Chest
Volume 83, Issue 1, January 1983, Pages 63-69
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Clinical Investigations
Diffuse Panbronchiolitis: A Disease of the Transitional Zone of the Lung

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Diffuse panbronchiolitis (DPB) is a disease with chronic inflammation exclusively located in the region of respiratory bronchioles. The pathologic features of the disease are characterized by thickening of the wall of the respiratory bronchiole with infiltration of lymphocytes, plasma cells and histiocytes, and extension of the inflammatory changes toward peribronchiolar tissues. In the advanced stage, secondary ectasia of proximal bronchioli may occur. These changes appear as diffusely disseminated small nodular shadows throughout both lungs on the chest roentgenogram. Obstructive respiratory functional impairment, occasional symptoms of wheezing, and also cough and sputum resemble the feature of emphysema, bronchial asthma, or chronic bronchitis, respectively. In the advanced stage, large amounts of purulent sputum and dilatation of proximal terminal conducting bronchioli resemble bronchiectasis. However, diffuse panbronchiolitis belongs to a distinctly different category from these diseases, and should be distinguished from them, because it may often show rapid progression with fatal outcome. The disease is dominant in males and the onset is unrelated to age. More than 1,000 cases of probable diffuse panbronchiolitis and 82 histologically-confirmed cases have been collected in Japan.

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Case 1

A 64-year-old man was admitted because of worsening nocturnal cough, sputum, and exertional dyspnea in 1973. He had been well until ten years prior to this admission when he noticed productive cough. Since 1967. he experienced exertional dyspnea, which gradually progressed. Physical examination on admission revealed cyanosis and orthopnea. Blood pressure was 110/62 mm Hg; pulse rate. 104 beats per minute and regular; respiratory rate. 27 per minute; and body temperature, 36.2°C. The chest was

Results

Through clinical, radiologic, physiologic, and pathologic analyses based on previously accumulated data since 1969 by Homma and Yamanaka,1, 2, 3, 4, 5, 6, 7, 8 a nationwide survey with the cooperation of 30 universities, national, and municipal institutions throughout the country was organized to study the incidence and the morbidity of the disease through the aid of the Ministry of Health and Welfare of Japan.9

In this survey, the clinical diagnostic criteria for this disease were as

Discussion

The typical pathologic features of the lesions of diffuse panbronchiolitis are thickening of the walls of the respiratory bronchiole with infiltration of lymphocytes, plasma cells, and histiocytes, and extension of these inflammatory changes toward the peribronchiolar tissues as shown in Figures 2 and 5. In the advanced stage, narrowing and constriction of respiratory bronchioli by infiltration of these cells, proliferation of lymph-follicles, and accumulation of foamy cells within the wall and

Acknowledgment

This work was supported by a grant from the Ministry of Health and Welfare of Japan.

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Manuscript received March 19; revision accepted July 30

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