The efficacy of pulmonary rehabilitation in improving the clinical status in idiopathic pulmonary fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fatal lung disease with a median survival rate of 2-4 years after diagnosis, occurring primarily in older adults. The diagnosis is suggested by histology or radiologic evidence of a usual interstitial pneumonia (UIP) pattern and exclusion of known cases of pulmonary fibrosis. There are some comorbidities associated with IPF such as pulmonary hypertension, emphysema, chronic obstructive pulmonary disease (COPD), asthma, lung cancer, cardiovascular disorders, gastroesophageal reflux disease (GERD), sleep disorders and psychiatric disturbances. The management of IPF focuses on the amelioration of symptoms, preserving lung function, improving health status, minimizing the adverse effects of therapy and improving survival. Pulmonary rehabilitation is suggested for IPF patients when adequate medical therapy controls poorly the disease progression and mental, physical or social consequences of the illness persist during daily life. Currently, there are only two approved available antifibrotic therapies, pirfenidone and nintedanib, capable to reduce disease progression and physical deterioration, but cure is elusive and improvements are hardly observed. In addition, there is a continuous need of non-drug therapy components which should be included in IPF patient management: education, psychosocial support, exercise training, nutrition, symptom management and palliative care, non-invasive ventilation and pulmonary transplant. These complementary therapies have been proven to improve dyspnea, exercise capacity, fatigue and quality of life. Key words: idiopathic pulmonary fibrosis, rehabilitation, quality of life, palliative care,