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The prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis.
Am J Respir Crit Care Med. 2000 Dec; 162(6):2213-7.AJ

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.

Authors+Show Affiliations

Department of Histopathology, Interstitial Lung Disease Unit, Royal Brompton Hospital, London, United Kingdom. a.nicholson@rbh.nthames.nhs.ukNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article

Language

eng

PubMed ID

11112140

Citation

Nicholson, A G., et al. "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, vol. 162, no. 6, 2000, pp. 2213-7.
Nicholson AG, Colby TV, du Bois RM, et al. The prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med. 2000;162(6):2213-7.
Nicholson, A. G., Colby, T. V., du Bois, 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, 162(6), 2213-7.
Nicholson AG, et al. The Prognostic Significance of the Histologic Pattern of Interstitial Pneumonia in Patients Presenting With the Clinical Entity of Cryptogenic Fibrosing Alveolitis. Am J Respir Crit Care Med. 2000;162(6):2213-7. PubMed PMID: 11112140.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis. AU - Nicholson,A G, AU - Colby,T V, AU - du Bois,R M, AU - Hansell,D M, AU - Wells,A U, PY - 2000/12/9/pubmed PY - 2001/2/28/medline PY - 2000/12/9/entrez SP - 2213 EP - 7 JF - American journal of respiratory and critical care medicine JO - Am J Respir Crit Care Med VL - 162 IS - 6 N2 - 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. SN - 1073-449X UR - https://www.unboundmedicine.com/medline/citation/11112140/The_prognostic_significance_of_the_histologic_pattern_of_interstitial_pneumonia_in_patients_presenting_with_the_clinical_entity_of_cryptogenic_fibrosing_alveolitis_ L2 - https://www.atsjournals.org/doi/10.1164/ajrccm.162.6.2003049?url_ver=Z39.88-2003&amp;rfr_id=ori:rid:crossref.org&amp;rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -