Therapy and management of idiopathic pulmonary fibrosis

IPF is one of the most common causes of ILD of unknown etiology. Although the pathogenesis of IPF is not well understood, the clinical, radiographic, and physiologic findings in this disease have been clearly described. Most patients are middle aged and present with progressive breathlessness and dyspnea on exertion. The most sensitive chest imaging technique is HRCT. Pulmonary function testing often reveals restriction and hypoxemia at rest or with exertion. Therapy for IPF usually involves corticosteroids or cytotoxic agents, such as cyclophosphamide or azathioprine, to decrease pulmonary inflammation and retard the fibrotic process. Unfortunately, only 20-30% of patients objectively respond to treatment. Lung transplantation offers the only potential for the restoration of pulmonary function.