Background 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.
Methods 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.
Results 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).
Conclusions 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.
- Immune System Diseases
- Autoimmune Diseases
Data availability statement
Data may be obtained from a third party and are not publicly available. Not applicable.
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Handling editor Josef S Smolen
Contributors ZSW, GM, DM and HKC had access to the study data, developed the figures and tables, and vouch for the data and analyses. GM and DM performed the statistical analyses and contributed to data quality control, data analysis and interpretation of the data. ZSW, GM, ES, NP-I, DM, CC, XF, YZ, JHS and HKC contributed to data collection, data analysis and interpretation of the data. ZSW directed the work, designed the data collection methods, contributed to data collection, data analysis and interpretation of the data and had final responsibility for the decision to submit for publication. ZSW accepts full responsibility for the work and/or the conduct of the study, had access to the data, and controlled the decision to publish. All authors contributed intellectual content during the draft and revision of the work and approved the final version to be published.
Funding This study was supported through a grant to Massachusetts General Hospital from Sanofi. ZSW is funded by NIH/NIAMS (K23AR073334 and R03AR078938) and the RRF (K Supplement).
Competing interests ZSW is funded by NIH/NIAMS (K23AR073334 and R03AR078938) and the RRF (K Supplement). ZSW reports research support from Bristol-Myers Squibb and Principia/Sanofi and consulting fees from Zenas Biopharma, Horizon, Sanofi, Shionogi, Viela Bio and MedPace. GM, ES, NP-I and DM are employees of Sanofi and may hold stock in Sanofi.
Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.
Provenance and peer review Not commissioned; externally peer reviewed.
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