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Idiopathic Nonspecific Interstitial Pneumonia: Report of an American Thoracic Society Project.
Travis, William D. 1,*; Hunninghake, Gary 2,*; King, Talmadge E. Jr. 3,*; Lynch, David A. 4,*; Colby, Thomas V. 5,*; Galvin, Jeffrey R. 6,*; Brown, Kevin K. 7; Chung, Man Pyo 8; Cordier, Jean-Francois 9; du Bois, Roland M. 10; Flaherty, Kevin R. 11; Franks, Teri J. 12; Hansell, David M. 13; Hartman, Thomas E. 14; Kazerooni, Ella A. 15; Kim, Dong Soon 16; Kitaichi, Masanori 17; Koyama, Takashi 18; Martinez, Fernando J. 11; Nagai, Sonoko 19; Midthun, David E. 20; Muller, Nestor L. 21; Nicholson, Andrew G. 22; Raghu, Ganesh 23; Selman, Moises 24; Wells, Athol 10
[Miscellaneous] American Journal of Respiratory & Critical Care Medicine. 177(12):1338-1347, June 15, 2008.

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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.