Patient considerations and drug selection in the treatment of idiopathic pulmonary fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease of unknown cause. Approximately 5,000 people are diagnosed with IPF in the UK every year. People with IPF suffer significant morbidity and, without any curative treatment at present, survival rates remain poor with a median survival of 3 years. While treatment remains largely supportive, many drug therapies have been trialed in IPF over the years. Pirfenidone and nintedanib are newly licensed treatments for IPF and the first drugs to have shown convincing evidence of slowing disease progression. In addition to evaluating clinical evidence, we also discuss elements affecting drug choice from the viewpoint of patients and health care professionals. We discuss pharmacological and nonpharmacological aspects of providing best supportive care for patients with IPF. However, few good quality studies exist focusing on controlling symptoms specifically in patients with IPF, and recommendations are often extrapolated from evidence in other chronic diseases. In covering these topics, we hope to provide readers with a comprehensive review of the available evidence pertaining to all aspects of care for patients suffering with IPF.

Keywords: clinical trials; decision making; forced vital capacity; high-resolution computed tomography; idiopathic pulmonary fibrosis; interstitial lung disease; usual interstitial pneumonia.

Figure 1

Histology of a video-assisted thoracic surgical lung biopsy showing a fibroblastic focus (black arrow), areas of fibrosis (F), intra-alveolar macrophages (M), chronic inflammatory cells (white arrow), and residual alveolar spaces (A).

Figure 2

High-resolution CT scan of the chest showing features of UIP, including peripheral reticulation (open arrows) and honeycombing (closed arrow). Abbreviations: CT, computed tomography; UIP, usual interstitial pneumonia.

Figure 3

Photosensitive rash affecting light-exposed areas in a patient taking pirfenidone for 3 months during the winter in the North of England.