IDIOPATHIC PULMONARY FIBROSIS - CURRENT CONCEPTS

Idiopathic pulmonary fibrosis (LPF) is generally defined as a progress ive, fibrosing inflammatory disease of the lung parenchyma of unknown cause. It is characterized by slowly increasing dyspnea, diffuse inter stitial lung infiltrates, restrictive lung dysfunction, and impaired g as exchange. Ultimately, it is fatal in most patients, and treatment o ptions remain unsatisfactory. The advent of high-resolution computed t omography of the chest and modifications in the histopathologic classi fication of interstitial pneumonias have reshaped the concept of IPF, Although initially thought to be a relatively specific clinicopatholog ic entity, it seems likely that IPF as previously defined is a heterog eneous disorder consisting of several clinicopathologic entities with differing histopathologic patterns, clinical course, response to thera py, and prognosis. The most common histologic pattern in cases previou sly defined as IPF is usual interstitial pneumonia, which is associate d 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 clinico-pathologic e ntities that have been included under the clinical heading of IPF, In the future, we recommend that the use of the term ''idiopathic pulmona ry fibrosis'' be restricted to patients with usual interstitial pneumo nia and that clinicians recognize the fact that other idiopathic inter stitial pneumonias do not have the same prognostic effect traditionall y ascribed to IPF.