Division of Rheumatology Allergy and Immunology, Massachusetts General Hospital, Boston, Massachusetts, USA
Harvard Medical School, Boston, Massachusetts, USA.
Ann Rheum Dis. 2023 Jul;82(7):957-962. doi: 10.1136/ard-2023-223950. Epub 2023 May 3.
IgG4-related disease (IgG4-RD) is an immune-mediated condition that can affect nearly any organ or anatomic site. We sought to describe the epidemiology of IgG4-RD in the USA.
We used Optum's deidentified Clinformatics Data Mart Database from 1 January 2009 to 31 December 2021 to identify IgG4-RD cases using a validated algorithm. We estimated the incidence rate and prevalence between 2015 and 2019 (when rates stabilised), standardised to the US population by age and sex. We compared mortality rates among patients with IgG4-RD to the non-IgG4-RD population matched in a 1:10 ratio on age, sex, race/ethnicity and encounter date. We used Cox proportional hazards models to estimate HRs and 95% CIs.
We identified 524 IgG4-RD cases. The mean age was 56.5 years with 57.6% female and 66% White. The incidence of IgG4-RD increased during the study period from 0.78 to 1.39 per 100 000 person-years in 2015 and 2019, respectively. The point prevalence on 1 Janury 2019 was 5.3/100 000 persons. During follow-up, there were 39 and 164 deaths among 515 IgG4-RD cases and 5160 comparators, resulting in a mortality rate of 3.42 and 1.46/100 person-years, respectively, and adjusted HR of 2.51 (95% CI 1.76 to 3.56).
The incidence of IgG4-RD is similar to that of systemic rheumatic diseases such as ANCA-associated vasculitis and systemic sclerosis but may be increasing as familiarity with this diagnosis grows. Clinicians should be aware of this condition, especially given the excess risk of death. Identification of effective therapies is an important research agenda.
IgG4 相关疾病（IgG4-RD）是一种免疫介导的疾病，可影响几乎任何器官或解剖部位。我们旨在描述 IgG4-RD 在美国的流行病学。
我们使用 Optum 的匿名 Clinformatics Data Mart 数据库，从 2009 年 1 月 1 日至 2021 年 12 月 31 日，使用经过验证的算法来确定 IgG4-RD 病例。我们估计了 2015 年至 2019 年（当发病率稳定时）的发病率和患病率，按年龄和性别标准化为美国人口。我们将 IgG4-RD 患者的死亡率与按年龄、性别、种族/族裔和就诊日期以 1:10 比例匹配的非 IgG4-RD 人群进行了比较。我们使用 Cox 比例风险模型估计 HR 和 95%CI。
我们确定了 524 例 IgG4-RD 病例。平均年龄为 56.5 岁，女性占 57.6%，白人占 66%。在研究期间，IgG4-RD 的发病率从 2015 年的 0.78 增至 2019 年的 1.39/100000 人年。2019 年 1 月 1 日的时点患病率为 5.3/100000 人。在随访期间，515 例 IgG4-RD 病例中有 39 例和 5160 例对照者死亡，导致死亡率分别为 3.42 和 1.46/100 人年，调整后的 HR 为 2.51（95%CI 1.76 至 3.56）。
IgG4-RD 的发病率与系统性风湿性疾病（如抗中性粒细胞胞浆抗体相关性血管炎和系统性硬化症）相似，但随着对该诊断的认识不断提高，发病率可能会增加。临床医生应注意这种情况，尤其是考虑到死亡风险增加。确定有效的治疗方法是一个重要的研究议程。
