The purpose of this paper is to descrive the typical features of clinical and pathological findings in patients with diffuse panbronchiolitis and how the author and his colleagues have come to reach their conclusion that this disease is a definite clinical and pathological entity, based on the data which have been accumulated in the last fifteen years by them.
The study is based on 39 cases, composed of 29 males and 10 females, or 10 autopsied cases and 29 strictly selected cases under observation at least more than 18 months including open-lung biopsied cases. The first autopsied case appeared in 1960.
1. Sex ratio: There are 29 males a 10 females giving the ratio ca 3 to 1.
2. Age of onset: Most of 39 cases are in thier fifties and sixties, but age of onset is variable from teens to old ages.
3. Mode of onset: Usually insidious. Initial symptoms are cough, tenacious sputum and shortness of breath, frequently accompanied by wheezing. More than 90% of the cases complain shortness of breath indicating early occurance of hypoxemia.
4. Smoking: No close relationship with the habit of smoking is noted.
5. Diagnosis prior to admission: Bronchial asthma, bronchiectasis, chronic bronchitis and pulmonary emphysema are most frequently suspected or diagnosed.
6. Complication: Over 70% of the cases have or once had chronic sinusitis.
7. Clubbed finger: 34% of the cases show clubbing of the digits on admission.
8. Sputum bacteriology: Hemophilus influenzae and Pneumococci are most often identified in the initial stage, later followed by Pseudomonas superinfection.
9. Chest x-ray findings: Miliary or fine nodular dissemination in both lung fields is characteristic picture, often accompanied by overinflation which makes the dissemination defficult to be seen. Bronchogram frequently shows slight dilatation of the terminal bronchioles which indicate the obstruction of the respiratory bronchioles.
10. Pulmonary function: Mixed ventilatory impairment consisted of slight restrictive and marked obstructive disturbance, early hypoxemia later accompanied by hypercapnea and cor pulmonale, and increase in closing volume.
11. Prognosis: Missing of early diagnosis and early treatment with corticosteroids may be followed by relentless progressive pulmonary insufficiency accerelated by pseudomonas pulmonary infection.
12. Pathology: The inflammatory lesions arising in the region of respiratory bronchioles are primary and essential pathological picture, These are noticed diffusely in both lungs. The lesions are composed of bronchiolitis and perbronchiolitis which are classified into two types; obliterative type charaeterized by obstruction of the lumina with granulation tissues and intra-mural proliferative type characterized by the marked round-cell infiltration with new growth of lymph follicles.
In conclusion, the description of diffuse panbronchiolitis at the present moment is as follows: a chronic inflammation confined in the region of respiratory bronchioles, namely, in the intermediate zone between the terminal end of the small airway and the pulmonary parenchyma, spreaded widely in the both lungs accompanying distinctive respiratory disturbance including blood gas impairment. Pathological lesions are usually made of a combination of bronchiolitis and peribronchiolitis, which is classified into two types: intramural proliferative type and obliterative type respectively. Diffuse panbronchiolitis may be called as a disease of the transit zone in the lung.
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