Diagnosis and Management of Idiopathic Pulmonary Fibrosis: Implications for Respiratory Care

Abstract

Although poorly understood, idiopathic pulmonary fibrosis is the most common of the idiopathic interstitial lung diseases. Its etiology is unknown, but how this fibrotic process develops in the lung has been studied over the last 60 years. It is a relatively rare disease, afflicting those 50–70 years of age, slightly more common among men than women, without racial predilection. The most common complaint is progressive shortness of breath. Pulmonary function testing reveals a restrictive ventilatory defect with a diminished diffusion capacity. The lungs demonstrate fibrotic and cystic areas interspersed with normal lung on radiographic and pathologic examination. No definitive medical treatment is available, although most patients are given trials of corticosteroids, alone or in combination with cytotoxic agents. On average, patients survive 2–4 years after diagnosis. Lung transplantation has been the only therapy shown to improve survival of those with idiopathic pulmonary fibrosis.