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.KEYWORDS: Idiopathic pulmonary fibrosis, lung allocation score, lung transplantation, oxygen therapy, palliative care, pulmonary rehabilitation I diopathic pulmonary fibrosis (IPF) is a chronic, progressive, fatal form of diffuse interstitial lung disease which is associated with substantial mortality and morbidity [1,2]. It accounts for nearly 30% of lung transplantation procedures performed worldwide [3]. The disease is usually characterised by gradual onset of symptoms, with dyspnoea being the most prominent and the most disabling [4]. Risk factors associated with disease development include smoking, drug exposure, chronic aspiration, infectious agents and genetic predisposition [1].The management of IPF requires a methodical approach with regular evaluations and the implementation of both pharmacological and nonpharmacological treatment strategies. Potential pharmacotherapy includes corticosteroids, immunosuppressive agents and/or antifibrotic agents [1]. Thus, it is important to consider what nonpharmacological interventions are available that might allow patients with IPF to optimise their quality of life [5].
REHABILITATIONPulmonary rehabilitation has become the standard of care for chronic lung disease because of its effectiveness in alleviating symptoms, reducing the duration of hospital stays, increasing exercise tolerance and maximising functional ability [6,7]. Exercise rehabilitation has also been associated with positive psychosocial outcomes, including reduced symptoms of anxiety and depression [8].Pulmonary rehabilitation in IPF has been associated with a statistically significant improvement in 6-min walk distance and dyspnoea [9], although the role of maintenance exercise programmes following the initial structured training remains unclear [10]. Patients should be educated with regard to the treatment and prevention of infections [11]. Patients undergoing lung transplantation are required to participate in preoperative and postoperative pulmonary rehabilitation. The programmes help physicians to select appropriate surgical candidates, and also prepare patients physically and psychologically for the stress of surgery [10].Successful pulmonary rehabilitation may require behavioural changes on the part of the patient, including: weight loss, adopti...