Within the spectrum of fibrosing interstitial lung diseases (ILDs) is a proportion of patients who experience progression of pulmonary fibrosis despite immunomodulatory treatment, known as the progressive fibrotic phenotype. The idea of progressive fibrosing ILD (PF-ILD) has been studied and applied primarily among patients with idiopathic pulmonary fibrosis (IPF), but there is an increasing focus on progressive fibrosis in other subtypes of patients on the wide spectrum of underlying ILD diagnoses.
Choosing an appropriate therapy in this setting can be challenging due to lack of precision in classifying the various forms of ILD, and it can be necessary to obtain tissue specimens for a definitive diagnosis. Other than IPF, there are no established treatment paradigms for other forms of progressive fibrosing ILD. Therapy is currently based on corticosteroids and/or immunomodulators, but the response to these therapies may vary, especially in more fibrosing forms of disease. Newer antifibrotic therapies are also available. Supportive care and treatment of comorbidities are important aspects of PF-ILD management.
Are you up-to-date with the management of PF-ILD? Test your knowledge with this quick quiz.
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Cite this: Zab Mosenifar. Fast Five Quiz: Interstitial Lung Disease Progressive Fibrosing Management - Medscape - Feb 01, 2022.