Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, 1-1-5 Sendagi, Bunkyo-ku, Tokyo, 113-8602, Japan.
Nippon Boehringer Ingelheim Co., Ltd, Tokyo, Japan.
Adv Ther. 2022 May;39(5):2222-2235. doi: 10.1007/s12325-022-02078-5. Epub 2022 Mar 22.
Systemic sclerosis (SSc) is a complex autoimmune disease with increased mortality, and interstitial lung disease (ILD) is a major cause of death. There are no recent epidemiological data on SSc and SSc-associated ILD (SSc-ILD) in Japan and little is known about how patients with these diseases are treated.
The incidence rate and prevalence of SSc and SSc-ILD in Japan were estimated using the Japanese Medical Data Centre (JMDC) database. The demographic and clinical characteristics of patients and the immunomodulatory medications they received were also assessed using JMDC and the Medical Data Vision (MDV) databases. All analyses were descriptive.
The overall incidence rates of SSc and SSc-ILD per 100,000 person-years were 6.6 (95% confidence interval [CI] 6.2-7.1) and 1.9 (95% CI 1.6-2.1), respectively, and the overall prevalence per 100,000 persons was 37.0 (95% CI 35.6-38.5) and 13.9 (95% CI 13.0-14.8), respectively. ILD was the most common comorbidity in patients with SSc present in approximately 30% of patients (JMDC, 29.3%; MDV, 30.1%). The immunomodulatory medications prescribed were similar in patients with SSc and SSc-ILD, and each of the medications in this analysis was prescribed in less than 15% of patients.
We have demonstrated that estimates of prevalence and incidence rates of SSc and SSc-ILD in Japan are comparable to similar database studies conducted in the USA, using a medical claims database. Only a small proportion of patients were receiving immunomodulatory treatments, suggesting undertreatment in Japan. Incidence Rate and Prevalence of Systemic Sclerosis and Systemic Sclerosis-associated Interstitial Lung Disease in Japan: Analysis Using Japanese Claims Databases-A Video Abstract. (MP4 68892 KB).
系统性硬化症（SSc）是一种复杂的自身免疫性疾病，死亡率较高，间质性肺疾病（ILD）是主要死亡原因。目前尚无日本 SSc 和 SSc 相关间质性肺病（SSc-ILD）的最新流行病学数据，也不清楚这些疾病患者的治疗情况。
使用日本医疗数据中心（JMDC）数据库估算日本 SSc 和 SSc-ILD 的发病率和患病率。还使用 JMDC 和医疗数据视觉（MDV）数据库评估患者的人口统计学和临床特征以及他们接受的免疫调节药物。所有分析均为描述性分析。
每 100,000 人年 SSc 和 SSc-ILD 的总发病率分别为 6.6（95%置信区间[CI] 6.2-7.1）和 1.9（95%CI 1.6-2.1），每 100,000 人的总患病率分别为 37.0（95%CI 35.6-38.5）和 13.9（95%CI 13.0-14.8）。ILD 是 SSc 患者最常见的合并症，约 30%的患者存在ILD（JMDC，29.3%；MDV，30.1%）。在 SSc 和 SSc-ILD 患者中开具的免疫调节药物相似，本分析中的每种药物的处方率都低于 15%。
我们使用医疗索赔数据库展示了日本 SSc 和 SSc-ILD 的患病率和发病率估计与美国类似的数据库研究相当。只有一小部分患者接受免疫调节治疗，表明日本的治疗不足。日本系统性硬化症和系统性硬化症相关间质性肺病的发病率和患病率：使用日本索赔数据库的分析-视频摘要。
