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Idiopathic Interstitial Lung Disease: A Review of Recent Classifications

Chapman, Jeffrey T. MD*; Farver, Carol F. MD

doi: 10.1097/01.cpm.0000107640.12630.c3
Interstitial, Inflammatory, and Occupational Lung Disease
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The interstitial lung diseases are a heterogeneous group of disorders that present with similar signs and symptoms. The term idiopathic interstitial pneumonia (IIP) refers to a subgroup of interstitial lung diseases without known cause and is not a specific diagnosis. Historically, there has been much confusion clinically and in the literature because of overlapping and subjective physical and pathologic findings. Liebow and Carrington first developed a working categorization the individual IIPs in 1969, and this has been the basis for subsequent treatment and research. Since that time, improvements in surgical techniques have allowed more frequent diagnostic tissue sampling, and better clinical and pathologic characterization of each distinct disorder has enabled recent refinements and changes in the classification of IIPs. Unfortunately, what some felt to be a confusing, but familiar, alphabet soup has changed once again. This review explains the recent changes and provides clinical, radiologic, and pathologic characteristics for each IIP entity. In addition, treatment options are discussed, some of which are evolving and may provide benefit for previously untreatable conditions.

The idiopathic interstitial pneumonias (IIPs) are a heterogeneous group of disorders with similar signs and symptoms but different treatment responses and prognoses. They have recently been reclassified, and the authors explain the recent changes and provide clinical, radiologic, and pathologic characteristics for each IIP entity. In addition, treatment options are discussed, some of which are evolving and may provide benefit for previously untreatable conditions.

*Department of Pulmonary and Critical Care Medicine, Cleveland Clinic Foundation, Cleveland, OH; and †Department of Clinical Pathology, Cleveland Clinic Foundation, Cleveland, OH.

Address correspondence to: Dr. Jeffrey T. Chapman, Department of Pulmonary and Critical Care Medicine, Cleveland Clinic Foundation, Desk A/90, Cleveland, OH 44195. Email:Chapmaj@ccf.org.

© 2004 Lippincott Williams & Wilkins, Inc.