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Acute Exacerbation of Interstitial Pneumonia Other Than Idiopathic Pulmonary Fibrosis^*

^* From the Division of Pulmonary and Critical Care Medicine (Drs. Park, D.S. Kim, Shim, Lim, Lee, Koh, W.S. Kim, and W.D. Kim) and Department of Pathology (Dr. Jang), College of Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea; and Department of Pathology (Dr. Colby), Mayo Clinic, Scottsdale, AZ.

Correspondence to: Dong Soon Kim, MD, Division of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center, 388–1 Pungnap-2dong, Songpa-gu, Seoul 138–736, Korea; e-mail: dskim{at}amc.seoul.kr

Abstract

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.

Key Words: acute exacerbation • collagen vascular disease • idiopathic nonspecific interstitial pneumonia • surgical lung biopsy