BACKGROUND Pulmonary alveolar proteinosis (PAP) is a very rare lung disease and its prevalence and incidence remain unclear. The prevalence and incidence of PAP were investigated by using nationwide claims data from the Korean Health Insurance Review and Assessment service. METHODS Data were extracted for adults who visited any secondary or tertiary medical institute between 2010 and 2016 with the PAP-related Korean Classification of Disease, 7th edition code J84.0 and the Rare Intractable Disease exempted calculation code V222. To robust case definition, a narrow case definition was made when all following factors were met: 1) more than two PAP-coded visits within 1 year of the first claim, and 2) more than one claim for both chest computed tomography and diagnostic procedures (bronchoscopy or surgical lung biopsy) within 90 days before or after the first claim. RESULTS A total of 182 patients (narrow, n = 82) with PAP-related codes were identified from 2010 to 2016 and 89 new patients (narrow, n = 66) visited medical institutes between 2012 and 2015. The prevalence of PAP was 4.44 (narrow: 2.27) per 106 population, with a peak age of 60-69 years. The incidence of PAP was 0.56 (narrow: 0.41) per 106 population at risk, with a peak age of 50-59 years. Among incident cases, the male-to-female ratio was 1.52 and about two-thirds had comorbidities, dyslipidaemia being the most common. CONCLUSIONS The prevalence and incidence of PAP in Korea are low, similar to those in other countries; however, Korean patients with PAP are characterized by older diagnostic age and a lower male-to-female ratio.

中文翻译：

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韩国的肺泡蛋白沉着症：一项基于全国人群的研究分析了患病率和发病率。

背景技术肺泡蛋白沉着症（PAP）是一种非常罕见的肺部疾病，其患病率和发病率仍不清楚。通过使用韩国健康保险审查与评估服务的全国理赔数据，对PAP的患病率和发生率进行了调查。方法提取2010年至2016年间访问任何二级或三级医疗机构的成年人的数据，这些成年人使用PAP相关的《韩国疾病分类》（第7版代码J84.0）和《罕见病难治性豁免计算代码V222》。为了完善案例定义，在满足以下所有条件时进行了狭义的案例定义：1）在首次提出索赔要求的一年内两次以上的PAP编码访问，2）在第一项权利要求之前或之后的90天内，对胸部计算机断层扫描和诊断程序（支气管镜或肺活检）提出了一项以上的权利要求。结果从2010年到2016年，共确定了182例与PAP相关代码的患者（狭窄，n = 82），并且在2012年至2015年之间，有89名新患者（狭窄，n = 66）访问了医疗机构。PAP的患病率为4.44（窄：2.27）每106个人口，最高年龄为60-69岁。每106个高危人群中PAP的发生率为0.56（狭窄：0.41），峰值年龄为50-59岁。在突发事件中，男女之比为1.52，约三分之二患有合并症，血脂异常是最常见的。结论韩国的PAP患病率和发病率较低，与其他国家相似。然而，