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Published ahead of print on January 11, 2007, doi:10.1164/rccm.200607-912OC

Am. J. Respir. Crit. Care Med., Volume 175, Number 7, April 2007, 705-711

A more recent version of this article appeared on April 1, 2007
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Submitted on July 5, 2006
Accepted on January 11, 2007

Prognosis of Fibrotic Interstitial Pneumonia: Idiopathic Versus Collagen Vascular Disease-Related

Joo Hun Park1, Dong Soon Kim1*, I-Nae Park1, Se Jin Jang2, Masanori Kitaichi3, Andrew G Nicholson4, and Thomas V Colby5

1 Division of Pulmonary and Critical Care Medicine, Department of Medicine, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea, Republic of, 2 Department of Pathology, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea, Republic of, 3 Laboratory and Anatomic Pathology, National Hospital Organziation Kinki-chuo Chest Medical Center, Osaka, Japan, 4 Department of Pathology, Brompton Hospital, London, United Kingdom, 5 Department of Pathology, Mayo Clinic, Scottsdale, Arizona, USA

* To whom correspondence should be addressed. E-mail: dskim{at}amc.seoul.kr.

Background: To investigate whether the better prognosis of interstitial pneumonias associated with collagen vascular disease compared to idiopathic interstitial pneumonias is due to higher frequency of non-specific interstitial pneumonia pattern, we compared the outcomes of patients from these two groups in the same histopathologic pattern. Subjects: The clinical features and survival of total 362 patients (269 idiopathic and 93 collagen vascular disease) diagnosed by surgical lung biopsy were analyzed. Result: (1) As a whole group, mean survival of collagen vascular disease group (131.0 months) was longer than idiopathic group (80.5 months) (p< 0.0001). (2) In usual interstitial pneumonia pattern, collagen vascular disease group (n=36) were younger, female, and non-smoker dominant compared to idiopathic group (n=203). Although baseline lung functions were not significantly different, collagen vascular disease group survived longer (mean: 177.0 months) than the idiopathic group (mean: 66.9 ± 6.5 months, p=0.001). By multivariate analysis, younger age, better pulmonary function, and the presence of a collagen vascular disease were independent prognostic factors. (3) In nonspecific interstitial pneumonia pattern, no significant differences in survival, clinical features or lung function were found between two groups. Conclusion: Our data suggest that the better prognosis of patients in the collagen vascular disease group is not solely due to the predominance of nonspecific interstitial pneumonia pattern. The prognosis of patients with usual interstitial pneumonia pattern in collagen vascular disease is better than idiopathic pulmonary fibrosis, despite the same pathologic pattern. In contrast, in non-specific interstitial pneumonia pattern, the prognosis is similar.
Key words: Prognosis, Collagen Vascular Disease-Interstitial Pneumonia, Idiopathic Interstitial Pneumonia, Usual Interstitial Pneumonia, Nonspecific Interstitial Pneumonia




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