Follow-up and nonpharmacological management of the idiopathic pulmonary fibrosis patient

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fatal form of diffuse interstitial lung disease. Management of IPF requires an orderly approach, with regular evaluations and implementation of both pharmacological and nonpharmacological treatments. Pulmonary rehabilitation can relieve patients from the distressing symptoms of IPF and improve quality of life. Oxygen therapy is central to treatment of all patients. Lung transplantation enhances survival in selected patients. Mechanical ventilation may be used in patients with acute exacerbations, but the prognosis is poor in these cases. Palliative care focuses on symptom management, advance directives and end-of-life planning. Patient support groups may also play an important role.

Figure 1.

Survival on waiting list (from placement on the list to death or transplantation; ·······) compared with survival of patients who underwent lung transplantation (from placement on the list including post-transplantation survival; –––––). Reproduced from [16] with permission from the publisher.