Chest
Volume 132, Issue 1, July 2007, Pages 214-220
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ORIGINAL RESEARCH
INTERSTITIAL LUNG DISEASE
Acute Exacerbation of Interstitial Pneumonia Other Than Idiopathic Pulmonary Fibrosis

https://doi.org/10.1378/chest.07-0323Get rights and content

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.

Section snippets

Diagnostic Criteria

For the diagnosis of I-NSIP and the individual pattern of interstitial pneumonia, we used the American Thoracic Society/European Respiratory Society consensus classification, and individual CVDs were diagnosed according to the criteria of each society.19202122232425 For the diagnosis of AE, we used the Akira criteria7: (1) subjective worsening of dyspnea within the last 1 month; (2) new ground-glass opacities or consolidation on chest radiograph or high-resolution CT (HRCT); (3) hypoxemia with

Frequency of AE

Among 74 patients with surgical lung biopsy-confirmed fibrotic I-NSIP, the estimated 1-year frequency of AE was 4.2%. The estimated 1-year frequency in surgical lung biopsy-confirmed CVD-IP (n = 93) was 3.3% (Fig 1); however, it was 5.6% among the patients with CVD-UIP (n = 36) and 11.1% in RA-UIP (n = 18).

Demographic and Clinical Features

Demographic and clinical characteristics are shown in Table 1. Median age at the time of AE was 58 years (range, 47 to 75); six patients (60%) were female. Two patients (I-NSIP, n =

Discussion

Our experience showed that AE occurred in the patients with I-NSIP and CVD-IP. The estimated 1-year frequency of AE in our series was 4.2% in surgical lung biopsy-confirmed I-NSIP, 3.3% in CVD-IP, 5.6% in surgical biopsy-proven CVD-UIP, and 11.1% in RA-UIP. The clinical, radiologic, and pathologic features of our patients were similar to that of IPF. The exact frequency of AE in IPF is not known, and the reported numbers vary widely because of the difference in study design, and also due to the

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    This work was performed in the Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center.

    All of the authors contributed substantially to this work and are all responsible for the content of the article. The authors have no conflicts of interest in association with this article to disclose.

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