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Abstract
Idiopathic pulmonary fibrosis is a prototype of chronic, progressive, and fibrotic
lung disease. Healthy tissue is replaced by altered extracellular matrix and alveolar
architecture is destroyed, which leads to decreased lung compliance, disrupted gas
exchange, and ultimately respiratory failure and death. In less than a decade, understanding
of the pathogenesis and management of this disease has been transformed, and two disease-modifying
therapies have been approved, worldwide. In this Seminar, we summarise the presentation,
pathophysiology, diagnosis, and treatment options available for patients with idiopathic
pulmonary fibrosis. This disease has improved understanding of the mechanisms of lung
fibrosis, and offers hope that similar approaches will transform the management of
patients with other progressive fibrotic lung diseases.