs and symptoms:

Dry cough, shortness of breath, chest discomfort, fatigue and weakness, loss of apppetite, loss of weight.

Available treatments (medicinal and non-medicinal):

Drug treatment for symptomatic relief, anti-fibrotics, pulmonary rehabilitation, oxygen therapy, lung transplant

Disease management tips:

N/A

References:

1. Cottin V, Hirani NA, Hotchkin DL, et al. Eur Respir Rev 2018; 27:180076. 2. Albera C, Verri G, Sciarrone F, et al. Biomedicines 2021;9(9):1237. 3. Wijsenbeek M, Kreuter M, Olson A, et al. Curr Med Res Opin: 2019;35(11):2015-2024. 4. Wong A, Ryerson CJ, Guler SA. Respir Res. 2020;21(1):32. 5. Cottin V, Hirani NA, Hotchkin DL, et al. Eur Respir Rev. 2018;27:180076 6. Ward J & McDonald CF. Aust Fam Physician. 2010;39(3):106-1107. Stanford Medicine Healthcare. Symptoms of Interstitial Lung Disease (ILD). Available at: https://stanfordhealthcare.org/medical-conditions/chest-lungs-and-airways/interstitial-lung-disease/symptoms.html. Accessed on March 10, 20238. Maher, TM & Strek ME. Respir Res. 2019; 20:205.

Other useful websites:

Knowing ILD:https://www.knowingild.org.hk

Acknowledgment

This page is contributed by Boehringer Ingelheim (HK) Ltd.