Journal Article

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

Kyoto University, Hansell, David M, University of Maryland [College Park] (UMD) ; University System of Maryland, Armed Forces Institute of Pathology, Flaherty, Kevin R, University of Ulsan, Mayo Clinic, Department of Radiology ; Mayo Clinic, Du Bois, Roland M, Nicholson, Andrew G, National Jewish Medical and Research Center, Hunninghake, Gary, National Hospital Organization Kinki-chuo Chest Medical Center ; Partenaires INRAE, Brown, Kevin K, Hartman, Thomas E, Lynch, David A, Kim, Dong Soon, Midthun, David E, Royal Brompton and Harefield NHS Foundation Trust, Kitaichi, Masanori, School of Medicine, Department of Medicine ; University of California [San Francisco] (UC San Francisco) ; University of California (UC)-University of California (UC), Hospices Civils de Lyon (HCL), Iowa State University (ISU), Colby, Thomas V, Martinez, Fernando J, Nagai, Sonoko, Rétrovirus et Pathologie Comparée (RPC) ; Institut National de la Recherche Agronomique (INRA)-École Pratique des Hautes Études (EPHE) ; Université Paris Sciences et Lettres (PSL)-Univer

2008

Overview

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.

• Publisher: American Thoracic Society,CCSD,Am Thoracic Soc,American Lung Association,Oxford University Press
• Subject: Adult / Aged / Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy / Biological and medical sciences / Diagnosis, Differential / Female / Humans / Intensive care medicine / Investigative techniques, diagnostic techniques (general aspects) / Life Sciences / Lung Diseases, Interstitial - diagnosis / Lung Diseases, Interstitial - diagnostic imaging / Lung Diseases, Interstitial - epidemiology / Lung Diseases, Interstitial - pathology / Male / Medical sciences / Middle Aged / Prognosis / Radiodiagnosis. Nmr imagery. Nmr spectrometry / Respiratory system / Sex Distribution / Survival Rate / Tomography, X-Ray Computed
• ISBN: 0002565497000