Abstract [eng] |
Idiopathic pulmonary fibrosis is the most common of all idiopathic interstitial pneumonias. It is a rare disease, and the main symptoms are gradually progressive dyspnea and non-productive cough as well as a decline in physical capacity. Pulmonary function tests usually indicate restrictive ventilatory lung impairment and reduced carbon monoxide diffusion capacity. Idiopathic pulmonary fibrosis is most common in the elderly. Although a comprehensive understanding of the pathogenesis of this disease is still lacking, epidemiological studies point to ageing and cigarette smoking as major risk factors. The disease is usually diagnosed by high-resolution computed tomography scanning when changes in the lungs typical of usual interstitial pneumonia are detected and other diseases are excluded. If the diagnosis is uncertain, it can be confirmed by lung biopsy. Treatment of the disease includes supportive care, oxygen therapy, if necessary, antifibrotic drugs, and, when the disease is very advanced, lung transplantation. Lung transplantation is only available for a small proportion of patients, and the antifibrotic drugs nintedanib and pirfenidone only delay the progress of the disease. It is recommended to test liver transaminase activity and bilirubin levels before prescribing antifibrotic drugs and to monitor them at regular intervals during treatment. This report presents the clinical case of a 70-year-old man with idiopathic pulmonary fibrosis. The patient was prescribed the antifibrotic drug nintedanib and, during treatment, the course of the disease was monitored for two years. In addition, this study also reviews the literature related to the aethiology, prevalence, pathogenesis, risk factors, diagnostics, exacerbations, disease course, prognosis, treatment, and follow-up of idiopathic pulmonary fibrosis. The literature review was conducted using the electronic database “PubMed” (MEDLINE). Study also based on the second updated edition of the “Guidelines for the Diagnosis and Treatment of Interstitial Lund Diseases”, published by the Lithuanian Society of Pulmonology in 2020, and the guidelines for the clinical practice of idiopathic pulmonary fibrosis, published by the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society and the Asociación Latinoamericana de Tórax. |