Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project. Conference Paper Review uri icon

Overview

MeSH

  • Adult
  • Aged
  • Diagnosis, Differential
  • Female
  • Humans
  • Male
  • Middle Aged
  • Prognosis
  • Sex Distribution
  • Survival Rate
  • Tomography, X-Ray Computed

MeSH Major

  • Lung Diseases, Interstitial

abstract

  • 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. 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? 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. 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%. 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.

publication date

  • June 15, 2008

has subject area

  • Adult
  • Aged
  • Diagnosis, Differential
  • Female
  • Humans
  • Lung Diseases, Interstitial
  • Male
  • Middle Aged
  • Prognosis
  • Sex Distribution
  • Survival Rate
  • Tomography, X-Ray Computed

Research

keywords

  • Consensus Development Conference
  • Journal Article
  • Review

Identity

Language

  • eng

Digital Object Identifier (DOI)

  • 10.1164/rccm.200611-1685OC

PubMed ID

  • 18388353

Additional Document Info

start page

  • 1338

end page

  • 1347

volume

  • 177

number

  • 12