慢性間質性コレステロール肺炎

抄録

Three cases of chronic cholesterol pneumonitis were experienced in the Okura National Hospital in the years from 1963 to 1967. They were Japanese male and aged 34, 39 and 47 years old. Initial complaints were fever and persistent hemosputum. The chest rentgenograms revealed that diffuse opacity of the lungs increased subacutely in the course of the disease in two cases but in one case it remained stationary. Laboratory findings showed anemia, increased serum gamma globulin and moderately reduced A/G ratio. One of them was remarkably severe. He was admitted to this hospital three weeks after the onset of preceding fever and hemosputum which continued until his death. The right middle and lower lobe lobectomy was performed two month after admission and enough amount of antibiotics were given. He expired by sudden massive hemoptysis. In this case the pulmonary vein (r V 3) was ruptured and right pleural cavity was filled with blood.
Other two cases recoverd well after the operation, one by middle lobe lobectomy and another by pneumonectomy.
The bacteriological examination of sputum or discharge from the resected specimens revealed no predominant causative bacteria and fungus.
In general pathological examinations of the affected lesions showed pneumonic consolidation of lung tissue with the diffuse discoloration of bright golden yellow on cut surface and partial necrosis in all cases. Golden yellow discolored pneumonic lesions were composed of fibrous thickening of alveolar septa involving moderate lymphocytic infiltration and aggregation of histiocytes. Alveolar lumina were filled with innumerable foamy macrophages which contained lipid substance, showing bright with oil-red staining, bluish gray with Baker's phospholipid staining and double refraction by Nicol's prism.
The necrotizing area of pneumonic lesion were found closed to the obstructive and suppurant bronchitis with the destruction of the wall and surrounding parenchyma.
These findings suggested that the disease began with the proliferative alveolitis followed by the appearance of lipid laden macrophages in the alveolar lumina. Cholesterol pneumonitis was accomplished and later replaced by dense fibrous tissue with time, but the obstructive bronchitis led to the destruction of pneumonic lesion as a secondary affection.
The pathogenesis of this disease is still open question, but it is likely that the bacteria would be excluded from the pathogenesis of the disease.