Idiopathic pulmonary fibrosis: epidemiology, clinic, diagnosis and treatment

Aistė Pučinskaitė1

1Faculty of Medicine, Vilnius University, Vilnius, Lithuania

Idiopathic pulmonary fibrosis (IPF) is defined as chronic fibrous interstitial pneumonia associated only with the lungs. IPF is a progressive disease with a poor long-term prognosis and few effective treatment options. The pathogenesis and etiology of the disease are unclear. It is known that healthy lung tissue is replaced by extracellular material and the architecture of the alveoli is destroyed. As a result, lung function and gas diffusion in the lungs are impaired, which ultimately leads to respiratory failure and death. The prevalence of patients with IPF has increased in recent years, presumably due to the optimization of diagnostic methods and increasing life expectancy. The classic complaints of IPF are progressive shortness of breath and unproductive dry cough. Therefore, the diagnosis of IPF should be suspected in all patients who suddenly develop and progress to dyspnoea, especially during exercise, less often have dry cough. Also, if crepitation is heard in the lungs during auscultation and the patient’s fingers are deformed. When IPF is suspected, the disease should be differentiated from other interstitial lung diseases. Lung function tests confirm restrictive ventilatory lung function and hypoxaemia and/or hypercapnia in the blood gas. Several diagnostic methods are required to diagnose IPF. Computed tomography (CT) images show stretching bronchiectasis, and bronchiolytase, which may appear with signs of ground glass opacities. A lung biopsy is performed to clarify the diagnosis of IPF. Treatment includes supportive care, oxygen supplementation if necessary, antifibrotic medications, and the possibility of lung transplantation in the event of severe illness. However, further research is needed to find new treatment options and to extend the life expectancy of patients with IPF.

Keywords: idiopathic pulmonary fibrosis, interstitial lung disease, pirfenidone, nintedanib.