Tuberc Respir Dis TRD Tuberculosis and Respiratory Diseases 1738-3536 2005-6184 The Korean Academy of Tuberculosis and Respiratory Diseases 10.4046/trd.2009.66.2.141 Research Report 2008 National Survey of Idiopathic Interstitial Pneumonia in Korea Scientific Committee of the Korean Academy of Tuberculosis and Respiratory Diseases 02 2009 28 02 2009 66 2 141 151 09 01 2009 09 02 2009 Copyright©2009. The Korean Academy of Tuberculosis and Respiratory Diseases. All rights reserved. 2009 Background

There is limited data on the epidemiology and relative frequency of idiopathic interstitial pneumonia (IIP) worldwide. This survey was performed to assess the epidemiology and relative frequency of IIP in Korea.

 Methods

The patients with IIP and who were confirmed by lung biopsy, except those patients with idiopathic pulmonary fibrosis, (IPF) over a 5 year period (from Jan. 1st, 2003 to Dec. 31st, 2007) were registered by a web-base questionnaire.

 Results

A total of 3,156 cases were registered, but 970 cases were excluded due to duplicative registration, inadequate data and the unmet ATS/ERS diagnostic criteria. A total of 2,186 cases were analyzed. The male to female ratio was about 2 : 1 and their mean age was 65 (range: 11~94). The most frequent disease was IPF (77.1%), followed in decreasing order by nonspecific interstitial pneumonia (NSIP) (11.9%), cryptogenic organizing pneumonia (COP) (8.5%), acute interstitial pneumonia (AIP) (1.1%), desquamative interstitial pneumonia (DIP) (0.9%), respiratory bronchiolitis-interstitial lung disease (RB-ILD) (0.4%) and lymphocytic interstitial pneumonia (LIP) (0.1%). The mean age of the patients with IPF, NSIP and COP was 67.8, 57.1 and 57.7 years old, respectively. The most frequent symptom was dyspnea on exertion (69%) followed by coughing (61%) and sputum (33%) for the whole population. The three year survival rate was 62% for the patients with IPF and the five year survival rate was 85% in both the NSIP and COP patients.

 Conclusion

This survey provides helpful information for the management of IIP and to produce management guidelines for this illness in Korea.

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Age and sex distribution of 2,186 patients with IIP. Generally male predominates female, but female predominate male a little less than 50 year-old. IIP: idiopathic interstitial pneumonias.

Frequency of IIP in Korean population. Percen tages and numbers in square mean relative frequency and population of female and male, respectively. IPF was the most frequent disease in IIP. IIP: idiopathic interstitial pneumonias; AIP: acute interstitial pneumonia; COP: cryptogenic organizing pneumonia; DIP: desquamative interstitial pneumomia; LIP: lymphocytic interstitial pneumonia; NSIP: nonspecific interstitial pneumonia; RB-ILD: respiratory bronchiolitis-intertitial lung disease; IPF: idiopathic pulmonary fibrosis.

Mean age of patients with IIP. The age of patients with IPF was significantly older than those of COP and LIP (p<0.05). Bars mean standard deviation. IIP: idiopathic interstitial pneumonias; AIP: acute interstitial pneumonia; COP: cryptogenic organizing pneumonia; DIP: desquamative interstitial pneumomia; LIP: lymphocytic interstitial pneumonia; NSIP: nonspecific interstitial pneumonia; RB-ILD: respiratory bronchiolitis-intertitial lung disease; IPF: idiopathic pulmonary fibrosis.

Smoking rate in patients with IIP. DIP and RB-ILD are associated with smoking. IIP: idiopathic interstitial pneumonias; AIP: acute interstitial pneumonia; COP: cryptogenic organizing pneumonia; DIP: desquamative interstitial pneumomia; NSIP: nonspecific interstitial pneumonia; RB-ILD: respiratory bronchiolitis-intertitial lung disease; IPF: idiopathic pulmonary fibrosis.

Associated symptoms in patients with IIP. The frequent symptoms were DOE, coughing, and sputum. IIP: idiopathic interstitial pneumonias; DOE: dyspnea on exertion; AIP: acute interstitial pneumonia; COP: cryptogenic organizing pneumonia; NSIP: nonspecific interstitial pneumonia; IPF: idiopathic pulmonary fibrosis.

HRCT findings in patients with IIP. IIP: idiopathic interstitial pneumonias; GGO: ground-glass opacity; AIP: acute interstitial pneumonia; COP: cryptogenic organizing pneumonia; DIP: desquamative interstitial pneumomia; NSIP: nonspecific interstitial pneumonia; RB-ILD: respiratory bronchiolitis-intertitial lung disease; IPF: idiopathic pulmonary fibrosis.

Treatment response in Korean patients with IIP. IIP: idiopathic interstitial pneumonias; AIP: acute interstitial pneumonia; COP: cryptogenic organizing pneumonia; DIP: desquamative interstitial pneumomia; NSIP: nonspecific interstitial pneumonia; RB-ILD: respiratory bronchiolitis-intertitial lung disease; IPF: idiopathic pulmonary fibrosis.

Kaplan-Meier survival curves of idiopathic interstitial pneumonia in Korean population. According to Kaplan-Meier survival curves, 3 year survival rates of AIP, IPF, and DIP were 57%, 62%, and 94%, respectively. Five years survival rates of COP and NSIP were equally 85%. AIP: acute interstitial pneumonia; IPF: idiopathic pulmonary fibrosis; DIP: desquamative interstitial pneumomia; COP: cryptogenic organizing pneumonia; NSIP: nonspecific interstitial pneumonia.

Regional distribution of enrolled hospitals and patients

Associated diseases with IIP

Data are expressed as percentages.

IIP: idiopathic interstitial pneumonias; IPF: idiopathic pulmonary fibrosis; NSIP: nonspecific interstitial pneumonia; COP: cryptogenic organizing pneumonia; AIP: acute interstitial pneumonia.