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The following is a summary of “Anakinra improves retention rate of targeted treatments in Erdheim-Chester disease,” published in the January 2025 issue of Rheumatology by Campochiaro et al.
Erdheim-Chester disease (ECD) is a rare histiocytosis with diverse clinical manifestations. Targeted therapies, such as BRAF- and MEK-inhibitors, are used following MAPK-ERK pathway mutations.
Researchers conducted a retrospective study to assess drug retention of targeted therapies in patients with ECD from 2 Italian referral centers.
They retrospectively analyzed the drug retention rate (DRR) in 50 patients with ECD who received targeted therapies, either alone or in combination with anakinra. The analysis focused on the 18-month DRR.
The results showed that among 52 patients undergoing 60 treatment courses, vemurafenib was the most used (72%), followed by cobimetinib (23%), trametinib (3%), and dabrafenib (2%). The overall 18-month DRR was 72%, with no significant differences between agents, and adverse reactions caused 76% of discontinuations. About 16 patients (27%) received anakinra combined with targeted therapies, resulting in a significantly higher 18-month DRR (94% vs 65%, P = 0.0251), with survival analysis showing better retention in the anakinra group (log-rank test P = 0.040).
Investigators found an 18-month DRR of 72% in patients with ECD on targeted therapies, with the anakinra combination significantly improving retention.
Source: academic.oup.com/rheumatology/advance-article-abstract/doi/10.1093/rheumatology/keaf023/7954689
