Idiopathic and Autoimmune Interstitial Lung Disease

Brian Gelbman, Ronald G. Crystal

Research output: Chapter in Book/Report/Conference proceedingChapter


Interstitial lung disease is a broad category of heterogeneous 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 idiopathic pulmonary fibrosis (IPF) and cryptogenic organizing pneumonia (COP), 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 will first discuss the history and the clinical features of COP and IPF, followed by a summary of the autoimmune, genetic, and environmental features, current concepts of pathogenesis, and the therapies currently available and under investigation for both disorders.

Original languageEnglish
Title of host publicationThe Autoimmune Diseases
Subtitle of host publicationFifth Edition
PublisherElsevier Inc.
Number of pages19
ISBN (Print)9780123849298
Publication statusPublished - 1 Dec 2013



  • Bronchiolitis obliterans
  • Bronchoalveolar lavage (BAL)
  • Cryptogenic organizing pneumonia (COP)
  • Idiopathic pulmonary fibrosis (IPF)
  • Usual interstitial pneumonia

ASJC Scopus subject areas

  • Immunology and Microbiology(all)

Cite this

Gelbman, B., & Crystal, R. G. (2013). Idiopathic and Autoimmune Interstitial Lung Disease. In The Autoimmune Diseases: Fifth Edition (pp. 1105-1123). Elsevier Inc..