OBLITERATIVE BRONCHIOLITIS

Interstitial lung disease is a broad category of heterogenous diseases which share the common feature of inflammatory and fibrotic changes that primarily affect the alveoli and small airways. The two most common manifestations are cryptogenic organizing pneumonia (COP) and idiopathic pulmonary fibrosis (IPF), both of which can occur as “idiopathic” conditions or in association with several autoimmune diseases. 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 COP and IPF as models for autoimmune disorders of the lung. To do so, we discuss the history and the clinical features of COP 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.