Chapter 67 - Idiopathic Bronchiolitis Obliterans, Idiopathic Pulmonary Fibrosis, and Autoimmune Disorders of the Lung

Abstract

Idiopathic bronchiolitis obliterans (IBO) is a disorder of small airways and idiopathic pulmonary fibrosis (IPF) is a disorder that affects primarily the alveoli. Both are chronic inflammatory or fibrotic disorders that are typically progressive and often fatal. Although both are classified as “lung disorders of unknown etiology,” there are multiple clues suggesting that autoimmune mechanisms play a role in the pathogenesis of both disorders. The focus of this chapter is to explore the link between autoimmune mechanisms underlying IBO, a disorder of the small airways, and IPF, a disorder that affects primarily the alveoli, are chronic inflammatory or fibrotic disorders that are typically progressive and often fatal as models for autoimmune disorders of the lung. The chapter first discusses the history and the clinical features of IBO and IPF, followed by a summary of the autoimmune, genetic, environmental features, current concepts of pathogenesis, and the therapies currently available and under investigation for both disorders.