Acute Exacerbation of Non-Specific Interstitial Pneumonia Compared to That of Usual Interstitial Pneumonia
Atsushi Miyamoto, Amita Sharma, Michiya Nishino, Mari Mino-Kenudson, Eugene J Mark. Toranomon Hospital, Tokyo, Japan; Massachusetts General Hospital, Boston, MA
Background: Usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia (NSIP) are related forms of interstitial lung disease. Acute exacerbation (AE) is a recognized complication of UIP but is much less appreciated in NSIP.
Design: To compare the features of acute exacerbation in UIP with those of NSIP, we performed a detailed retrospective analysis of 42 patients from a single institution who had an initial pathologic diagnosis of UIP or NSIP. All patients were discussed at a multidisciplinary conference (MDC) involving pathologists, a pulmonologist, and a radiologist.
Results: Of the 42 patients initially diagnosed as UIP or NSIP, 15 were determined to have a disease other than UIP or NSIP at the MDC, leaving 27 cases for further analysis. The MDC classified the remaining cases as having stable UIP (n=4), acute exacerbation of UIP (AE-UIP, n=6), stable NSIP (n=6), and acute exacerbation of NSIP (AE-NSIP, n=7). Four patients with UIP were transplanted and classified as indeterminate for AE because the timing of symptoms was unclear. Characteristic histologic features of AE were more numerous in patients who were classified as having AE-NSIP compared to AE-UIP.
Conclusions: Patients with AE-NSIP have qualitatively similar but quantitatively greater pathology as patients with AE-UIP. This finding suggests similarities in the pathogenesis of NSIP and UIP and supports our hypothesis that the more severely scarred lungs in UIP has less reserve than those of NSIP and may be more prone to acute clinical deterioration after a lesser degree of acute lung injury.
Tuesday, March 5, 2013 1:00 PM
Poster Session IV # 285, Tuesday Afternoon