Idiopathic Pulmonary Fibrosis and Progressive Pulmonary Fibrosis: A Call for Collaborative Drug Development

Fibrosing Interstitial Lung Diseases (ILDs) are a heterogeneous group of conditions primarily affecting people aged ≥ 50 years. Idiopathic Pulmonary Fibrosis (IPF), the most common fibrosing ILD is a chronic progressive and irreversible condition with a high risk of mortality, especially in individuals with comorbidities. The term Progressive Pulmonary Fibrosis (PPF) covers ILDs other than IPF that also show progressive fibrosing phenotype. IPF and PPF are devastating parenchymal lung diseases with poor prognosis, resulting in progressive respiratory failure and death. IPF and PPF share common pathological, radiological and histologic features and have similar clinical course of disease progression. However despite their similarities, drug development appears to be progressing separately. Chronic administration of anti-fibrotic drugs, i.e., nintedanib and pirfenidone, has shown a significant reduction in the rate of forced vital capacity decline and mortality and are currently approved for IPF treatment. Recent studies have also demonstrated the clinical efficacy of these drugs in PPF patients. We conducted a non-systematic literature search for peer reviewed articles and clinical trials to review available data regarding the similarities in pathogenesis and progression pathways between IPF and PPF and discuss current challenges in the management of these conditions. Based on the available scientific evidence, we propose it is timely to treat IPF and PPF as similar conditions, at least in terms of patient management and unified approaches are reasonable to be implemented in future clinical trials for both IPF and PPF.

Author(s): Peter Kiely, Botond Nagy and Anthony Bohnert

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