Manejo actual de la fibrosis pulmonar

Abstract

INTRODUCTION: Idiopathic pulmonary fibrosis is the most common form of idiopathic interstitial pneumonias, characterized by the constant injury and subsequent remodeling of the interstitial matrix, with an increase in cases worldwide and a symptomatology characterized by progressive dyspnea, cough not productive and generally fatal outcome. Although its etiology remains unknown, it is possible to add genetic factors, environmental factors, as well as pathologies that can predispose and facilitate a patient to develop or allow a more rapid evolution of the disease, a better study and knowledge of the clinical and semiological characteristics could help to have a better diagnosis that is timely and allows to provide a better quality of life. the pathogenesis is considered as a continuous cycle in which the predisposition, consequently activation and progression, the distal bronchiolar and alveolar epithelial lung cells are shown as the pathologically abnormal cells in the IPF lungs and the fibrosis occurs as a consequence of this dysfunction of the epithelial cells. Therefore, it is considered a disease with effect on lung epithelial cells that manifests as fibrosis instead of being an intrinsically fibrotic disease. OBJECTIVES: - Define the triggering factors that cause Idiopathic Pulmonary Fibrosis (IPF). - Describe the main drugs used by current guidelines. - Show the drugs that currently do not have a predilection for the treatment of IPF. - Identify which is the best form of the therapeutic approach of this disease. METHODS: This research review was conducted through the use of the following academic data search platforms: ScienceDirect, Cochrane, Pubmed, Medline and Google Scholar in the period from 2013 to 2018. DEVELOPMENT: Articles related to Idiopathic Pulmonary Fibrosis that were found in levels Ia, Ib, IIa, IIb and III according to the US Agency for Health Care Policy and Research (now US Agency for Healthcare Research and Quality) were selected and classified. adequate quality standards and reliability of platforms defined by percentiles; making the comparison between the clinical and / or semiological characteristics of the pathology mentioned previously. CONCLUSIONS: Idiopathic pulmonary fibrosis is a multifactorial nosological entity with genetic background with increasing number of cases, subclinically identified so that most of the time ends in a fatal outcome, the progress in the treatment has been very important because it decreases the advance and offers a better quality of life, although in severe cases only lung transplantation is recommended and in our country only a lung transplant has been reported. In the present work, a compilation of several guidelines was made where it was found that there are many recommendations against the use of drugs described in past works such as ambrisentan, anticoagulants, the combination of prednisone, azathioprine, imatinib and N-acetylcysteine (NAC), always and when there is no comorbidity that aggravates the clinical picture, although several studies showed the efficacy of B2-agonists as an adjuvant of antimuscarinics in case of exacerbations, with favorable clinical results. The most effective treatment in this disease is lung transplantation, which is currently carried out in Ecuador, but due to the rapid progression of the pathology and the low number of donors in our country it is still very difficult to access.