A Rarity In Autoimmunity: IgG4-RD is More Common in Males
Although autoimmune diseases are generally more common in women, research across the world has consistently found that men are more than twice as likely to develop IgG4-RD than women. In addition, IgG4-RD demonstrates “striking” sex-dependent distribution of organ involvement as well as B-cell response, according to data published recently in The Lancet Rheumatology.
To learn more, IgG4ward! Founder Dr. John Stone and his colleagues at Massachusetts General Hospital conducted a retrospective cohort study of patients enrolled in the Massachusetts General Hospital Rheumatology Clinic IgG4-RD Registry.
The study examined data from 328 patients (69% male) enrolled in the cohort from January 2008 – May 2023 and learned:
Male prevalence increased with each decade of life:
47% among those diagnosed were younger than 40 years were male
77% among those diagnosed were over 70 years were male
In addition, findings from the study also showed that:
Men were an average 5.5 years older at diagnosis than women
Men exhibited higher ACR/EULAR classification criteria scores at baseline compared to women
Pancreatic and renal involvement were almost twice as likely to occur among men than women (50% vs. 26%).
Men were more likely to have “observable, active B-cell responses in the blood as defined by proportional expansions of plasmablasts.”
Dr. Arezou Khosroshahi, a rheumatologist and an Associate Professor at Emory School of Medicine, shares that research among her cohort produced similar results.
“Our cohort at Emory reflects similar findings, with 60% of our patients being male. This epidemiological fact is significant for investigating the causes of IgG4-RD, as it contrasts with many other autoimmune diseases.”
Dr. Khosroshahi emphasizes the importance, however, of not letting a patient's sex determine diagnosis and treatment options:
“It is crucial to emphasize that clinical decisions regarding the diagnosis and treatment of patients with IgG4-RD should not be influenced by the patient's sex. Although women may present less frequently with certain organ involvement or severe disease compared to men, they can still experience severe manifestations and require the same treatment."
Dr. Emma Culver, a Consultant Hepatologist & Gastroenterologist at John Radcliffe Hospital in Oxford, UK, concurs:
“Similarly, we observe an increased predilection for males to present with IgG4-related disease involving the pancreas, bile ducts and kidneys in our United Kingdom patient cohort. We also recognize a greater disease burden in terms of serological IgG4 levels, multiplicity of organ involvement, and often more urgent disease manifestations presenting in men.”
The increased frequency of IgG4-RD in males is an extremely interesting characteristic of this condition, and one that we must ultimately understand thoroughly as we unravel the many mysteries about this disease that remain.
If you’d like to learn more about the frequency of IgG4-RD in men vs. women, we encourage you to do so here.
