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Idiopathic Nonspecific Interstitial Pneumonia

Report of an American Thoracic Society Project

¹ Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York; ² Department of Medicine, University of Iowa, Iowa City, Iowa; ³ Department of Medicine, University of California, San Francisco, San Francisco, California; ⁴ Department of Radiology, National Jewish Medical and Research Center, Denver, Colorado; ⁵ Department of Pathology, Mayo Clinic, Scottsdale, Arizona; ⁶ Department of Radiology, University of Maryland, Baltimore, Maryland; ⁷ Department of Pulmonary Medicine, National Jewish Medical Research Center, Denver, Colorado; ⁸ Division of Pulmonary and Critical Care Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea; ⁹ Service de Broncho-Pneumologie, Hôpital Cardiovasculaire et Pneumologique, Lyon, France; ¹⁰ Department of Pulmonary Medicine, Royal Brompton Hospital, London, United Kingdom; ¹¹ Department of Pulmonary Medicine, University of Michigan, Ann Arbor, Michigan; ¹² Department of Pulmonary and Mediastinal Pathology, Armed Forces Institute of Pathology, Washington, DC; ¹³ Department of Radiology, Royal Brompton Hospital, London, United Kingdom; ¹⁴ Department of Radiology, Mayo Clinic, Rochester, Minnesota; ¹⁵ Department of Radiology, University of Michigan, Ann Arbor, Michigan; ¹⁶ Department of Pulmonary Medicine, Asan Medical Center, Ulsan University, Seoul, Korea; ¹⁷ Laboratory of Anatomic Pathology, National Hospital Organization Kinki-chuo Chest Medical Center, Osaka, Japan; ¹⁸ Department of Radiology, and ¹⁹ Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan; ²⁰ Pulmonary Division, Mayo Clinic, Rochester, Minnesota; ²¹ Department of Radiology, University of British Columbia, Vancouver, Canada; ²² Department of Pathology, Royal Brompton Hospital, London, United Kingdom; ²³ Pulmonary and Critical Care, University of Washington Medical Center, Seattle, Washington; and ²⁴ Instituto Nacional de Enfermedades Respiratorias, Mexico City, Mexico

Correspondence and requests for reprints should be addressed to William D. Travis, M.D., Attending Thoracic Pathologist, Department of Pathology, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065. E-mail: travisw{at}mskcc.org

Rationale: The 2002 American Thoracic Society/European Respiratory Society classification of idiopathic interstitial pneumonias identified nonspecific interstitial pneumonia (NSIP) as a provisional diagnosis. Concern was expressed that NSIP was a "wastebasket" category, difficult to distinguish from other idiopathic interstitial pneumonias.

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.

Measurements and Main Results: Sixty-seven 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 high-resolution computed tomography, 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. Five-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 computed tomography scan • usual interstitial pneumonia • pathology • hypersensitivity pneumonitis • lung biopsy

AT A GLANCE COMMENTARY Scientific Knowledge on the Subject Nonspecific interstitial pneumonia was accepted by the 2002 American Thoracic Society/European Respiratory Society classification of idiopathic interstitial pneumonias, but it was regarded as "provisional," pending further study. What This Study Adds to the Field Idiopathic nonspecific interstitial pneumonia is a distinct form of idiopathic interstitial pneumonia. The diagnosis requires a dynamic integrated multidisciplinary approach because the histologic pattern can be seen in other disorders, such as hypersensitivity pneumonitis.

 

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