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Year : 2020  |  Volume : 22  |  Issue : 1  |  Page : 1-16

Idiopathic pulmonary fibrosis: What has changed in the diagnosis and treatment from past to present?

1 Department of Chest Diseases, Health Sciences University, Izmir Dr. Suat Seren Chest Diseases and Chest Surgery Training and Research Hospital, Izmir, Turkey
2 Department of Chest Diseases, Haydarpasa Sultan Abdulhamid Training and Research Hospital, Istanbul, Turkey
3 Department of Chest Diseases, Health Sciences University Derince Training and Research Hospital, Kocaeli, Turkey
4 Department of Chest Diseases, Adnan Menderes University, Aydin, Turkey
5 Department of Chest Diseases, Bülent Ecevit University, Zonguldak, Turkey

Correspondence Address:
Dr. Ceyda Anar
Department of Chest Diseases, Health Sciences University, Izmir Dr. Suat Seren Chest Diseases and Chest Surgery Training and Research Hospital, Izmir
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ejop.ejop_45_19

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Idiopathic pulmonary fibrosis (IPF) is the most common and most fatal of all lung diseases that cause widespread scarring in the lungs. High-resolution computed tomography (HRCT) has high diagnostic value in the diagnosis of IPF. Patients exhibiting a pattern of usual interstitial pneumonia (UIP) can be diagnosed with IPF without the need for a biopsy if no other conditions exist that could cause this pattern. If no pattern of UIP exists, a multidisciplinary council should gather to discuss the HRCT and pathological and clinical findings and to decide upon a diagnosis. Appropriate supportive therapies such as oxygen therapy, pulmonary rehabilitation, and seasonal flu and pneumococcal vaccines should be included in the management of the disease. Comorbidities must be investigated and treated. There have been studies identifying the benefits of pirfenidone and nintedanib in patients with mild-to-moderate IPF. There is a lack of appropriate data to guide the selection between pirfenidone and nintedanib, and the patient's preferences and drug tolerance must be considered when making such a drug selection. There have been no randomized studies to date showing the benefits of drugs in severe IPF. The prevention of acid reflux may be beneficial, but the symptoms are obscure. Lung transplantation can be an option for young patients with a severe and progressive disease when there are no comorbidities to pose a contraindication.

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