原发性肺动脉高压症首见于1891年,属少见疾病。1967～1969年瑞士、澳大利亚、西德等国发现应用食欲抑制剂Aminorex后引起本病-过性流行,促使1973年世界卫生组织主办本病的国际会议。1975年日本卫生部组织全国性“原发性肺动脉高压病调查研究班”经过2次全国性流行病学调查,明确本病的实况。本文是1975年和1976年研究结果的总结资料。本病占肺心病例2.1%,未见地区性特征。年龄从出生后第3天至66岁,20～30岁最多见,女性好发,男:女=1∶2.3,15岁以下为1∶4.3。最多的初发症状是劳力性气喘,占81.8%,其次是易疲劳,占38.8%。尚有痰血,下肢浮肿,Raynaud’s现象,晕 Primary pulmonary hypertension seen in 1891, is a rare disease. 1967 ~ 1969 Switzerland, Australia, West Germany and other countries found that the application of the appetite suppressant Aminorex caused the disease - the epidemic, prompting the 1973 World Health Organization to host the international conference on the disease. In 1975, Japan’s Ministry of Health organized a national “primary pulmonary hypertension research class” after two national epidemiological surveys to confirm the actual situation of the disease. This article summarizes the findings of the studies in 1975 and 1976. The disease accounted for 2.1% of pulmonary heart disease, no regional characteristics. From the age of 3 days after birth to 66 years old, 20 to 30 years of age the most common, women are fat, male: female = 1: 2.3, 15 years old was 1: 4.3. The first symptom of onset was labored asthma, accounting for 81.8%, followed by fatigue, accounting for 38.8%. There are sputum blood, lower extremity edema, Raynaud’s phenomenon, halo