Published ahead of print on April 3, 2008, doi:10.1164/rccm.200611-1685OC Am. J. Respir. Crit. Care Med., Volume 177, Number 12, June 2008, 1338-1347 A more recent version of this article appeared on June 15, 2008
Submitted on November 22, 2006 Idiopathic Nonspecific Interstitial Pneumonia Report of an ATS ProjectWilliam D Travis1*,1 Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA, 2 Department of Medicine, University of Iowa, Iowa City, IA, USA, 3 Department of Medicine, University of California San Francisco, San Francisco, CA, USA, 4 Department of Radiology, National Jewish Medical and Research Center, Denver, CO, USA, 5 Department of Pathology, Mayo Clinic, Scottsdale, AZ, USA, 6 Department of Radiology, University of Maryland, Baltimore, MD, USA, 7 Department of Pulmonary Medicine, National Jewish Medical Research Center, Denver, CO, USA, 8 Division of Pulmonary and Critical Care Medicine, Samsung Medical Center; Sungkyunkwan University School of Medicine, Seoul, Korea, Republic of, 9 Service de Broncho-Pneumologie, Hopital CardioVascuaire et Pneumologique, Lyon, France, 10 Department of Pulmonary Medicine, Royal Brompton Hospital, London, England, United Kingdom, 11 Department of Pulmonary Medicine, University of Michigan, Ann Arbor, MI, USA, 12 Department of Pulmonary and Mediastinal Pathology, Armed Forces Institute of Pathology, Washington, DC, USA, 13 Department of Radiology, Royal Brompton Hospital, London, England, United Kingdom, 14 Department of Radiology, Mayo Clinic, Rochester, MN, USA, 15 Department of Radiology, University of Michigan, Ann Arbor, MI, USA, 16 Department of Pulmonary Medicine, Asan Medical Center, Ulsan University, Seoul, Korea, Republic of, 17 Laboratory of Anatomic Pathology, National Hospital Organization Kinki-chuo Chest Medical Center, Osaka, Japan, 18 Department of Radiology, Graduate School of Medicine, Kyoto University, Kyoto, Japan, 19 Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan * To whom correspondence should be addressed. E-mail: travisw{at}mskcc.org.
Rationale: The 2002 ATS/ERS Classification of idiopathic interstitial pneumonias (IIPs) identified nonspecific interstitial pneumonia (NSIP) as a provisional diagnosis. Concern was expressed that NSIP was a "wastebasket" category, difficult to distinguish from other IIPs. Objectives: The following questions were addressed: 1) Is idiopathic NSIP a distinct entity? 2) If so, what are its clinical, radiologic and pathologic characteristics? 3) What is the role of radiology and pathology in establishing the diagnosis? 4) To make a diagnosis of idiopathic NSIP, what other disorders need to be excluded and how should this be done? Methods: Investigators who had previously reported cases of idiopathic NSIP were invited to submit cases for review (n = 305). After initial review, cases with complete clinical, radiologic and pathologic information (n = 193) were reviewed in a series of workshops. Results: 67 cases were identified as NSIP. Mean age was 52 years: 67% were women, 69% were never smokers and 46% were from Asian countries. The most common symptoms were dyspnea (96%) and cough (87%); 69% had restriction. By HRCT, the lower lung zones were predominantly involved in 92% of cases; 46% had a peripheral distribution; 47% were diffuse. Most showed a reticular pattern (87%) with traction bronchiectasis (82%) and volume loss (77%). Lung biopsies showed uniform thickening of alveolar walls with a spectrum of cellular to fibrosing patterns. 5-year survival was 82.3%. Conclusions: Idiopathic NSIP is a distinct clinical entity that occurs mostly in middle-aged women who are never-smokers. The prognosis of NSIP is very good. Key words: High resolution CT scan, usual interstitial pneumonia, pathology, hypersensitivity pneumonitis, lung biopsy
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