Men develop IgG4-related disease more than twice as often as women
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Key takeaways:
- Pancreatic and renal involvement was nearly twice as common in men, who showed more active B-cell responses.
- The results pose a “striking contrast” to other autoimmune diseases, which predominantly affect women.
Men are more than twice as likely than women to develop IgG4-related disease, and demonstrate “striking” sex-dependent organ distribution and B-cell response, according to data published in The Lancet Rheumatology.
“IgG4-related disease (IgG4-RD) is a condition that has been identified as a unique disease only in this century — just more than 20 years ago. For this reason, much remains unknown about the epidemiology of this disorder,” John H. Stone, MD, MPH, professor of medicine at Harvard Medical School, told Healio.
“Case series from a variety of institutions around the world have focused on specific organ manifestations of IgG4-RD and may have suggested a male predominance for some organ manifestations,” he added. “However, there has been no large, single-center study, to date, that studies patients with all types of organ manifestations and uses the American College of Rheumatology/EULAR classification criteria as the basis for inclusion.”
To better understand sex differences in IgG4-RD, Stone and colleagues conducted a retrospective cohort study of patients enrolled in the Massachusetts General Hospital Rheumatology Clinic IgG4-RD Registry. The study included 328 patients meeting the ACR/EULAR classification criteria for IgG4-RD and examined data from January 2008 to May 2023.
The researchers assessed age at diagnosis, baseline organ involvement, treatment status and pre-treatment laboratory values. In addition, they used flow cytometry to quantify circulating plasmablasts and B-cell subsets.
According to the researchers, the results showed a “strong male predominance,” with 69% of study participants being men. This male predominance grew with each decade of life starting at age 40 years, from 47% among those diagnosed while younger than 40 years, to 77% among those diagnosed after 70 years.
Additionally, men were an average 5.5 years older at diagnosis than women (63.7 years vs. 58.2 years; P = .0031) and demonstrated higher ACR/EULAR classification criteria scores at baseline (median 35 vs. 29.5; P = .001). Meanwhile, pancreatic and renal involvement was almost twice as common among men as among women — 50% vs. 26%. Men were also more likely to have “observable, active B-cell responses in the blood as defined by proportional expansions of plasmablasts” (P = .0095), the researchers wrote.
“The study demonstrates not only that IgG4-RD is more likely to occur in men, but also that males are more likely to have severe disease in the sense of greater organ involvement and higher degrees of serological activity,” Stone said. “IgG4-RD is generally considered to be an autoimmune disease, even though there is no specific autoantibody that has been associated with the condition. The great majority of autoimmune diseases have an overwhelming female predominance. The data from this study pose a striking contrast, therefore, because two-thirds of the patients in this study were male.
“A logical follow-up to these findings is the question of whether or not male and female patients have differential treatment outcomes,” he added. “This will be critical to examine not only in this cohort, but also in ongoing clinical trials, such as the MITIGATE trial.”
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