The reports of sarcoidosis autopsies in medical journals, meetings and registrations with the Japanese Pathological Society were collected from all over Japan together with those of so-called giant cell myocarditis. Seventy-seven cases were re-examined by one of the authers with the final diagnosis of sarcoidosis for 57 cases, so-called giant cell myocarditis for 13 cases, 3 of which showed insufficient evidence of being sarcoidosis, and of sarcoid reaction for 3 cases. Wheather sarcoidosis or sarcoid reaction was not determined because of lack of informations in 1 case, and the remaining 3 were not judged to be sarcoidosis and therefore excluded from the series.
The average number of sarcoidosis cases autopsied during the 12 years from 1961 to 1972 was 4.2 per year, and the age and sex adjusted rate of sarcoidosis cases among all the autopsy cases was estimated as 29.5 per 100, 000 which far exceeds the prevalence of the disease in Japan in 1968 when it was 4.2 per 100, 000 population. It was presumed that far greater number of sarcoidosis patients exist than these actually diagnosed.
Death from sarcoidosis occurs often in females over 40 years of age, and the ages of all the cases which ranged from 17 to 83 years, showed a plateau from the forties to the sixties. The most frequent cause of death among sarcoidosis patients was myocardial sarcoidosis of which 32 cases died, and death of pulmonary sarcoidosis or brain sarcoidosis were 5 or 4 respectively. Sarcoidosis patients dying of such non-sarcoid diseases as cardiovascular disease or complicating infection were found in 15 cases. The length of period from oncet of the disease to death was found to be shortest in myocardial sarcoidosis (in average 2.3 years) and longest in pulmonary sarcoidosis (in average 10.4 years) with brain sarcoidosis (in average 5.9 years) in between. Clinical diagnose of sarcoidosis were made in 12.5% of the cases died of myocardial sarcoidosis, 4 of 5 pulmonary sarcoidosis and 2 of 4 brain sarcoidosis, illustrating the difficulty of diagnosing myocardial sarcoidosis.
Histological examinations revealed that the lung was the most highly affected organ as were the hilar and mediastinal lymph nodes. The liver and the spleen were the next frequently involved organs, and the myocard was involved in around 30% of the patients who died of non-sarcoid diseases. Progression of the disease may occure either through new dissemination of granulomas or through local continous spread of conglomerated granulomas. Epithelioid cell granuloma appearing in marked lymphocytic infiltration around the capillary vessels was recognized as early lesions in the lung and especially in the brain. Sarcoid reaction was characterized by ill-defined granulomas consist of diffusely proliferated epithlioid cells that sometimes transformed into reticulosis in this series.
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