Kitamura Nobutaka, Ohkouchi Shinya, Tazawa Ryushi, Ishii Haruyuki, Takada Toshinori, Sakagami Takuro, Tanaka Takahiro, Nakata Koh
Clinical and Translational Research Center, Niigata University Medical and Dental Hospital, Niigata, Japan.
Dept of Respiratory Medicine and Dept of Occupational Health, Tohoku University Graduate School of Medicine, Sendai, Japan.
ERJ Open Res. 2019 Mar 18;5(1). doi: 10.1183/23120541.00190-2018. eCollection 2019 Feb.
The incidence and prevalence of autoimmune pulmonary alveolar proteinosis in Japan were previously estimated to be 0.49 and 6.2 per million, respectively. Thereafter, an increase in serological diagnosis forced a re-estimation of the incidence based on more contemporaneous data using more robust methods. Sera of 702 patients were positive for granulocyte-macrophage colony-stimulating factor autoantibody during the 2006-2016 period (group A). Of these patients, 43 were actively surveyed in Niigata prefecture (group B) for estimation of the incidence. To estimate the survival period, 103 patients (group C) were investigated retrospectively for the 1999-2017 period using restricted mean survival time. In group A, the number of patients diagnosed in each prefecture was closely correlated with the corresponding population, indicating no regional integration of onset. In group B, a total of 43 patients were diagnosed, the annual number followed a Poisson distribution and the incidence was thus estimated to be 1.65 per million. In group C, the retrospective cohort study revealed the mean survival period to be 16.1 years. Taken together, the prevalence was estimated to be 26.6 per million, indicating that the previous data for incidence and prevalence was an underestimation.
日本自身免疫性肺泡蛋白沉积症的发病率和患病率此前估计分别为每百万人口0.49例和6.2例。此后，血清学诊断的增加促使人们基于更及时的数据和更可靠的方法对发病率进行重新估计。在2006 - 2016年期间，702例患者的血清中粒细胞 - 巨噬细胞集落刺激因子自身抗体呈阳性（A组）。在这些患者中，43例在新潟县接受了主动调查（B组）以估计发病率。为了估计生存期，对1999 - 2017年期间的103例患者（C组）进行回顾性研究，采用受限平均生存时间进行分析。在A组中，每个县诊断出的患者数量与相应人口密切相关，表明发病无区域聚集性。在B组中，共诊断出43例患者，每年的病例数呈泊松分布，因此发病率估计为每百万人口1.65例。在C组中，回顾性队列研究显示平均生存期为16.1年。综合来看，患病率估计为每百万人口26.6例，这表明之前的发病率和患病率数据被低估了。
