Acute exacerbation of interstitial pneumonia other than idiopathic pulmonary fibrosis

I. Nae Park, Dong Soon Kim, Tae Sun Shim, Chae Man Lim, Sang Do Lee, Younsuck Koh, Woo Sung Kim, Won Dong Kim, Se Jin Jang, Thomas V. Colby

Research output: Contribution to journalArticlepeer-review

248 Scopus citations


Backgrounds: Acute exacerbation (AE) in idiopathic pulmonary fibrosis (IPF) is increasingly recognized as a relatively common and highly morbid clinical event. However, clinical data on AE in non-IPF interstitial pneumonia are sparse. This study was performed to find the frequency, clinical features, and outcome of AE in non-IPF interstitial pneumonia. Methods: Retrospective analysis of 10 patients who satisfied the modified Akira criteria for AE during follow-up of 74 patients with surgical lung biopsy-confirmed idiopathic nonspecific interstitial pneumonia (I-NSIP) and 93 patients with biopsy-confirmed interstitial pneumonia associated with collagen vascular disease (CVD-IP). Results: AE occurred in six patients with I-NSIP (1-year frequency, 4.2%) and in four patients with CVD-IP (rheumatoid arthritis [RA], n = 3; scleroderma, n = 1), with 1-year frequency of 3.3%. Median age was 58 years (range, 47 to 75); six patients were female. AE occurred in two patients immediately after surgical biopsy. Median duration of acute symptom before hospital admission was 10 days (range, 1 to 30). Median ratio of PaO2 to the fraction of inspired oxygen (FIO2) was 172 (range, 107 to 273), and PaO2/FIO2 ratio was < 200 in six patients. Surgical lung biopsy performed at the time of AE in two patients revealed diffuse alveolar damage superimposed on nonspecific interstitial pneumonia pattern. Four patients with I-NSIP survived to discharge and were followed up for 24 months (range, 6 to 121). Conclusion: AE occurred in the patients with I-NSIP with apparently better prognosis. In patients with CVD-IP, AE occurred mostly with RA-usual interstitial pneumonia in our small series with poor outcome.

Original languageEnglish (US)
Pages (from-to)214-220
Number of pages7
Issue number1
StatePublished - Jul 2007


  • Acute exacerbation
  • Collagen vascular disease
  • Idiopathic nonspecific interstitial pneumonia
  • Surgical lung biopsy

ASJC Scopus subject areas

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


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