This Article Order Full text via Infotrieve Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Fukuda, Y. Articles by Crystal, R. G. Search for Related Content PubMed PubMed Citation Articles by Fukuda, Y. Articles by Crystal, R. G.

American Journal of Pathology, Vol 137, 415-424, Copyright © 1990 by American Society for Investigative Pathology

REGULAR ARTICLES

Intraluminal fibrosis and elastic fiber degradation lead to lung remodeling in pulmonary Langerhans cell granulomatosis (histiocytosis X)

Y Fukuda, F Basset, P Soler, VJ Ferrans, Y Masugi and RG Crystal
Department of Pathology, Nippon Medical School, Tokyo, Japan.

To evaluate the morphogenesis of lung remodeling in pulmonary Langerhans cell granulomatosis (LCG; previously called histiocytosis X or eosinophilic granuloma), lung tissues obtained by open biopsy from 62 patients with pulmonary LCG were studied by light and electron microscopy. Tissues from 20 patients were also studied by immunohistochemical methods for the detection of fibronectin, elastin, and S-100 protein, and samples from six patients were studied using OKT6 monoclonal antibody. In early stages of pulmonary LCG, the epithelial lining cells were detached and Langerhans cells, inflammatory cells, and myofibroblasts migrated into intraluminal spaces through gaps in the epithelial basement membranes in and around the granulomatous lesions. In late stages, intraluminal fibrosis led to obstruction of alveolar spaces and airways and to coalescence of alveolar walls in and around the granulomatous lesions. Adjacent to these lesions, irregularly dilated alveoli were found with degraded and disrupted elastic fibers. Together, these observations suggest that intraluminal fibrosis and elastic fiber degradation are important processes of lung remodeling in pulmonary LCG.

This article has been cited by other articles:

				E. Roger Parra, R. Adib Kairalla, C. R. R. de Carvalho, and V. L. Capelozzi Abnormal deposition of collagen/elastic vascular fibres and prognostic significance in idiopathic interstitial pneumonias Thorax, May 1, 2007; 62(5): 428 - 437. [Abstract] [Full Text] [PDF]

				J. L. Myers and M.-C. Aubry Pulmonary Langerhans Cell Histiocytosis: What Was the Question? Am. J. Respir. Crit. Care Med., December 1, 2002; 166(11): 1419 - 1421. [Full Text] [PDF]

				Y. Hirasawa, N. Kohno, A. Yokoyama, Y. Inoue, M. Abe, and K. Hiwada KL-6, a Human MUC1 Mucin, Is Chemotactic for Human Fibroblasts Am. J. Respir. Cell Mol. Biol., October 1, 1997; 17(4): 501 - 507. [Abstract] [Full Text]