Diffuse interstitial fibrosis of the lung of unknown etiology was studied in cases detected in mass surveys and at autopsiy throughout Japan to clarify the epidemiological features of the disease.
1) Cases showing bilateral diffuse fibrous shadows were selected from 1978 mass survey chest X-ray films, excluding the following cases: Cases suspected to have COPD due to the presence of long-standing sputum and/or abnormal FEV 1.0%, cases with a history of pneumoconiosis and the cases with collagen disease. The remaining cases were considered to haves idiopathic diffuse interstitial fibrosis.
The age and sex distribution of these cases showed high prevalence in the age group older than 50 years, showing a peak in 80 and male incidence was around 10 times that of females (Table 1).
The prevalence was highest in Nagano, Okayama and Ehime prefectures, and it was a striking fact that the highest prevalence was found in rural areas, the lowest incidence was observed in the large cities and other cities showed intermediate values. Significant difference was detected statistically between prevalence in the rural and the combined urban areas (Table 3). The follow up study carried out on 27 cases one year later, revealed increase of the shadow in 5 cases and appearance of lung cancer in 2 cases.
2) Autopsy records collected from throughout Japan during the period from 1975 to 1977 were analyzed. Age and sex distribution of the autopsy cases which were diagnosed as idiopathic diffuse interstitial pneumonia and fibrosis of the lung were somewhat different from those found in mass surveys, showing younger age distribution and more frequent female incidence. This difference was suspected to be partly due to the predominance of young and female cases in the acute and subacute cases which were included in the autopsy series (Table 5). The rate of idiopathic interstitial fibrosis of the lung per 10, 000 autopsies in each district demonstrated higher rates in Hokuriku, Chugoku and Koshinetsu districts, 8.8, 7.5 and 5.7 respectively, than the average rate in Japan, 4.4, and presumed rate of the death per 100, 000 population in each district showed similar tendencies (Table 6). The rate of the disease per 10, 000 autopsy in the 10 largest cities showed 3.7, in the other cities, 4.9 and in the rural area, 5.7, showing significant difference (P<0.05) between the largest cities and rural ares. The rates per 100, 000 population were 1.9, 2.8 and 4.8 respectively (Table 7).
It can be concluded that there are more cases of idiopathic diffuse interstitial fibrosis of the lung in rural than in the urban areas, showing the necessity of further epidemiological studies on this problem.
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