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Cited by 489

The DOI matches its Crossref record; 34 of the 34 references it cites were found in a registry or an index.

The Prognostic Significance of the Histologic Pattern of Interstitial Pneumonia in Patients Presenting with the Clinical Entity of Cryptogenic Fibrosing Alveolitis

NICHOLSON, ANDREW G., COLBY, THOMAS V., DUBOIS, ROLAND M., HANSELL, DAVID M., WELLS, ATHOL U.·2000·American Journal of Respiratory and Critical Care Medicine
DOI 10.1164/ajrccm.162.6.2003049
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Abstract

Lone cryptogenic fibrosing alveolitis (CFA) is a progressive interstitial lung disease, with a median survival of 3 to 6 yr from the onset of dyspnea. CFA can be subdivided into prognostically significant histopathologic patterns, including nonspecific interstitial pneumonia (NSIP). We reviewed 78 patients with a clinicopathologic diagnosis of CFA, biopsied between 1978 and 1989, to evaluate the prevalence and prognostic significance of these histopathologic patterns, in particular NSIP. Biopsy appearances were reclassified by two pulmonary histopathologists as usual interstitial pneumonia (UIP) (47%), NSIP (36%), or desquamative interstitial pneumonia (DIP)/respiratory bronchiolitis-associated interstitial lung disease (RBILD) (17%). The kappa coefficient of agreement between pathologists was 0.49. In 67 cases, follow-up was complete to death or 10 yr after biopsy, with 50 deaths during a median follow-up of 42 mo (UIP, 89%; NSIP, 61%, DIP/RBILD, 0%). Survival was highest in DIP/RBILD and higher in NSIP than UIP, p < 0.0005. When analysis was confined to patients with UIP or NSIP, the mortality of UIP remained higher, p < 0.01, but the 5-yr survival in patients with fibrotic NSIP was only 45%, indicating that this histologic appearance is often associated with a poor outcome. A response to treatment was more frequent in DIP/RBILD than in NSIP (p < 0.01) or UIP (p < 0.0005). This study confirms the prognostic value of subclassifying patients with CFA according to histopathologic pattern. However, in patients with clinically typical CFA, a histologic diagnosis of fibrotic NSIP needs to be interpreted with caution and does not necessarily denote a good outcome.

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Metadata

Authors
NICHOLSON, A. G., COLBY, T. V., DUBOIS, R. M., HANSELL, D. M., & WELLS, A. U.
Venue
American Journal of Respiratory and Critical Care Medicine
Publisher
Oxford University Press (OUP)
Published
2000-12-01
Volume
162
Issue
6
Pages
2213-2217
ISSN
  • 1073-449X (ISSN-L)
  • 1535-4970
DOI
10.1164/ajrccm.162.6.2003049
OpenAlex
W2018529629
Cited by
489
References
34
FWCI
19.74
Type
journal article
Language
en
Open access
closed

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CoChat. (2026). Citation check: The Prognostic Significance of the Histologic Pattern of Interstitial Pneumonia in Patients Presenting with the Clinical Entity of Cryptogenic Fibrosing Alveolitis. https://app.cochat.ai/papers/10.1164/ajrccm.162.6.2003049
The paper
NICHOLSON, A. G., COLBY, T. V., DUBOIS, R. M., HANSELL, D. M., & WELLS, A. U. (2000). The Prognostic Significance of the Histologic Pattern of Interstitial Pneumonia in Patients Presenting with the Clinical Entity of Cryptogenic Fibrosing Alveolitis. American Journal of Respiratory and Critical Care Medicine. https://doi.org/10.1164/ajrccm.162.6.2003049

Data sources

Bibliographic data: Semantic Scholar, OpenAlex, Crossref.

Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.