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Management of IPF

There is no cure for idiopathic pulmonary fibrosis (IPF) but a number of potential pharmacological treatment options and therapeutic strategies for patients to manage their condition exist. As the clinical course of IPF is highly variable between patients, management of the disease may also vary considerably between patients. Management strategies may include pharmacologic therapies, long-term oxygen treatment, pulmonary rehabilitation, lung transplantation or participation in clinical trials.

Management guidelines for IPF

In 2011, clinical guidelines for the management of idiopathic pulmonary fibrosis (IPF) were developed by an international collaboration of major respiratory societies in the US (ATS), Europe (ERS), Japan (JRS) and Latin America (ALAT). Together, these professional bodies reviewed the clinical evidence base, and made recommendations about pharmacologic treatment options and therapeutic strategies for patients with IPF.1

Update of IPF treatment guidelines in 2015

The 2015 “Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis” was published in July 2015, updating treatment recommendations according to evidence reported since the publication of the prior guideline in 2011 (see sections below).2

Watch the IPF webinar: Update of the IPF treatment guidelines in 2015 with Prof Jürgen Behr and Prof Carlo Vancheri

A number of non-pharmacologic therapies (addressed only in 2011) are recommended for patients with IPF:1

  • Lung transplantation has shown to confer a survival benefit in selected patients with IPF; however, it is only available for a small number of patients.
  • Patients with IPF and resting hypoxemia may receive long-term oxygen therapy to ameliorate oxygenation and potentially improve exercise capacity.
  • The majority of patients should receive pulmonary rehabilitation.
  • Palliative care is a central part of the management of patients with IPF and is particularly important given the current lack of effective treatments for the disease. Palliative care should aim to relieve physical and emotional suffering of patients, provide psychological and spiritual support and assist patients in end-of-life care planning.
References: 
  1. Raghu G, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011;183:788–824.
  2. Raghu G., et al. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline. Am J Respir Crit Care Med 2015;192:e3–e19.