Idiopathic pulmonary fibrosis: the role of pathobiology in making a definitive diagnosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease of unknown etiology characterized by fibrosis of the interstitium, resulting in progressive respiratory insufficiency and shortened lifespan. Treatment focus tends to shift from disease-centered to symptom-centered as the disease progresses. Over the years, a number of pharmacologic strategies have been used to treat IPF, albeit without solid evidence demonstrating a beneficial impact on the disease course. The previously held theory that inflammation was the predominant underlying feature of IPF led to the use of corticosteroids and immunosuppressive therapy as the standard of care. However, a greater understanding of the pathogenesis of IPF has evolved and guidelines were developed using evidence-based criteria. Guided by the data, treatment guidelines developed in 2011 stated that no pharmacologic therapy showed a proven benefit for patients with IPF and issued recommendations against the use of most treatments. The treatment landscape changed in October 2014, when the FDA approved pirfenidone and nintedanib for the treatment of IPF. For the first time, clinicians have therapeutic options with demonstrated clinical efficacy to treat patients with IPF. To provide effective high-value care for patients with IPF, healthcare professionals require thorough knowledge and awareness about these medications, including their safety concerns.