5PSQ-074 Canakinumab in familial mediterranean fever and secondary amyloidosis: a case report

Background Familial mediterranean fever (FMF) is an autosomal recessive disease characterised by repeated and self-limited seizures of fever and serositis. Classically, FMF has been treated with colchicine, although currently we have interleukin-1β inhibitors such as anakinra or canakinumab. Purpose To describe a case of FMF and secondary amyloidosis in current treatment with canakinumab. Material and methods Description of a case of FMF, in follow-up in our hospital and in current treatment with canakinumab. Data was collected from the electronic medical record and analytics were reviewed in the laboratory application. The variables analysed were sex, age, neutrophil value, haemoglobin, C-reactive protein (CRP) and renal function before and after treatment with canakinumab and adverse reactions to treatment. Results A 74-year-old female diagnosed with FMF, chronic kidney disease and hypertensive heart disease was followed up in our hospital since 2006. Treatment with colchicine 0.5 mg daily since then. Febrile episodes in 2009. Period 2010–2014 practically asymptomatic, with some episodes of fever that were self-limited with acetaminophen. She was admitted to hospital in December 2014 due to a fever outbreak and amyloidosis with renal insufficiency. In January 2015, anakinra 100 mg subcutaneous three times weekly was started, which was suspended in September 2015 due to severe renal failure and lack of response. In September 2015 etanercept 50 mg subcutaneous weekly was started but continued with fever outbreaks. There were four admissions due to decompensated heart failure in summer 2017 associated with outbreaks of FMF and anaemia (8.7 g/dL) despite darbepoetin. Other values: CRP:100 mg/L; neutrophils: 68.9%; and glomerular filtration:12 mL/min. In September 2017 treatment with canakinumab 150 mg subcutaneous every 8 weeks was requested, which was currently associated with colchicine 0.5 mg daily. The patient did not present an admission or febrile seizures since the onset of canakinumab: haemoglobin had reached normal values (13.7 g/dL), despite the fact that neutrophilia continued (83%), elevated CRP (70 mg/L) and deficient renal function (13 mL/min). No adverse reactions were reported. Conclusion Canakinumab is a valid therapeutic alternative in the treatment of FMF in case of poor response to other therapies: the observed evolution is favourable until now, being also safe and well tolerated. However, more prospective studies are needed to assess their suitability in this context. References and/or acknowledgements https://ejhp.bmj.com/content/24/Suppl_1/A149.1 No conflict of interest.