Dr. Obici has received fees for lecturing (less than $10,000) from Ortho Biotech.
Persistent efficacy of anakinra in patients with tumor necrosis factor receptor–associated periodic syndrome
Article first published online: 25 APR 2008
Copyright © 2008 by the American College of Rheumatology
Arthritis & Rheumatism
Volume 58, Issue 5, pages 1516–1520, May 2008
How to Cite
Gattorno, M., Pelagatti, M. A., Meini, A., Obici, L., Barcellona, R., Federici, S., Buoncompagni, A., Plebani, A., Merlini, G. and Martini, A. (2008), Persistent efficacy of anakinra in patients with tumor necrosis factor receptor–associated periodic syndrome. Arthritis & Rheumatism, 58: 1516–1520. doi: 10.1002/art.23475
- Issue published online: 25 APR 2008
- Article first published online: 25 APR 2008
- Manuscript Accepted: 11 FEB 2008
- Manuscript Received: 13 OCT 2007
- Telethon Italia
- Fondazione C. Golgi, Brescia, Italy
To evaluate the efficacy and safety of treatment with the interleukin-1 receptor antagonist anakinra in patients with tumor necrosis factor receptor–associated periodic syndrome (TRAPS) requiring high cumulative doses of steroids.
Four children (mean age 9.1 years [range 4–13 years]) and 1 adult (age 33 years) with TRAPS were enrolled in the study. The 3 children with cysteine mutations (C52Y, C55Y, C43R) had prolonged and frequent attacks of fever. One child with the R92Q mutation and the adult patient with the C43R mutation displayed a more chronic disease course, with fluctuating, nearly continuous symptoms and persistent elevation of acute-phase reactant levels (including serum amyloid A [SAA]). All patients were treated with anakinra (1.5 mg/kg/day).
All of the patients had a prompt response to anakinra, with disappearance of symptoms and normalization of acute-phase reactant levels, including SAA. In all pediatric patients, anakinra was withdrawn after 15 days of treatment. After a few days (mean 5.6 days [range 3–8]) a disease relapse occurred, which dramatically responded to reintroduction of anakinra. During the following period of observation (mean 11.4 months [range 4–20 months]), the patients did not experience episodes of fever or other disease-related clinical manifestations. Levels of acute-phase reactants remained in the normal range. No major adverse reactions or severe infections were observed.
Continuous treatment with anakinra effectively controlled both the clinical and laboratory manifestations in patients with TRAPS and prevented disease relapses.