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Prognosis of Fibrotic Interstitial Pneumonia

Idiopathic versus Collagen Vascular Disease–related Subtypes

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

Correspondence and requests for reprints should be addressed to Dong Soon Kim, M.D., Division of Pulmonary and Critical Care Medicine, Asan Medical Center, College of Medicine, University of Ulsan, 388-1, Poongnap-dong, Songpa-gu, Seoul, South Korea. E-mail: dskim{at}amc.seoul.kr

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.

Key Words: prognosis • collagen vascular disease–interstitial pneumonia • idiopathic interstitial pneumonia • usual interstitial pneumonia • nonspecific interstitial pneumonia

AT A GLANCE COMMENTARY Scientific Knowledge on the Subject Even though interstitial pneumonia associated with collagen vascular disease has a better prognosis than idiopathic pulmonary fibrosis, it is not certain whether this is due to the predominance of a nonspecific interstitial pneumonia pattern or to a genuine difference in the same pathologic pattern. What This Study Adds to the Field Patients with collagen disease–usual interstitial pneumonia had significantly better survival than those with idiopathic usual interstitial pneumonia–idiopathic pulmonary fibrosis. In patients with a nonspecific interstitial pneumonia pattern, no significant difference was noted between the two groups.

 

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