Ceasing both immunosuppressants, glucocorticoids leads to IgG4-RD relapse in most patients
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SAN DIEGO — Maintaining immunosuppression with or without low-dose glucocorticoids is superior to withdrawing both therapies in preventing relapses in IgG4-related disease, according to data presented at ACR Convergence 2023.
“We know IgG4 disease can affect a variety of organs,” Linyi Peng, MD, of Peking Union Medical College Hospital, in Beijing, China, told attendees. “Early treatment can prevent organ damage.”
Peng noted that first-line glucocorticoids are effective in about 95% of patients.
“Most patients respond well,” she said. “However, the disease has a high relapse rate after stopping steroids.”
To assess whether discontinuing glucocorticoids and immunosuppressive agents would lead to relapse in patients with stable IgG4-related disease, Peng and colleagues conducted the multicenter, open-label, randomized controlled WInS IgG4-RD trial. The analysis included 146 patients with clinically inactive IgG4-related disease who were receiving maintenance therapy with glucocorticoids and immunosuppressive agents for at least 12 months.
The participants were divided into three groups. Study group one included 48 patients who withdrew both glucocorticoids and immunosuppressants, while group two was comprised of 49 patients who withdrew glucocorticoids but not immunosuppressants, and group three included 49 patients who maintained maintenance therapy with both. The 18-month relapse rate in each group served as the primary endpoint. The researchers additionally assessed changes in responder index, physician global assessment, serum IgG4 and IgG, as well as adverse events.
According to the researchers, the 18-month relapse rate among patients who withdrew both immunosuppressants and glucocorticoids was 52.1%, compared with 14.1% for those who withdrew only glucocorticoids and 12.2% for those who maintained both therapies (P < .001).
“Groups two and three showed significantly higher freedom from relapse compared with group one,” Peng said. “No significant differences showed between groups two and three.”
According to Peng, risk factors for relapse included higher levels of submandibular gland and prostate involvement.
Further data showed that the change in responder index and physician’s global assessment among patients in the double withdrawal group were significantly higher than in those who withdrew only glucocorticoids (P < .001) or maintained both therapies (P < .001).
“Compared to withdrawal of glucocorticoids and immunosuppressants, immunosuppressants with or without glucocorticoids showed lower disease relapse rates and mitigated the elevation of serum IgG4 levels, IgG4-related disease responder index and Physician’s Global Assessment scores,” Peng said.
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Peng L. Abstract L16. Presented at: ACR Convergence 2023; Nov. 10-15, 2023; San Diego.
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