Prognosis of fibrotic interstitial pneumonia: Idiopathic versus collagen vascular disease-related subtypes

Hun Park Joo, Soon Kim Dong, I. Nae Park, Jin Jang Se, Masanori Kitaichi, Andrew G. Nicholson, Thomas V. Colby

Research output: Contribution to journalArticlepeer-review

415 Scopus citations


Background: To investigate whether the better prognosis of interstitial pneumonias associated with collagen vascular disease (CVD) compared with idiopathic interstitial pneumonia (IIP) is due to higher frequency of the nonspecific interstitial pneumonia (NSIP) pattern in CVD, we compared the outcomes of patients from these two groups with the same histopathologic pattern. Subjects: The clinical features and survival of 362 patients (269 with IIP and 93 with CVD) diagnosed using surgical lung biopsy were analyzed. Results: The mean survival of the CVD group (131.0 mo) was longer than that of the IIP group (80.5 mo) (p < 0.0001). The patients with usual interstitial pneumonia pattern among the CVD group (n = 36) was younger, female, and predominantly nonsmoking compared with the IIP group (n = 203). Although baseline lung functions were not significantly different, the CVD group survived longer (mean, 177.0 mo) than the IIP group (mean, 66.9 ± 6.5 mo; p = 0.001). By multivariate analysis, younger age, better pulmonary function, and the presence of a CVD were independent prognostic factors. In NSIP pattern, no significant differences in survival, clinical features, or lung function were found between the two groups. Conclusion: Our data suggest that the better prognosis of patients in the CVD group is not solely due to the predominance of the NSIP pattern. The prognosis of patients with the usual interstitial pneumonia pattern in CVD is better than in those with idiopathic pulmonary fibrosis, despite the same pathologic pattern. In contrast, in those with an NSIP pattern, the prognosis is similar in both groups.

Original languageEnglish (US)
Pages (from-to)705-711
Number of pages7
JournalAmerican journal of respiratory and critical care medicine
Issue number7
StatePublished - Apr 1 2007


  • Collagen vascular disease-interstitial pneumonia
  • Idiopathic interstitial pneumonia
  • Nonspecific interstitial pneumonia
  • Prognosis
  • Usual interstitial pneumonia

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine


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