PW03-020 – A decade of ANTI-IL-1 therpay for CAPS in the UK

In October 2002, the first patient with CAPS was treated successfully with the anti-IL-1 agent anakinra at our Centre in the UK, and in 2009 a nationally funded canakinumab treatment service initiated for CAPS in England. By the end of 2012, 82 symptomatic individuals have been assessed at our Centre.


Introduction
In October 2002, the first patient with CAPS was treated successfully with the anti-IL-1 agent anakinra at our Centre in the UK, and in 2009 a nationally funded canakinumab treatment service initiated for CAPS in England. By the end of 2012, 82 symptomatic individuals have been assessed at our Centre.

Objectives
To describe our experience, and outcomes of 82 individuals with clinical CAPS, including treatment and natural history.

Methods
We examined all available medical and laboratory records.
24 patients are on anakinra and over a median FU of 47 months (IQR 12-72) 20 remain in CR and 4 in PR. 6 patients have previously tried canakinumab; 2 had CR but were converted due to: planned pregnancy; planned insertion of a ventricular peritoneal shunt. One adult CINCA patient with a good PR could not tolerate travel to our Centre. A female with mutation negative MWS previously in CR on anakinra opted for a trial of canakinumab, but developed a massive disease flare resulting in hospitalisation. She subsequently reverted to anakinra and is once again in CR. 2 males (A439V, T346I) discontinued canakinumab due to: lack of efficacy; development of major systemic inflammation and a morphea like rash. On anakinra the former remains in PR whilst the latter has experienced CR.
2 children with mild R260W disease have declined treatment.

Conclusion
In this series T348M underlies more severe disease than the other common mutations. A decade of IL-1 blockade confirms its efficacy and relative safety in CAPS across the clinical severity spectrum. Canakinumab is 1 National Amyloidosis Centre, University College London, UK Full list of author information is available at the end of the article the more popular drug due to its long action; however, a small number of patients are unresponsive to this therapy, suggesting a possible role of IL-1α.

Disclosure of interest
None declared.