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. Progressive pulmonary fibrosis (PPF) encompasses a wide range of diseases with disparities in clinical patterns and disease features. Despite the difference in the underlying ILD subtype, the clinical course of PPF is similar and leads to accelerated respiratory failure, frequent disease exacerbation, and earlier mortality. Choosing an appropriate therapy in this setting can be challenging due to lack of effective treatment options and guideline recommendations. Therapy is currently based on immunosuppression depending on the underlying ILD subtype, but the response to these therapies may vary. Accurate diagnosis and urgent treatment are needed, as mortality is higher in patients with PF-ILD than in those without progressive disease.
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 - Mar 06, 2023.