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Prevalence and Mortality of IgG4-related disease in the USA

May 04, 2023 5:00 pm

A current review of the epidemiology of IgG4-related disease (IgG4-RD) in the USA shows a low prevalence with possibly increasing numbers, owing to increasing awareness of this unique immune-mediated condition.

Epidemiologic data and outcomes were gleaned from analysis of commercial insurance claims (Optum’s Clinformatics Data Mart Database; 2009 to 2021). Mortality rates for IgG4-RD were compared to a non-IgG4-RD population matched in a 1:10 ratio on age, sex, and race/ethnicity. 

They identified a total of 524 IgG4-RD cases, with a mean age of 56.5 years (57.6% female).  Key frequency indicators included:

  • IgG4-RD incidence increased between 2015 and 2019; from 0.78 to 1.39 per 100 000 person-years
  • January 2019 point prevalence was 5.3/100 000 persons.
  • Mortality rates were increased in IgG4-RD (3.42 vs 1.46/100 person-years; adjusted HR 2.51 [95% CI 1.76 to 3.56])

IgG4-RD appears to be nearly as common as ANCA-associated vasculitis or systemic sclerosis.  Acknowledgement of this disorder is important given the excess mortality risk.


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The author has no conflicts of interest to disclose related to this subject