Successful treatment of Erdheim-Chester disease by interleukin-1 receptor antagonist protein

Oncogene-Induced Senescence as a New Mechanism of Disease: The Paradigm of Erdheim–Chester Disease

“…Clinical observations on small numbers of patients demonstrated that treatment with cytokine-blocking agents could be effective in the management of the disease. In particular, treatment with the IL-1 receptor antagonist anakinra was associated to a favorable clinical response in a significant number of ECD patients with different disease manifestations (30–32). More limited clinical experience with TNF-α-blocking drugs also yielded encouraging results (33).…”

Variability in the efficacy of the IL1 receptor antagonist anakinra for treating Erdheim-Chester disease

“…We therefore investigated the association between all-cause mortality and carriership of somatic mutations in genes linked to clonal expansion of hematopoietic stem cells in a large elderly subsample (N 5 864, 80 years and older) derived from 2 large-scale communitydwelling Dutch cohort studies. 5,6 For the present study, we investigated whole-blood-derived genomes of 646 individuals of 80 years and older from the Rotterdam Study 5 (RS; mean age at inclusion, 84.6 years; range, 80.0-105.8 years; supplemental Appendix 2, available on the Blood Web site) and 218 individuals of 89 years and older from the Leiden Longevity Study 6 (LLS; mean age at inclusion, 94.0 years; range, 88.9-103.4 years; supplemental Appendix 2). Jointly, this elderly subsample consists of 597 participants aged 80 to 89 years and 267 participants aged over 90 years, which is twice the number of participants for the respective age categories as compared with any other study previously conducted on this topic.…”

“…1 Several reports have suggested that an interleukin (IL)1 receptor antagonist, anakinra, is effective in treating ECD. [2][3][4][5][6][7][8] Twelve patients (7 men and 5 women; median age, 70 years; range, 22-80 years) with biopsy-proven ECD 9 and previous failure (cases 2, 3, 4, 9, and 11), poor tolerance, or contraindication (cases 1, 2, 5, 6, 7, 8, 10, and 12) to interferon-a (IFN-a) therapy received alternative treatment with daily subcutaneous injection of 100 mg anakinra. Data collection and analysis were performed retrospectively.…”

“…Since then, the use of IL-1 blockade in ECD has been reported in 6 additional reports [3][4][5][6][7][8] (Table 2), which did not mention any changes in bone lesions or retroperitoneal infiltration. Killu et al 5 described a patient with a right atrial mass in which FDG uptake decreased under anakinra treatment, although there was no MRI evaluation of the cardiac infiltration.…”

Therapeutic evaluation of interleukin 1-beta antagonist Anakinra against traumatic brain injury in rats

“…Anakinra, an antagonist expressed in many tissues and organs, has been shown to competitively inhibit the binding of IL-1 to the IL-1 type receptor. [4] In patients with rheumatoid arthritis, this natural IL-1 receptor antagonist is not found in effective concentrations to counteract elevated IL-1 concentrations. Thus, Kineret is not considered a disease-modifying anti-rheumatic drug, but rather a biological response modifier due to its ability to selectively target the pathologic element of the disease.…”