貝殼細工人にみられた慢性間質性肺線維症の一例

抄録

A case of chronic interstitial pulmonary fibrosis in a seventy two year old male was presented with pertinent clinico-pathological findings.
The patient had worked in a shell ornament firm over fifty years when he developed exertional dypnea, cough and yellowish sputum. The chest X ray film taken at the time of his first visit to this clinic already showed diffuse bilateral linear and reticular shadow with partial conggregation. Pulmonary function study disclosed marked restrictive ventilatory disturbance and impairment of diffusion with mild hypoxemia. Dyspnea became progressively worse with each episode of recurrent respiratory infection, although supportive treatment with various antibiotics, bronchodilators and oxygen administration brought him temporal amelioration of symptoms. The patient eventually died of pulmonary insufficiency and heart failure secondary to intractable respiratory infection three years after the initial examination.
Cardinal pathologic findings were chronic interstitial pneumonitis and cor plumonale which were compatible with clinical and laboratory data. Microscopic findings of the lung consisted of variable degrees of hyaline membrane formation, interstitial fibrosis and edema, chronic cellular infiltation, bronchiolectasis and abnormal proliferation of bronchiolar epithelium. These pathological changes were taken as rather indicative of chronic pneumonitis than established fibrosis. The significance of the patient's longstanding exposure to the shell dust was discussed in view of the etiology of so-called idiopathic interstitial pulmonary fibrosis and postulation for possible mechanism of its contribution to the development of those pathologic findings was made.