Idiopathic nonspecific interstitial pneumonia: Report of an American Thoracic Society Project

William D. Travis, Gary Hunninghake, Talmadge E. King, David A. Lynch, Thomas V. Colby, Jeffrey R. Galvin, Kevin K. Brown, Pyo Chung Man, Jean Francxois Cordier, Roland M. Du Bois, Kevin R. Flaherty, Teri J. Franks, David M. Hansell, Thomas E. Hartman, Ella A. Kazerooni, Soon Kim Dong, Masanori Kitaichi, Takashi Koyama, Fernando J. Martinez, Sonoko NagaiDavid E. Midthun, Nestor L. Müller, Andrew G. Nicholson, Ganesh Raghu, Moisés Selman, Athol Wells

Research output: Contribution to journalArticlepeer-review

393 Scopus citations

Abstract

Rationale: The 2002American 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 fromother 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.

Original languageEnglish (US)
Pages (from-to)1338-1347
Number of pages10
JournalAmerican journal of respiratory and critical care medicine
Volume177
Issue number12
DOIs
StatePublished - Jun 15 2008

Keywords

  • High-resolution computed tomography scan
  • Hypersensitivity pneumonitis
  • Lung biopsy
  • Pathology
  • Usual interstitial pneumonia

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine

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