Idiopathic pulmonary fibrosis: current concepts

Abstract

Idiopathic pulmonary fibrosis (IPF) is generally defined as a progressive, fibrosing inflammatory disease of the lung parenchyma of unknown cause. It is characterized by slowly increasing dyspnea, diffuse interstitial lung infiltrates, restrictive lung dysfunction, and impaired gas exchange. Ultimately, it is fatal in most patients, and treatment options remain unsatisfactory. The advent of high-resolution computed tomography of the chest and modifications in the histopathologic classification of interstitial pneumonias have reshaped the concept of IPF. Although initially thought to be a relatively specific clinicopathologic entity, it seems likely that IPF as previously defined is a heterogeneous disorder consisting of several clinicopathologic entities with differing histopathologic patterns, clinical course, response to therapy, and prognosis. The most common histologic pattern in cases previously defined as IPF is usual interstitial pneumonia, which is associated with a median survival of less than 3 years. For accurate prognosis and optimal management of patients, the clinician should attempt to be as precise as possible in distinguishing various clinicopathologic entities that have been included under the clinical heading of IPF. In the future, we recommend that the use of the term "idiopathic pulmonary fibrosis" be restricted to patients with usual interstitial pneumonia and that clinicians recognize the fact that other idiopathic interstitial pneumonias do not have the same prognostic effect traditionally ascribed to IPF.