Management and Treatment of IPF – Update

Idiopathic Pulmonary Fibrosis, or IPF, is a growing problem worldwide with increasing numbers of people being affected. There is no cure and treatment options are limited to expensive anti-fibrotic drugs that can slow down the progression of the disease, but not reverse it or stop it completely. These medications have multiple side effects, which can further impact on patients’ quality of life, and only patients with moderate lung function impairment have approved funding to receive them.

The management of patients with IPF is multifaceted and consists of patient education and support, regular outpatient surveillance, symptom relief, pulmonary rehabilitation, annual vaccinations to prevent respiratory infection, supplemental oxygen, managing of comorbidities and ultimately palliative care or, in a minority of patients, referral for lung transplantation.

Following the publication of the ASCEND (A Phase III Trial of Pirfenidone in Patients with Pulmonary Fibrosis) and IMPULSIS (Investigating the Safety and Efficacy of Nintedanib in IPF) trials, two new anti-fibrotic treatments became available for patients who meet stringent National Institute for Health and Care Excellence (NICE) criteria. Pirfenidone and Nintedanib neither cure nor reverse the fibrosis, and have little impact on symptoms, but have been shown to reduce rates of lung function decline and, in the case of Pirfenidone, improve progression-free survival.

Both Nintedanib and Pirfenidone, which are available for use in patients with moderate IPF as defined by an FVC of 50-80% predicted, are associated with side-effects that can affect a patient’s ability to tolerate treatment. Commonly reported side-effects of both are gastrointestinal including diarrhoea, nausea, abdominal pain, and vomiting as well as weight loss and liver enzyme derangement. Additionally, Pirfenidone is associated with skin photosensitivity. These side-effects can be managed with dose reduction, anti-motility agents, taking medication with meals and avoiding sun exposure, but undoubtedly further impact upon health related quality of life.

Update by Dr Emma Denneny, October 2019