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Idiopathic and collagen vascular disease nonspecific interstitial pneumonia: clinical significance of remodeling process.

Abstract：

:Recently, active remodeling may indicate a good prognosis in idiopathic interstitial pneumonias. In this study we sought to validate the importance of the collagen/elastic system in the extracellular matrix remodeling and to study the relationships between the collagen/elastic system in nonspecific interstitial idiopathic pneumonia (NSIP) and collagen vascular disease associated with nonspecific interstitial idiopathic pneumonia (CVD-NSIP). We examined collagen/elastic system fibers in open lung biopsies of 20 idiopathic NSIP and 21 CVD-NSIP patients. The clinical features were analyzed with respect to age, gender, pulmonary functional tests, chest X-ray and computed tomography, treatment, and survival. We used the picrosirius polarization method and Weigert's resorcin-fuchsin histochemistry and morphometric analysis to evaluate the amount of collagen/elastic system fibers and their association with the NSIP histologic pattern. No differences in clinical features and pulmonary function tests were observed between idiopathic NSIP and CVD-NSIP, but a significantly higher collagen and elastic fiber proliferation was detected in CVD-NSIP lungs and fibrosing NSIP histologic pattern. Multivariate Cox model analysis demonstrated that sex and quantitative elastic fiber staining added important prognostic information (p=0.01) and was indicative of a worse prognosis than collagen staining. A cut point at the mean staining of 1.5% for elastic fibers divided the patients into two groups with distinctive survival times. Those with elastic fibers greater than 1.5% had a median survival time of just 52 months. We concluded that idiopathic NSIP and CVD-NSIP were clinically similar but pathologically different, suggesting that different remodeling profiles in NSIP may represent evolutionary adapted responses to injury grade, which depend, at least in part, on the extent of elastic extracellular matrix deposition. Patients with greater than 1.5% of elastic fibers comprise a subset with a high risk for dying due to NSIP and may be an appropriate target for prospective studies.

journal_name

Lung

journal_title

Lung

authors

Felício CH,Parra ER,Capelozzi VL

doi

10.1007/s00408-006-0104-2

subject

Has Abstract

pub_date

2007-01-01 00:00:00

pages

39-46

issue

eissn

0341-2040

issn

1432-1750

journal_volume

185

pub_type

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Idiopathic and collagen vascular disease nonspecific interstitial pneumonia: clinical significance of remodeling process.