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We previously reported a case of amyloidosis secondary to Muckle-Wells syndrome (MWS).1 Briefly, it was a 26-year-old female patient with periodic fever, nephrotic syndrome and sensorineural hearing loss. Mild conjunctivitis and arthralgia-myalgias during attacks were the accompanying features. Inter-attack serum acute phase reactants were elevated with peaks during attacks. Kidney biopsy revealed amyloid A amyloidosis and a clinical diagnosis of MWS was established. No mutation was detected in CIAS1 gene and no family history was present. She responded well to treatment with anakinra after which 24-hour urinary protein decreased from 6.1 to 1.2 g and remained stable thereafter (Figure 1). Periodic fever, increased acute phase reactants and serum amyloid A (SAA), hypoalbuminemia, edema and other disease features resolved completely as well, except hearing loss which needed a cochlear implant to be corrected. After 18 months of treatment with anakinra, 100 mg (2 mg/kg) per day subcutaneously, the patient complained of difficulty with daily injections impairing drug adherence and the anti-interleukin-1 therapy was switched to canakinumab, 150 mg/ month subcutaneously. Interestingly, proteinuria decreased further to almost normal levels (around 200 mg/day) after the switch (Figure 1). She is now being followed-up under treatment with canakinumab without any sign of disease activity and normal serum C-reactive protein (CRP) and SAA levels.

Additional Benefit of Canakinumab on Proteinuria in a Case With Muckle-Wells Syndrome in Remission Under Anakinra