Current management of idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible and eventually fatal chronic interstitial pneumonia limited to the lung and associated with the histological and/or radiological pattern of usual interstitial pneumonia. The mean age of the disease is 65 and it is more frequent in men than women. The disease was historically considered as an inflammatory disease, but currently this has shifted towards a prominent role of impaired wound healing process. The diagnosis of disease requires exclusion of other known causes of interstitial lung disease, the presence of a usual interstitial pneumonia pattern on high - resolution computed tomography (HRCT) in patients or specific combinations of HRCT and surgical lung biopsy patterns. The patients are considered as mild, moderate and severe according to the symptoms, radiological and pulmonary function tests. It is difficult to predict the course of the disease; clinical exacerbation can be seen after a long stable period. Average median survival is approximately 3 years. Advanced age, smoking, low body mass index, widespread radiological involvement, comorbidities and complications (pulmonary hypertension, emphysema, and bronchogenic cancer) are considered as poor prognostic factors. The treatment approach is the basis on the severity of the disease and patient preference. The recent positive result of the pirfenidone and nintedanib phase II and III clinical trials based on the prevailing mechanism of IPF pathogenesis particularly targeted fibroblast activation and myofibroblast differentiation have currently been reported. Initiating therapy with pirfenidone and nintedanib is recommended for the patients with mild and moderate IPF who do not have underlying liver disease and who live in area where these drugs are available. Nausea and rashes are more common in treatment with pirfenidone while diarrhea and deterioration in liver function tests are seen more frequently in nintedanib. The information regarding participation in randomized trials should be given to all patients and also early referral for transplantation should be considered.