Pulmonary rehabilitation in restrictive thoracic disorders

Abstract

The interstitial lung diseases (ILDs) are a group of over 200 debilitating conditions characterised by lung inflammation and/or fibrosis. Idiopathic pulmonary fibrosis (IPF), the most common and lethal of the ILDs, accounts for approximately one-third of the ILDs and has a median survival of 3 years from diagnosis [1]. However, the clinical course varies widely and some patients with IPF will experience long periods of stability, whilst others will have frequent exacerbations or a rapid decline [2]. Interstitial lung disease may also occur due to an underlying systemic disease process, such as connective tissue disease or sarcoidosis, or an occupational exposure, such as asbestosis or silicosis. The incidence of ILD is increasing globally, largely due to a rise in the number of people who are diagnosed with IPF. Idiopathic pulmonary fibrosis is a disease of older people, with an estimated prevalence of 19 per 100,000 in those aged 55-64 years, rising to 88 per 100,000 in those 75 and over [3]. Consequently there will be more people living with IPF as the population ages in developed nations.