Idiopathic pulmonary fibrosis: acute exacerbation and lung cancer associated with pulmonary fibrosis

Abstract

Abstract: The 5-year survival rate for idiopathic pulmonary fibrosis (IPF) is 30-50%, and the major causes of death are respiratory failure, cardiac failure, lung cancer, infection, and pulmonary thromboembolism. Clinical conditions of acute exacerbation of IPF, which was first reported from Japan and is also now being recognized in Europe and the US, include deterioration of dyspnea, exacerbation in image findings, a decrease in the partial pressure of oxygen in arterial blood, and elevation in the levels of KL-6 and SP-D, in a short period of time. Its clinical characteristics are those of the ARDS and its pathological findings are those of diffuse alveolar damage (DAD). Infection, an inappropriate tapering of the dose of steroids, certain medications, invasive tests, and surgical operations are among the inducing factors. Combination therapy by steroid pulse and cyclosporin-A has been attracting attention. The complication of lung cancer in IPF, which is frequently observed in patients aged 70 or older, is one of the prognostic factors, and the knowledge of IPF is indispensable to treat this. Expression and mutations of various genes during the repairing process of damaged DNA is presumed to be involved in the development of this lung cancer. Special attention is required for the treatment because surgery, administration of anti-cancer drugs, and radiation can possibly induce the acute exacerbation